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We present physical properties of ZnO nanorods irradiated with slow highly charged Bismuth (Bi) ions of different fluences. Samples were grown on Si substrates by hydrothermal technique at 90 °C and were found to be randomly distributed. Room temperature Raman measurements showed the decrease and the blue shift of $$ {E}_{2}^{\text{high}} $$ mode of irradiated samples, suggesting the suppression of crystallinity and the growth of stress. Signature of dynamic annealing (defect-annihilation) of radiation defects at higher doses was observed. Room temperature photoluminescence studies showed similar features in two regions: ultraviolet and broad visible regions. Both regions were unaffected with increased ion fluences. UV emission was found to be related to the FX-2LO transition and visible emission to the intrinsic defects of the samples. X-ray emissions detected in ion-surface interactions confirmed the formation and the decay of hollow Bi atoms during their approach to the sample surface. [ABSTRACT FROM AUTHOR]

In allo-stem cell transplantation (SCT), it is unclear whether donor-specific anti-HLA Abs (DSAs) can actually mediate graft rejection or if they are simply surrogate markers for the cellular immunity that causes graft rejection. Here, we first analyzed a case of cord blood allograft rejection in which DSA and cytotoxic T lymphocyte (CTL) specific for donor HLA-B*54:01 were detected at the time of graft rejection. Both the DSA and CTL inhibited colony formation by unrelated bone marrow mononuclear cells sharing HLA-B*54:01, suggesting that the humoral and cellular immune responses were involved in the graft rejection. Interestingly, the DSA and CTL were also detected in cryopreserved pre-transplant patient blood, raising a hypothesis that the presence of anti-HLA Abs could be an indicator for corresponding HLA-specific T cells. We then evaluated the existence of HLA-specific CD8+ T cells in other patient blood specimens having anti-HLA class I Abs. Interferon-γ enzyme-linked immunospot assays clearly confirmed the existence of corresponding HLA-specific T-cell precursors in three of seven patients with anti-HLA Abs. In conclusion, our data demonstrate that integrated humoral and cellular immunity recognizing the same alloantigen of the donor can mediate graft rejection in DSA-positive patients undergoing HLA-mismatched allo-SCT. Further studies generalizing our observation are warranted. [ABSTRACT FROM AUTHOR]

Significant fraction of the cosmic diffuse soft X-ray emission (0.1-1 keV) is caused by the Solar Wind Charge eXchange (SWCX) process between the solar wind ion (C, N, O etc.) and the interplanetary neutral matter. It is difficult to identify spectral features of SWCX with the spectral resolution of existing X-ray astronomy satellites. We are developing a laboratory experimental system with transition edge sensor (TES) X-ray microcalorimeters, in order to clarify the SWCX mechanism. This experiment is designed to measure Charge eXchange (CX) X-rays using Electron Cyclotron Resonance Ion Source (ECRIS) that generates multi-charged ions. Emission lines (O: 2p→1s; 654 eV) by CX between O and neutral He atom is aimed to be measured with energy resolution better than 10 eV. The TES microcalorimeter is cooled by a double-stage adiabatic demagnetization refrigerator (DADR), however, our TES microcalorimeter are not working potentially due to magnetic field contamination. This paper reports our experimental system, present results, and future prospects. [ABSTRACT FROM AUTHOR]

Comparison of polarized optical absorption spectra of natural Ca-rich diopsides and synthetic NaCrSiO and LiCrSiO clinopyroxenes, evidences as vivid similarities, as noticeable differences. The similarities reflect the fact that in all cases Cr enters the small octahedral M1-site of the clinopyroxene structure. The differences are due to some iron content in the natural samples causing broad intense near infrared bands of electronic spin-allowed dd transitions of Fe(M1, M2) and intervalence Fe/Fe charge-transfer transition, and by different symmetry and different local crystal fields strength of Cr in the crystal structures. The positions of the spin-allowed bands of Cr, especially of the low energy one caused by the electronic A → T transition, are found to be in accordance with mean M1-O distances. The local relaxation parameter ε calculated for lim → 0 from the spectra and interatomic $$ \left\langle {Cr - O} \right\rangle $$ and $$ \left\langle {Mg - O} \right\rangle $$ distances yields a very high value, 0.96, indicating that in the clinopyroxene structure the local lattice relaxation around the 'guest' ion, Cr, deviates greatly from the 'diffraction' value, ε = 0, than in any other known Cr-bearing systems studied so far. Under pressure the spin-allowed bands of Cr shift to higher energies and decrease in intensity quite in accordance with the crystal field theoretical expectations, while the spin-forbidden absorption lines remain practically unshifted, but also undergo a strong weakening. There is no evident dependence of the Racah parameter B of Cr reflecting the covalence of the oxygen-chromium bond under pressure: within the uncertainty of determination it may be regarded as practically constant. The values of CrO octahedral modulus, $$ k_{\text{poly}}^{\text{loc}} $$, derived from high-pressure spectra of natural chromium diopside and synthetic NaCrSiO kosmochlor are very close, ~203 and ~196 GPa, respectively, being, however, nearly twice higher than that of MgO octahedron in diopside, 105(4) GPa, obtained by Thompson and Downs (). Such a strong stiffening of the structural octahedron, i.e. twice higher value of $$ k_{{{\text{Cr}}^{3 + } }}^{\text{loc}} $$ comparing with that of $$ k_{{{\text{Mg}}^{2 + } }}^{\text{loc}} $$, may be caused by simultaneous substitution of Ca by larger Na in the neighboring M2 sites at so-called jadeite-coupled substitution Mg + Ca → Cr + Na. It is also remarkable that the values of CrO octahedral modulus of NaCrSiO kosmochlor obtained here are nearly twice larger than that of 90(16) GPa, evaluated by high-pressure X-ray structural refinement by Origlieri et al. (). Taking into account that the overall compressibility of the clinopyroxene structure should mainly be due to the compressibility of M1- and M2-sites, our $$ k_{{{\text{Cr}}^{3 + } }}^{\text{loc}} $$-value, ~196 GPa, looks much more consistent with the bulk modulus value, 134(1) GPa. [ABSTRACT FROM AUTHOR]

We report on the growth of very thick (>260 µm) high-crystalline-quality single-crystal CdTe epitaxial films on (211) Si substrates in a metalorganic vapor-phase epitaxy reactor, and the development of gamma ray detectors and their radiation detection properties. Films were grown with a high growth rate varying from 40 µm/h to 70 µm/h. A heterojunction diode was fabricated by growing a 90-µm-thick CdTe layer on an n+-Si substrate, which exhibited good rectifying behavior and had a low reverse bias leakage current of 0.18 µA/cm² at 100 V bias. The diode clearly demonstrated its gamma radiation detection capability by resolving energy peaks from the 241Am radioisotope during room-temperature measurements. By cooling the diode detector to -30°C, the leakage current could be reduced by three orders of magnitude from the room-temperature value. At this operating condition dramatic improvements in the pulse height spectrum were observed. [ABSTRACT FROM AUTHOR]

To examine geographic differentiation in Asarum heterotropoides var. heterotropoides in Hokkaido Is. in the northern Japan, in which two putative cryptic species have been suspected to exist, extensive and detailed morphological research on 794 individuals from 44 populations throughout Hokkaido Is. was performed. Among the characters examined, the angle between and tip shape of the calyx lobes and the supratecta of the pollen grains were significantly correlated and were found in similar geographic clines. Among them, the pollen showed two discrete states in almost distinct distribution. Multidimensional scaling analysis showed that individuals within each of the two pollen types had different trends in flower characters. Consequently, we assumed a cline from south to north on Hokkaido Is. For the causes of the cline, the two hypotheses were proposed, primary geographic differentiation or extensive introgressive hybridization between two distinct geographical species existing in the past. [ABSTRACT FROM AUTHOR]

The objective of this study was evaluate the relationships between abnormal pulmonary circulation, lung function, and respiratory response during exercise in Fontan patients. Pulmonary function and cardiopulmonary exercise tests were performed in 101 Fontan patients and 122 controls. A small vital capacity (VC) with a high residual volume-to-total lung capacity ratio and a slight but significant low arterial saturation with hypocapnia were observed in Fontan patients. The number of surgical procedures determined VC. Total cavopulmonary connection, fenestration. higher pulmonary arterial wedge pressure, and smaller VC were independent determinants of low arterial saturation, which was the only determinant of hypocapnia. Arterial saturation decreased during exercise and resting arterial saturation correlated with that at peak exercise. Improvement in dead space ventilation was less in Fontan patients and was independently determined by resting arterial saturation. A steeper minute ventilation-carbon dioxide production slope was determined by resting arterial saturation, arterial carbon dioxide tension, and peak oxygen uptake. In Fontan patients, in addition to dead space ventilation, surgery-related reduced VC, the type of repair, and high pulmonary arterial wedge pressure cause arterial desaturation with subsequent hypocapnia. resulting in accelerated inefficient ventilation at rest and during exercise. [ABSTRACT FROM AUTHOR]

Background. The objectives of this study were to evaluate the effects of the angiotensin II receptor antagonist losartan on blood pressure (BP), proteinuria, and renal function in hypertensive patients with IgA nephropathy. Method. The study subjects comprised 18 patients with biopsy-proven IgA nephropathy with mild hypertension. Patients were classified into three groups (good/relatively good, relatively poor, poor) according to renal histologic findings and treated once a day with losartan 50 mg for 12 months. Changes in BP, proteinuria, renal function, and biochemical parameters were prospectively evaluated before and after the treatment. Results. BP began to fall after 1 month and proteinuria decreased significantly after 9 months of therapy. Glomerular filtration rate (GFR), renal plasma flow (RPF), and filtration fraction (FF) did not change throughout the observation period. There was no significant difference between the three different histologic groups in relation to the effects of losartan on BP, proteinuria, and renal function. We found that in patients with a proteinuria reduction rate of more than 50%, RPF increased and FF decreased significantly. Although it has been reported that losartan has a uric acid lowering effect, this study could not confirm such an effect. Conclusions. Losartan lowers blood pressure and decreases proteinuria in hypertensive patients with IgA nephropathy. These effects appear to be independent of renal histologic findings. This study suggests that the antiproteinuric effect of losartan is primarily mediated by changes in glomerular hemodynamics. [ABSTRACT FROM AUTHOR]

Mirror-image polydactyly of hands and feet (MIP) is a very rare congenital anomaly characterized by mirror-image duplication of digits. To isolate the gene responsible for MIP, we performed translocation breakpoint cloning from an MIP patient with t(2;14)(p23.3;q13). We isolated a good candidate gene for MIP that was disrupted by the translocation of the patient. We had previously constructed a 1.2-megabase bacterial artificial chromosome (BAC)/P1-derived artificial chromosome (PAC) contig covering the 14q13 breakpoint of t(2;14)(p23.3;q13). From a 500-kb segment consisting of seven BAC/PAC clones in the contig, we isolated a novel gene (the mirror-image polydactyly 1 gene, designated as MIPOL1, GenBank Accession No. AY059470), in addition to the hepatocyte nuclear factor 3 alpha gene (HNF3A, GenBank Accession No. XM 007360). MIPOL1 spans about 350 kb, comprises 15 exons, and encodes 442 amino acids. Northern blot analysis revealed that MIPOL1 expression is definite but very weak in adult heart, liver, skeletal muscle, kidney, and pancreas, and in fetal kidney. In view of the genome sequence and the contig map constructed, the 14q13 breakpoint of the patient was identified as located in intron 11 of MIPOL1, indicating that the gene was disrupted by the translocation, and that the breakage resulted in MIPOL1 protein truncation. Whole-mount in situ hybridization in mouse resulted in mouse Mipol1 signals all over E10.5–E13.5 mouse embryos. Two other unrelated patients with limb anomalies similar to MIP were subjected to mutation analysis of MIPOL1, but none had any mutations. We then isolated BAC clones from the other breakpoint, 2p23.3. A search for genes and expressed sequence tags in a more than 300-kb region around the 2p23.3 breakpoint found only the neuroblastoma-amplified protein gene (NAG, GenBank Accession No. NM 015909), which is located at least 50 kb centromeric to the breakpoint and is not likely to be related to MIP. MIPOL1 is a good candidate... [ABSTRACT FROM AUTHOR]

Background. The type X collagen gene (COL10A1) is currently known as the disease-causing gene of metaphyseal dysplasia type Schmid (MDS), whereas a mutation of COL10A1 has been reported to cosegregate with a disease phenotype of mild spondylometaphyseal dysplasia (SMD) in a Japanese family.¶Objective. To elucidate whether or not spondylar dysplasia is common in patients with mutations of COL10A1.¶Materials and methods. We re-evaluated the radiological manifestations in six patients with mutations of COL10A1, who had been previously reported as having MDS.¶Results. Two of six patients showed mild platyspondyly in infancy and early childhood. In both patients, the spondylar dysplasia tended to normalize with age, but mild alterations of the vertebral bodies persisted, even into late childhood. The other radiological manifestations of both patients were identical to those of MDS.¶Conclusion. Our observation suggests that mild spondylar dysplasia may not be uncommon in MDS. [ABSTRACT FROM AUTHOR]

Recent studies have indicated a close relationship between inactivation of tumor suppressor genes (TSGs) and disease progression. The genes encoding the cyclin-dependent kinase inhibitors p16INK4A and p15INK4B are potent TSGs, and correlations between their inactivation and disease progression have also been reported in various malignancies. In this study, we analyzed the methylation status of p16INK4A and p15INK4B gene promoters in plasma cell dyscrasias (PCDs) by methylation-specific PCR (MSP). In analyses using DNAs extracted from bone marrow mononuclear cells (BM-MNCs), patients with multiple myeloma (MM) showed frequent hypermethylation of the p16INK4A gene (15/37, 41%), whereas p15INK4B gene methylation was not so frequent (5/37, 14%). Many patients whose BM-MNC showed dense methylation of the p16INK4A gene had extramedullary plasmacytoma (extra-PC), and all available extra-PC samples showed alterations of the p16INK4A gene (4; dense methylation, 1; homozygous deletion). In contrast to MM, hypermethylation of the p16INK4A gene was significantly infrequent in indolent PCDs (2/22, 9%, P= 0.0055). The infrequency in indolent PCDs was also confirmed by analyses using DNAs extracted from BM smears (1/29, 3%). It is possible that hypermethylation of the p16INK4A gene promoter contributes to progression to aggressive MM from indolent PCD, especially to extra-PC development. [ABSTRACT FROM AUTHOR]

Accumulating evidence suggests the involvement of TGF-β in the process of corneal opacity, which is one of the serious causes of visual loss. However, whether TGF-β is indeed critical for the pathogenesis remains unknown. We constructed an adenovirus expressing an entire ectodomain of the human type II TGF-β receptor fused to Fc portion of human IgG (AdTβ-ExR): this soluble receptor is secreted from AdTβ-ExR-infected cells, binds to TGF-β and inhibits TGF-β signaling. When AdTβ-ExR was injected into the femoral muscle of Balb/c mice, a high level of the soluble receptor protein (2.0-3.5 × 10³ pM) was detectable in the serum and in the ocular fluid for at least 10 days. In the mice subjected to corneal injury with silver nitrate and to intramuscular injection with either saline or a control adenovirus expressing β-galactosidase (AdLacZ), corneal opacification composed of extracellular matrix (ECM) accumulation, of infiltration of neutrophils and monocytes/macrophages, and of angiogenesis were all induced. In contrast, they were markedly reduced in the mice injected with AdTβ-ExR. Immunohistochemical analysis revealed that TGF-β, fibronectin, macrophage chemoattractant protein-1, and vascular endothelial growth factor were densely stained in the edge of wounded cornea, but they were scarcely present in the injured-cornea of AdTβ-ExR-treated mice. Our results demonstrate that TGF-β indeed plays a critical role in the process of cornea opacification, and that adenovirusmediated expression of a soluble TGF-β receptor can be therapeutically useful. [ABSTRACT FROM AUTHOR]

Unlabelled: We report the case of a male patient with infantile hypophosphatasia associated with severe hypercalcaemia and mild respiratory insufficiency. At the age of 2 months, severe hypercalcaemia, low levels of serum alkaline phosphatase activity, and elevated urinary excretion of calcium and phosphoethanolamine were noted. Radiological findings showed generalized osteopenia and disturbed and irregular ossification of the metaphyses. Their involvement had spontaneously improved at the age of 6 months. A genetic study revealed that the tissue-nonspecific alkaline phosphatase gene of the patient had two novel mutations, K207E and G409C, derived from the mother and father, respectively. A reconsitution experiment revealed that both mutant gene products had low but significant enzymatic activity.Conclusion: The detection of tissue-nonspecific alkaline phosphatase gene mutations and expression studies to determine the enzymatic activity of mutant gene products was useful for assessing the clinical course of this patient with hypophosphatasia. [ABSTRACT FROM AUTHOR]

Interposed fibrous tissue at bone–implant interfaces was quantitatively measured in the presence or absence of polyethylene (PE) or alumina particles. Three different conditions of the interface were designed by implanting a pre-polymerized polymethylmethacrylate (PMMA) plug (plug group), a doughy PMMA (injection group) and a hydroxyapatite (HA) plug (HA group) in the hole drilled at the intercondylar notch of rabbit knees. PE (170±18 μm) or alumina particles (88±26 μm) were repeatedly administered into the knee joints at one month intervals (six times). All animals were sacrificed seven months after the implantation. The bone–implant interface was histomorphometrically examined using undecalcified ground sections. In the plug group, the PE particles significantly increased the extent of the interposed fibrous tissue ( p < 0.05), while the alumina particles showed no effect. In contrast, both particles showed no significant effects in the injection and the HA groups. These results indicate that both particle characteristics and conditions of the bone–implant interface affected particle-induced fibrous tissue interposition. The loose PMMA plug with PE particles induced the greatest amount of fibrous tissue interposition. ©2000 Kluwer Academic Publishers [ABSTRACT FROM AUTHOR]

We previously reported that the hypermethylation of the p15INK4B gene promoter was frequently observed in myelodysplastic syndromes (MDS), and that it may be associated with disease progression. An unanswered question is whether p15INK4B gene methylation is restricted to undifferentiated blastic cells, or whether differentiated cells such as granulocytes or erythrocytes of MDS origin also harbor this epigenetic alteration. In this study, we analyzed the methylation status of the p15INK4B gene in MDS by the methylation-specific PCR (MSP) method, which is more sensitive than Southern blotting. The bone marrow mononuclear cells (BM-MNCs) of 23 MDS patients were analyzed, and six of them showed p15INK4B methylation. Progenitor assay with methylcellulose medium was also performed in all patients. In two of the six patients with p15INK4B-methylated BM-MNCs, erythroid and/or non-erythroid colonies formed were subjected to molecular analysis. Colonies with and without p15INK4B methylation were detected in both patients. Furthermore, X-chromosome inactivation (XCI) pattern of each colony was simultaneously determined by MSP-based human androgen receptor gene analysis (HUMARA-MSP), and all p15INK4B-methylated colonies showed the same XCI pattern, which was dominant among the colonies, while p15INK4B-unmethylated colonies showed both patterns of XCI, in each of the two patients. We then examined the methylation status of the p15INK4B gene of granulocyte (PB-PMN) fractions from 10 patients with available peripheral blood cells. In all four patients with p15INK4B-methylated BM-MNCs, their PB-PMNs showed p15INK4B methylation. These results suggest that p15INK4B methylation in hematopoietic cells in MDS patients is restricted to the MDS clone but not necessarily to blast cells. [ABSTRACT FROM AUTHOR]

An inv(11)(q13.5;q25) inversion was previously identified in a 9-month-old male patient with complex cyanotic heart defects, altered lung lobation, symmetric liver, and abnormally lobulated spleen (polysplenia). This chromosomal rearrangement was inherited from the phenotypically normal father. We termed these regions DHTX-A (disrupted in heterotaxy) –A at 11q13.5 and DHTX-B at 11q25. Here, we report the isolation and characterization of the inversion breakpoints and the gene that is disrupted by the DHTX-A breakpoint. The putative DHTX is identical to the UVRAG gene, which was originally identified as a gene that complements the UV sensitivity of xeroderma pigmentosum complementation group C. The 4-kb mRNA was found to be encoded by a large gene, at least 300 kb long, composed of 15 exons. The function of the gene product remains largely unknown. However, the near central portion of the UVRAG protein is predicted to contain a coiled-coil domain, which has been implicated in mediating protein-protein interactions. Southern analyses and fluorescence in situ hybridization (FISH) revealed that the DHTX-A breakpoint in the patient and his father lies within the intron between exons 6 and 7 of UVRAG. Northern blot analysis indicated strong expression in human fetal and adult tissues and in mouse embryonic day-7 and adult tissues, respectively. Whole mount in situ hybridization also showed that the Uvrag gene is expressed in the presomite-stage embryo. Several hypotheses are discussed to explain the relationship between the chromosomal inversion and the accompanying phenotypes. [ABSTRACT FROM AUTHOR]

The human androgen-receptor gene (HUMARA) has been used for analysis of X chromosome inactivation (XCI) pattern because of a polymorphic short tandem repeat (STR) near the 5'-promoter region correlated with XCI. We introduce a novel method to analyze XCI pattern, named HUMARA methylation-specific PCR (HUMARA-MSP) assay, which analyzes methylation status of the HUMARA gene by bisulfite modification instead of a methylation-sensitive restriction enzyme. Although the original MSP method shows whether there is a methylated band or not, our HUMARA-MSP method identifies the patterns of methylated and unmethylated bands. Because this method identifies either unmethylated or methylated alleles in each PCR tube and shows opposite band patterns dependent on methylation status, we can assess the XCI pattern independently twice. This method can avoid false results by incomplete enzyme digestion and incomplete bisulfite modification will not affect the results. Extremely small quantities of samples, such as hematopoietic colonies, were also available for HUMARA-MSP assay. Because DNA modified by sodium bisulfite is also available for assessment of methylation status of other genes by setting specific primers for them, we performed the simultaneous assessment of clonality and aberrant hypermethylation of p15INK4B gene in myelodysplastic syndromes. These simultaneous assessments were easily possible and provided much information despite requiring only a small volume of DNA. The HUMARA-MSP assay may facilitate the analyses for pathogenesis of hematological disorders because of its simplicity, sensitivity and wide applicability. Leukemia (2000) 14, 207-212. [ABSTRACT FROM AUTHOR]

The proliferation and differentiation of cells are greatly influenced by their environment. Many growth factors and cytokines are reported to be environmental factors that affect the proliferation and differentiation of cells. Mechanical stress is also considered to influence these physiological reactions. The joint capsule, which is a part of the joint tissue, plays a very important role in the stability of the joint and in maintaining the intracapsular phenomenon. In patients with dislocated hip arthropathy, this capsule is involved in the weightbearing function by forming a sliding surface between the capsule and the femoral head articular cartilage. The surface of the tissue macroscopically shows cartilaginous change, which indicates cartilaginous differentiation caused by mechanical stress. We examined the cartilage-specific proteoglycan component, which is composed of cartilaginou matrix at the differentiation site. We investigated proteoglycan production, molecular size, and the gene expression of cartilaginous substrate. At the inner layer of the weightbearing area of the joint capsule, proteoglycan production was significantly higher than that of other noncartilaginous tissue. We also identified the gene expression of cartilaginous proteoglycan using the reverse transcription polymerase chain reaction (RT-PCR) method. [ABSTRACT FROM AUTHOR]

This paper deals with the characterization of mechanical properties of impacted morsellized cancellous allograft (IMCA) produced by dynamic compaction of allograft femoral heads ground by commercially available bone mills, i.e. rotating rasp and reciprocating type bone mills. Various ranges and profiles of particle size in the graft aggregates were obtained using these bone mills, and the effect of number of compaction as well as the distribution of particle sizes on the mechanical properties of IMCA under quasistatic compression and shear loading conditions was discussed. The morsellized cancellous allograft prepared by the reciprocating type bone mill showed a broad distribution of particle sizes, and gave IMCA superior mechanical properties to the graft with a more uniform size distribution, or prepared by the rotating rasp type bone mills. The increase of number of compaction also improved the mechanical properties of IMCA in compression. [ABSTRACT FROM AUTHOR]

X chromosome inactivation and polymorphism of the human androgen receptor (HUMARA) gene has been applied for analyzing the clonality of blood cells. In the present study, the clonal relationship was investigated between peripheral blood polymorphonuclear cells (PMNCs) and marrow progenitor cells and the origin of ringed sideroblasts in patients with refractory anemia with ring sideroblasts (RARS) by polymerase chain reaction (PCR) of HUMARA gene. The X-inactivation patterns of circulating PMNCs and T lymphocytes as well as individual granulocyte colonies grown in vitro from bone marrow cells were analyzed. The development of ringed sideroblasts in erythroid colonies by iron staining and their X-inactivation pattern were also examined. All three RARS patients showed monoclonal PMNCs. In granulocyte colonies, however, two different X-inactivation patterns were observed in all patients, indicating that non-clonal progenitor cells remained in the bone marrow. All erythroid colonies consisted of ringed sideroblasts exclusively showed one pattern dominant in those of PMNCs. Our findings suggest that non-clonal progenitor cells persist in some RARS cases, that erythroid progenitors show mosaicism, and that ringed sideroblasts may be derived from an abnormal clone involved in the pathogenesis of this disease. [ABSTRACT FROM AUTHOR]

Recent molecular studies have revealed that a 22q11 deletion is frequently detected in DiGeorge syndrome (DGS), velo-cardio-facial syndrome (VCFS), and conotruncal anomaly face syndrome (CTAFS). As one of the major clinical manifestations in these three syndrome is conotruncal cardiac malformation, we prospectively studied the frequency of a 22q11 deletion in a group of patients with conotruncal cardiac malformation. Fluorescence in situ hybridization (FISH) analyses using N25 (D22S75) DiGeorge Chromosome Region probe were performed on 64 patients with conotruncal cardiac malformation, who visited our clinic from October 1993 to January 1994. Of the 64 patients studied, a 22q11 deletion was detected in 5 patients (7.8%): 3 out of 30 patients with tetralogy of Fallot, one of three with interruption of the aortic arch, and one hemitruncus patient. No deletion was found in 16 patients with complete transposition of the great arteries, 8 with double outlet right ventricle and 2 with aortopulmonary window. In these five patients with 22q11 deletion, patient 1 was clinically diagnosed as having DGS, patients 2 and 3 had CTAFS, and patient 4 had VCFS. Patient 5 could not be dysmorphologically evaluated. It was noteworthy that all patients with a 22q11 deletion, except a non-evaluated patient, had some symptoms DGS, CTAFS or VCFS, and that we failed to identify a non-syndromic 22q11 deletion positive patients in the present series' of 64 patients. Conclusion. This study suggests that it is advisable to bear 22q11 deletion in mind when a patient with conotruncal cardiac anomalies has some other features of DGS, VCFS or CTAFS. [ABSTRACT FROM AUTHOR]

In single cells isolated from guinea-pig ileal smooth muscle, held under voltage clamp at −40 mV or −50 mV by patch pipette in the whole-cell recording mode, carbachol (CCh) evoked an oscillatory inward cationic current. The frequency of current oscillations increased with increasing CCh concentration. CCh-evoked current oscillations were followed very closely by oscillations in intracellular free Ca estimated from the Indo-1 signal, and were abolished by inclusion of EGTA in the pipette solution. Ryanodine and heparin, but not nifedipine, blocked the generation of current oscillations. CCh-evoked current oscillations were abolished upon withdrawal of extracellular calcium and restored upon its reintroduction. Inclusion of GTP[ γS] in the pipette solution caused the generation of an oscillatory inward current, which was blocked by ryanodine. The present results are consistent with the hypothesis that CChevoked cationic current is gated by activation of a G protein and is steeply dependent on [Ca], fluctuations in the release of Ca from stores during carbachol's action produce oscillations in [Ca] which cause similar oscillations in the cationic current. [ABSTRACT FROM AUTHOR]

A protein modification method has been developed for the production of human big endothelin (ET)-1. Production of a large quantity of big ET-1 by the method described here is expected to facilitate future experiments such as X-ray crystallography and nuclear magnetic resonance studies, aimed at understanding the tertiary structure of big ET-1 and its dynamics. The plasmid pETB-50 used for the synthesis carries the gene for a fusion protein consisting of 34-amino acid (aa) residues of an N-terminal portion of β-galactosidase and the 38-aa residues of big ET-1. The fusion protein ETB-50P contains an arginine residue in the big ET-1 portion at its second C-terminal site and three lysine residues including the C-terminal site in the β-galactosidase portion, all of which are susceptible to trypsin. Tryptic digestion of the fusion protein quantitatively produced big ET-1 (1-37), which is depleted in the C-terminal serine. However, a treatment of the fusion protein with 1,2-cyclohexanedione prior to tryptic digestion gave full-length big ET-1 with N,-N-(1,2-dihydroxycyclohex-1,2-ylene)-arginine. This modification was reversed to the intact arginine residue when the modified big ET-1 was incubated in 0.5 M TRIS-HCI buffer, pH 8.0. Consequently, a combination of such a reversible protein modification and tryptic digestion gave 1.74 mg of recombinant big ET-1 from 2.01 of culture broth. The procedure described here may be applied to produce other arginine-containing peptides from fusion proteins. [ABSTRACT FROM AUTHOR]

Human Namalwa cells were screened in serum-free medium and in 6-thioguanine, then fused with human lymphocytes from lymph nodes of lung adenocarcinoma cancer patients. Extensive testing using 14 lung cancer cell lines, 11 other cancer cell lines and 4 normal fibroblast lines identified monoclonal antibodies produced by 4 hybridoma clones that reacted specifically with lung adenocarcinoma cells. These monoclonal antibodies also reacted with lung adenocarcinoma tissues and not normal tissues or erythrocytes of any blood type. These hybridoma clones grew and stably secreted the antibodies in serum-free medium as well as in serum-containing medium. [ABSTRACT FROM AUTHOR]

In this paper, two time relaxation parameters are introduce to a thermal lattice BGK, model to make its Prandtl number controllable. The dependency of the Prandtl number on the two parameters is derived. Numerical measurement of the transport coefficients is used to demonstrate the validity of the method. Furthermore, two examples of convective heat transfer are calculated, with one to show the effectiveness, and the other to show the breakdown of the two-parameter formulation under different conditions. [ABSTRACT FROM AUTHOR]

The urethral syndrome is generally not associated clinically with urethral changes, however, histology often reveals inflammatory or degenerative alterations. The conventionally diagnosed urethral syndrome can certainly be considered to be a synonym of chronic urethritis in adult females. The remarkable histological lesions were rupture of elastic fibers in the submucosa and the vascular wall, and a decrease of acid mucopolysaccharides in each layer of the urethra, accompanied by lymphocytic infiltration. [ABSTRACT FROM AUTHOR]

In three-quarters of the patients with urethral syndrome, chronic urethritis was found endoscopically. Of the remainder some percentage could be said to have a psychosomatic disorder. Therefore, the patients with unexplained urethral syndrome were unexpectedly few. Although urethral syndrome attacks adult females of all ages, the incidence was highest in the middle age range. Against this, chronic bacterial cystitis was spread evenly over the entire age range. The urethra was rather narrow in women with urethritis, but of normal size in those without urethritis, even if they had urethral syndrome. A satisfactory result was obtained through the topical administration of steroid solution, even where general treatments, including the use of antibiotics, have failed. [ABSTRACT FROM AUTHOR]

The lattice gas automata (LGA) is a microscopic model recently introduced to the field of fluid dynamics. The lattice Boltzmann equation (LBE) is an alternative implementing scheme of the LGA, which is totally free of noise. The modeled fluid is called as the cellular automaton (CA) fluid. It is known that the pressure of the CA fluid contains a velocity dependent part. It reflects the simple and discrete nature of the model and either brings about unphysical oscillation in the flow energy or spoils the pressure field of the simulated flow. In this paper, we begin with a general introduction of the lattice gas models. Then we give a full description for such spurious terms and suggest a compensation method to eliminate the effect in the lattice Boltzmann model. Four numerical simulations are used as illustration for the effectiveness of this method. [ABSTRACT FROM AUTHOR]

The relationship between surface expression of the interleukin-2 receptor (IL-2R) and release of the soluble form of the receptor (sIL-2Rα or sCD25) was investigated with peripheral blood mononuclear cells (PBMCs) from individuals with rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE). The spontaneous release of sCD25 was significantly increased in PBMCs from RA patients and decreased in cells from SLE patients, compared with normal controls. However, the extent of sCD25 release from phytohaemagglutinin (PHA)-stimulated PBMCs did not differ between RA or SLE patients and normal controls. The serum concentration of sCD25 was significantly increased in SLE or RA patients compared with the normal controls. Whereas the surface expression of CD25 by unstimulated PBMCs did not differ among the three groups of subjects, this parameter was significantly reduced for PHA-stimulated PBMCs from RA patients relative to those from normal controls. The surface expression of CD25 showed a positive correlation with sCD25 release for PBMMCs from SLE patients under either basal or stimulated conditions. No such relation was apparent for cells from RA patients. The surface expression of IL-2Rβ (CD122) under basal or stimulated conditions was significantly reduced in PBMCs from RA or SLE patients, compared with cells from normal controls. Thus, the increased concentration of sCD25 in the serum of individuals with these autoimmune rheumatic diseases may result from two different mechanisms: an increase in the spontaneous release of sCD25 in RA, and reduced clearance of this protein in SLE. [ABSTRACT FROM AUTHOR]

Objective: Because eosinophils likely play important roles in the pathophysiology of allergic diseases, specific inhibitors of eosinophils may be desirable to treat such diseases. To evaluate the capacity of a novel compound, sulochrin, as an inhibitor of eosinophilic inflammation, we examined the effects of this compound on various effector functions of eosinophils.¶ Materials and methods: We examined the effects of sulochrin on degranulation of human eosinophils stimulated with platelet-activating factor (PAF) or Sepharose 4B beads coated with secretory IgA (sIgA) or IgG. The effects of sulochrin on other effector functions of human eosinophils, including superoxide anion (O− 2) production, leukotriene (LT) C4 release, and interleukin (IL)-8 production induced by sIgA-beads were also studied. Finally, using PAF and LTB4 as chemoattractants, we evaluated the potency of sulochrin to inhibit eosinophil migration in vitro and in vivo.¶ Results: Sulochrin inhibited EDN release by eosinophils stimulated with sIgA-beads, IgG-beads and PAF in a concentration-dependent manner; IC50 values were 0.75 μM, 0.30 μM and 0.03 μM. Eosinophil O− 2 production, LTC4 release, and IL-8 production were also inhibited by sulochrin. Furthermore, PAF-induced chemotaxis of human eosinophils and LTB4-induced chemotaxis of guinea pig eosinophils were abolished by 1 μM of sulochrin. Finally, sulochrin potently inhibited LTB4-induced infiltration of eosinophils into the skin of guinea-pig in vivo.¶ Conclusions: These results suggest that sulochrin is a potent inhibitor of various effector functions of eosinophils. Sulochrin and its derivatives may be useful in the development of therapeutic approaches for patients with allergic diseases. [ABSTRACT FROM AUTHOR]

A test tunnel for the study of airfoil performances under air/water two-phase flow condition has been designed and constructed. This facility will serve for a better understanding of the flow phenomena and characteristics of hydraulic machinery under gas/ liquid two-phase flow operating conditions. At the test section of the tunnel, a two-dimensional isolated airfoil or a cascade of airfoils is installed in a two-phase inlet flow with a uniform velocity (up to 10 m/s) and void fraction (up to 12%) distribution. The details of the tunnel structure and the measuring systems are described and the basic characteristics of the constructed tunnel are also given. As an example of the test results, void fraction distribution around a test airfoil is shown. [ABSTRACT FROM AUTHOR]

The c-MYB proto-oncogene encodes a transcription factor which plays an important role in hematopoiesis. We detected truncated c-MYB mRNA (2.0 kb) and c-Myb protein (55 kDa) in the TK-6 cell line, which was established from a patient with chronic myelogenous leukemia in T cell blast crisis. Mutated c-MYB cDNA clone (WTK-1) was isolated from a TK-6 cell cDNA library and sequenced in its entirety. Compared with the wild-type human c-MYB sequence, the WTK-1 sequence diverged at the 3' ends of exons 9. A termination codon was present as the second codon downstream from the point of divergence and was followed by a previously unknown rearranged sequence. The conceptual protein encoded by WTK-1 (Myb(TK-6)) comprises 402 amino acids and lacks the negative regulatory domain of the normal c-Myb, reminiscent of the activated form of Myb protein. Luciferase reporter assay in NIH3T3 cells showed that the expression vector encoding Myb(TK-6) stimulated Myb-regulated mim-1 promoter more effectively than that encoding wild-type human c-Myb, suggesting that Myb(TK-6) is functional as a transcription factor, and thus as a potential transforming protein. Southern blot and mutant allele-specific polymerase chain reaction analyses showed that the same rearrangement of the c-MYB gene in TK-6 was present in late, but not in early, specimens obtained from the patient, indicating that this mutation had been acquired during disease progression. [ABSTRACT FROM AUTHOR]

Charge acceleration during an intense light field application to solids attracts much attention as elementary processes in high-harmonic generation and photoelectron emission. For manipulating such attosecond dynamics of charge, carrier-envelope-phase (CEP: relative phase between carrier oscillation of light field and its envelope function) control has been employed in insulators, nanometal and graphene. In superconducting materials, collective control of charge motion is expected because of its strongly coherent nature of quasi-particles. Here we report that, in a layered organic superconductor, a non-linear petahertz current driven by a single-cycle 6 femtosecond near infrared field shows up as second harmonic generation (SHG), which is in contrast to the common belief that even harmonics are forbidden in the centrosymmetric system. The SHG represents a CEP sensitive nature and an enhancement near the superconducting temperature. The result and its quantum many-body analysis indicate that a polarized current is induced by non-linear acceleration of charge, which is amplified by superconducting fluctuations. This will lead to petahertz functions of superconductors and of strongly correlated systems. Here the authors show second harmonic generation (SHG) from a centrosymmetric organic superconductor κ-(BEDT-TTF)2Cu[N(CN)2]Br. They find unusual temperature dependence and CEP-sensitive nature of the SHG which are explained in terms of nonlinear current. [ABSTRACT FROM AUTHOR]

Focuses on myelodysplastic syndrome with unbalanced t(1;7) after severe aplastic anemia in childhood treated with cyclosporin A and recombinant human granulocyte colony-stimulating factor. Pathogenesis of monosomy 7.

Describes the characterization of myelodysplastic syndrome(MDS) by frequent evolution to acute myelogenous leukemia. Role of P15[sup INK4B] gene in clonal expression in MDS; Analysis of the expression level of DNA methyltransferase activity in human cancers; Extraction of DNA and total RNA from cell lines.

Isolated hepatocytes from male Japanese eel at various stages of human chorionic gonadotropin (HCG)-induced spermatogenesis were cultured in the presence of estradiol-17ß (E2). Hepatic vitellogenin (VTG) production in response to E2 increased during testicular development. This indicates that VTG production is influenced by gonadal maturity not only in females but also in males, where serum levels of 11-ketotestosterone (11KT) increase during testicular development. Recently, it has been confirmed that significant levels of 11KT are detected in maturing female eel. Therefore, in a next experiment, effect of 11KT on E2-induced VTG production was examinedin vitro. As a result, in both male and female hepatocytes, 11KT enhanced E2-induced VTG production, although 11KT alone failed to induce VTG production. [ABSTRACT FROM AUTHOR]

Reports on the study conducted on the oncogene BCL10 gene in lymphoma with both RNA and DNA. Process of the cloning of BCL10 gene; Amplification of the RNA from follicular lymphomas which are involvedwith BCL10 gene; Findings related to BCL10 RNA clones with post-transcriptional sequence.

Cites a study conducted by the authors to determine the relationship between methylation of the P15 gene and ectopic expression of the EVI-1 gene in myelodysplastic syndromes (MDS). Molecular abnormalities found in MDS; Suppression of the P15 gene by the ectopic expression of the EVI-1 gene.

Presents a letter to the editor regarding the splice site mutation in the hepatocyte nuclear fator-1β gene associated with maturiy-onset diabetes of the young, renal dysplasia and bicornuate uterus, previously published in "Diabetologia."

Focuses on the treatment of myelodysplastic syndromes (MDS). Ultimate survival rate of the disease; Treatment used for adult cases of MDS.

Discusses the results of a study on genotype-phenotype correlations in patients with distal 10q monosomy. Evidence suggesting that genes for urinary development may reside in the approximately 20 cM region distal to D10S186 and that a novel gene for genital development resides in the approximately 10 cM region distal to D10S1248; Need for more studies to determine if these genes for genitourinary development are identical.