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Limb‐girdle muscular dystrophy recessive 1 (LGMDR1), previously known as LGMD2A, is a specific LGMD caused by a gene mutation encoding the calcium‐dependent neutral cysteine protease calpain‐3 (CAPN3). In our study, the compound heterozygosity with two missense variants c.635 T > C (p.Leu212Pro) and c.2120A > G (p.Asp707Gly) was identified in patients with LGMDR1. However, the pathogenicity of c.635 T > C has not been investigated. To evaluate the effects of this novel likely pathogenic variant to the motor system, the mouse model with c.635 T > C variant was prepared by CRISPR/Cas9 gene editing technique. The pathological results revealed that a limited number of inflammatory cells infiltrated the endomyocytes of certain c.635 T > C homozygous mice at 10 months of age. Compared with wild‐type mice, motor function was not significantly impaired in Capn3 c. 635 T > C homozygous mice. Western blot and immunofluorescence assays further indicated that the expression levels of the Capn3 protein in muscle tissues of homozygous mice were similar to those of wild‐type mice. However, the arrangement and ultrastructural alterations of the mitochondria in the muscular tissues of homozygous mice were confirmed by electron microscopy. Subsequently, muscle regeneration of LGMDR1 was simulated using cardiotoxin (CTX) to induce muscle necrosis and regeneration to trigger the injury modification process. The repair of the homozygous mice was significantly worse than that of the control mice at day 15 and day 21 following treatment, the c.635 T > C variant of Capn3 exhibited a significant effect on muscle regeneration of homozygous mice and induced mitochondrial damage. RNA‐sequencing results demonstrated that the expression levels of the mitochondrial‐related functional genes were significantly downregulated in the mutant mice. Taken together, the results of the present study strongly suggested that the LGMDR1 mouse model with a novel c.635 T > C variant in the Capn3 gene was significantly dysfunctional in muscle injury repair via impairment of the mitochondrial function. [ABSTRACT FROM AUTHOR]

A PEGylated 96-well polystyrene (PS)microplate was first introducedfor applications in high-throughput screening for selective bloodtyping to minimize the risks in blood transfusions. Herein, we presenta hemocompatible PS 96-well microplate with adjustable PEGylated hyperbranchbrush coverage prepared by ozone pretreated activation and thermallyinduced surface PEGylation. The grafting properties, hydration capacity,and blood compatibility of the PEGylated hyperbrush immobilized PSsurfaces in human blood were illustrated by the combined chemicaland physical properties of the surface, and the dependence of thespecific absorption of human plasma fibrinogen onto the PEGylatedsurfaces on the grafting density was analyzed by monoclonal antibodies.The surface coverage of PEGylated brushes plays a major role in thebioadhesive properties of modified PS microplates, which in turn controlthe level of agglutination sensitivity in blood typing. The bioadhesiveresistance toward proteins, platelets, and erythrocytes in human wholeblood showed a correlation to the controlled hydration propertiesof the PEGylated hyperbrush-modified surfaces. Therefore, we suggestedthat the surface coverage of PEGylated hyperbrushes on PS surfacescan increase the sensitivity of cross-matching blood agglutinationby up to 16-fold compared to that of the conventional 96-well virginPS due to the regulated biorecognition of hematocrit and antibodiesof the PEGylated hyperbrush-modified surfaces. [ABSTRACT FROM AUTHOR]

RopB is a quorum-sensing regulator that binds to the SpeB-inducing peptide (SIP) under acidic conditions. SIP is known to be degraded by the endopeptidase PepO, whose transcription is repressed by the CovR/CovS two-component regulatory system. Both SIP-bound RopB (RopB-SIP) and SIP-free RopB (apo-RopB) can bind to the speB promoter; however, only RopB-SIP activates speB transcription. In this study, we found that the SpeB expression was higher in the ropB mutant than in the SIP-inactivated ( SIP *) mutant. Furthermore, the deletion of ropB in the SIP * mutant derepressed speB expression, suggesting that apo-RopB is a transcriptional repressor of speB Up-regulation of PepO in the covS mutant degraded SIP, resulting in the down-regulation of speB We demonstrate that deleting ropB in the covS mutant derepressed the speB expression, suggesting that the speB repression in this mutant was mediated not only by PepO-dependent SIP degradation but also by apo-RopB. These findings reveal a crosstalk between the CovR/CovS and RopB-SIP systems and redefine the role of RopB in regulating speB expression in group A Streptococcus ., (© 2023 Chiang-Ni et al.)

Objective To observe the effects of Banxia Xiexin Decoction (BXD) containing ser- um on proliferation, invasion and metastasis of in vitro human gastric cancer peritoneum cell strain GC9811-P (which has high metastatic potential). Methods BXD containing serum was prepared. GC9811-P cells were inoculated in the E-Plate 96 and CIM Plate 16, and then 0, 25, 50, 100 μL/mL BXD containing serums were added respectively. Meanwhile, GC9811-P cells were stained by Diff-Quik stai- ning method. Inhibition of BXD containing serum on cell index (CI) for proliferation of GC9811-P cells, invasion and metastasis were observed by real time cellular analysis (RTCA) and Diff-Quik staining method. Results BXD containing serum could obviously inhibit the proliferation of GC9811-P cells. The Cl approximated to 0 after 70 h. Most stained Diff-Quik cells died. Cell migration curve showed that 25, 50, 100 μL/mL BXD containing serums could obviously inhibit the capacities for cell migration of GC9811-P cells in concentration dependent manner. The number of migration cells was reduced more obviously, as com- pared with 0 μL/mL BXD containing serum (P <0. 05). Conclusion BXD containing serums could inhibit the proliferation, invasion and metastasis of GC9811-P cells, which might be associated with blocking peritoneal metastasis of gastric cancer.

Objective: To analyze the characteristics of pulmonary function and the clinical significance of non-specific pattern (NSP)., Methods: A total of 1933 pulmonary function tests of adult patients were analyzed, and those with NSP were selected. The pulmonary function test results, clinical diagnosis and radiological manifestations were analyzed. Normal distribution data were compared by t test, while non-normal distribution data were compared by Mann-Whitney U test, and χ(2) test was used to compare ratios., Results: There were 61 patients in the NSP group and 1017 in the control group. The BMI of the 2 groups was (24.5 ± 4.6) and (24.5 ± 3.8) kg/m(2), respectively, being not significantly different (t = 0.008, P > 0.05). The age was 64 (49-74) years and 56 (42-70) years, and the smoking index was 0.4 (0-20) and 0 (0-10), respectively, showing no significant differences (Z values were -2.209 and -2.571, respectively, all P < 0.05). In the NSP group, FEV1 was 69% (66%-73%) predicted, FVC 75% (70%-77%) predicted, FEV1/FVC 75% (73%-78%), RV 118% (105%-145%) predicted, and TLC 86% (82%- 93%) predicted, which were significantly different as compared to those of the control group [95% (87%-104%), 98% (90%-106%), 79% (76%-84%), 101% (88%-114%) and 94% (88%-102%), respectively],(Z values are -13.059--5.185, all P < 0.05). RV/TLC was (52 ± 11)% in the NSP group and (39 ± 9)% in the control group, the difference being significant (t = -10.351, P < 0.05). The decreased TLC indicated restricted ventilation, while the increased RV and RV/TLC indicated air trapping. The clinical diagnosis of NSP included obstructive and restrictive diseases, some of which showed severe radiological abnormalities, but there were 31 patients without pulmonary lesions., Conclusions: Age and smoking, but not obesity, may play a role in NSP.NSP has characteristics of obstructive and restrictive ventilation defects, but does not associate with particular diseases, thus having limited clinical significance.

Objective: To evaluate the frequency of sensitization to Aspergillus antigens and the prevalence of allergic bronchopulmonary aspergillosis (ABPA) in asthmatic patients., Methods: Two hundred consecutive non-smoking outpatients with asthma (≥ 18 years) underwent skin testing with aeroallergens, peripheral eosinophil counting, measurements of total serum IgE level and specific IgE against Aspergillus fumigatus, radiologic investigations and pulmonary function tests., Results: Eleven patients (5.5%) had a positive skin reactivity to Aspergillus antigens. Five of these 11 patients (45.5%) met the diagnostic criteria of ABPA, an overall prevalence of 2.5% (5/200). There were 2 males and 3 females, aging from 19 to 62 years, with a disease duration from 15 to 40 years. All of the patients had asthmatic symptoms such as cough and wheeze. Moderate to severe obstructive ventilatory defect was found in 4 patients. Total serum IgE levels and specific IgE against Aspergillus fumigates were elevated significantly in all the patients, and elevated eosinophil count was found in 3 patients. Three cases were diagnosed as ABPA-CB because of the presence of central bronchiectasis on HRCT., Conclusions: The prevalence of ABPA in Chinese patients with asthma was underestimated. Clinical features of ABPA were similar to asthma alone, but with longer duration and more severe lung function defect. Sensitization to Aspergillus, increased eosinophils and total serum IgE levels were important indicators for the diagnosis of ABPA.