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Hermansky-Pudlak syndrome (HPS) is characterized by defects of multiple tissue-specific lysosome-related organelles (LROs), typically manifesting with oculocutaneous albinism or ocular albinism, bleeding tendency, and in some cases with pulmonary fibrosis, inflammatory bowel disease or immunodeficiency, neuropsychological disorders. Eleven HPS subtypes in humans and at least 15 subtypes in mice have been molecularly identified. Current understanding of the underlying mechanisms of HPS is focusing on the defective biogenesis of LROs. Compelling evidences have shown that HPS protein-associated complexes (HPACs) function in cargo transport, cargo recycling, and cargo removal to maintain LRO homeostasis. Further investigation on the molecular and cellular mechanism of LRO biogenesis and secretion will be helpful for better understanding of its pathogenesis and for the precise intervention of HPS.

Aim To explore whether self‐efficacy has any positive or negative mediating effects between family functioning and quality of life among elders with chronic diseases. Design A cross‐sectional study. Methods Questionnaires were collected from 516 community‐dwelling elderly individuals with chronic diseases using a convenience sampling method. The questionnaires included the Self‐efficacy for Managing Chronic Disease Six‐Item Scale, the Family Adaptation Partnership Growth Affection Resolve Index and the MOS 36‐Item Short Form Health Survey. Results Family functioning and self‐efficacy impacted the quality of life of community‐dwelling elderly individuals with chronic diseases. Family functioning was mediated by self‐efficacy and had an indirect impact on quality of life. The mediating effect accounted for 62.50% of the total effect.

Aim The study was to explore the significant influence of an English nursing course in nursing postgraduate internationalization education. Design A cross‐sectional study. Method The research object included three grades (a total of 18) of nursing postgraduate students in the Nursing School of Yangzhou University. A standardized four‐section questionnaire designed by the authors was applied to the survey. Results 88.89% were satisfied with the course design and application of the English nursing course, and the scores for all 6 items were above the average, but only 44.4% of the postgraduate students understood the course completely. In teaching design and content aspect, 88.9%–94.4% postgraduate nurses felt that the course learning requirements were clear and could improve their knowledge and capacity for scientific research. All of the postgraduate nurses were identified with the teacher's moral and academic characters. Conclusions To establish English nursing courses for nursing postgraduate students is beneficial for postgraduate internationalization education.

Three polymers [Zn2(p-CPIDA)(OH)]n (1), [Zn2(p-HCPIDA)2(bipy)(H2O)4]·2H2O (2), and [Zn(m-HCPIDA)(bipy)]n (3) [p/m-H3CPIDA = N-(4/3-carboxyphenyl) iminodiacetic acid, bipy = 4, 4′-bipyridine] were hydrothermally synthesized and characterized by single-crystal X-ray diffraction, X-ray powder diffraction patterns (XPRD), FTIR spectroscopy, and fluorescence analyses. Compound 1 displays an interesting three-dimensional framework consisting of novel paddle wheel Zn8O10C2 units, which are further linked by p-CPIDA3– ligands with versatile coordination modes. In 2, the structural motifs formed by terminal p-HCPIDA2– ligands and ZnII ions are bridged by ancillary bipy to give a zero-dimensional Z-shaped dimer. Compound 3 has an infinite zigzag chain linked by bipy, in which terminal m-HCPIDA2– ligands alternately run along the chain like flags. Compounds 2 and 3 are further connected by hydrogen bonding and π···π stacking interactions to result in 3D supramolecular frameworks. Moreover, compounds 1–3 exhibit intense solid state fluorescent emissions at room temperature.

Many studies have shown that adenosine triphosphate (ATP), as a neurotransmitter, is involved in plastic changes of synaptic transmission in central nervous system. In the present study, we tested whether extracellular ATP can induce long-term potentiation (LTP) of C-fiber-evoked field potentials in spinal dorsal horn. The results showed the following: (1) ATP at a concentration of 0.3 mM induced spinal LTP of C-fiber-evoked field potentials, lasting for at least 5 h; (2) spinal application of 2',3'-O-(2,4,6-trinitrophenyl)adenosine-5-triphosphate (TNP-ATP; an antagonist of P2X(1-4) receptors), but not pyridoxal-phosphate-6-azophenyl-2',4'-disulphonic acid (PPADS; an antagonist of P2X(1,2,3,5,7) receptors), 30 min before ATP blocked ATP-induced LTP, indicating that ATP may induce spinal LTP by activation of P2X(4) receptors; (3) at 60 min after LTP induction the level of phospho-p38 mitogen-activated protein kinase (p-p38 MAPK) was significantly elevated and at 180 min after LTP the number of P2X(4) receptors increased significantly; both p-p38 and P2X(4) receptors were exclusively co-located with the microglia marker, but not with neuronal or astrocyte marker; (4) spinal application of TNP-ATP but not PPADS prevented p38 activation; (5) spinal application of SB203580, a p38 MAPK inhibitor, prevented both spinal LTP and the upregulation of P2X(4) receptors. The results suggested that ATP may activate p38 MAPK by binding to intrinsic P2X(4) receptors in microglia, and subsequently enhance the expression of P2X(4) receptors, contributing to spinal LTP.

Previous works have shown that activation of extracellular signal-regulated kinase (ERK)/cAMP response element binding protein (CREB) pathway is essential for long-term potentiation (LTP) in hippocampus. In the present study, the role of the ERK/CREB pathway in LTP of C-fiber evoked field potentials in spinal dorsal horn, which is relevant to pathologic pain, was investigated in adult rats. Western blotting analysis showed that the protein level of phosphorylated ERK (p-ERK) in ipsilateral spinal dorsal horn was transiently increased after LTP induction, starting at 15 min and returning to control at 60 min after tetanic stimulation and that the protein level of p-CREB increased at 30 min, persisting for at least 3 hr after LTP induction. Double immunofluorescence staining showed that p-ERK and p-CREB were only located in neurons but not in glial cells in the spinal dorsal horn after LTP induction. More importantly, we found that spinal application of PD 98059 (100 microM), a selective MEK inhibitor, at 30 min before tetanic stimulation blocked LTP induction and prevented the increase in p-ERK and p-CREB in spinal dorsal horn. When applied 15 min after LTP induction, PD98059 reversed established LTP. The drug, however, did not affect the spinal LTP, when applied at 30 min after LTP. Our results suggested that activation of ERK/CREB pathway in spinal dorsal neurons is necessary for induction and maintenance of long-term potentiation of the C-fiber evoked field potentials.