Regulating the PO4 and TiO6 Polyhedral Building Blocks in TiP2O7 Boosts the Potassium Ion Diffusion Kinetics.

Achieving fast and durable potassiation/depotassiation of anode materials for potassium ion batteries (PIB) still remains an elusive yet fascinating goal. Herein, we challenge the conventional wisdom in synthesizing the TiP2O7 superstructure and report a nanocarbon coating on TiP2O7 (TiP2O7/C) using layered MXene as a Ti source to realize an effective tuning in the TiO6 and PO4 building blocks for boosting the K+ diffusion kinetics in PIB. Experimental investigations coupled with systematic theoretical simulations indicate that the interface interaction between TiP2O7 and coated nanocarbon could induce internal adjustment in individual Ti-O bonding and relieve the local distortions of TiO6 octahedra, which endows the TiP2O7/C with favorable regulation in a K+ hopping manner and significantly reduces the K+ diffusion barrier via the diffusion propagation along PO4 blocks with dominant coordination between O/P and K+. Consequently, the TiP2O7/C anode could retain 230 mA h g-1 even after 2200 long-term cycles with an ultralow degradation rate of 0.005%.

Heat shock factor 4 regulates lysosome activity by modulating the αB-crystallin-ATP6V1A-mTOR complex in ocular lens.

BACKGROUND: Germline mutations in heat shock factor 4 (HSF4) cause congenital cataracts. Previously, we have shown that HSF4 is involved in regulating lysosomal pH in mouse lens epithelial cell in vitro. However, the underlying mechanism remains unclear. METHODS: HSF4-deficient mouse lens epithelial cell lines and zebrafish were used in this study. Immunoblotting and quantitative RT-PCR were used for expression analysis. The protein-protein interactions were tested with GST-pull downs. The lysosomes were fractioned by ultracentrifugation. RESULTS: HSF4 deficiency or knock down of αB-crystallin elevates lysosomal pH and increases the ubiquitination and degradation of ATP6V1A by the proteasome. αB-crystallin localizes partially in the lysosome and interacts solely with the ATP6V1A protein of the V1 complex of V-ATPase. Furthermore, αB-crystallin can co-precipitate with mTORC1 and ATP6V1A in GST pull down assays. Inhibition of mTORC1 by rapamycin or siRNA can lead to dissociation of αB-crystallin from the ATP6V1A and mTORC1complex, shortening the half-life of ATP6V1A and increasing the lysosomal pH. Mutation of ATP6V1A/S441A (the predicted mTOR phosphorylation site) reduces its association with αB-crystallin. In the zebrafish model, HSF4 deficiency reduces αB-crystallin expression and elevates the lysosomal pH in lens tissues. CONCLUSION: HSF4 regulates lysosomal acidification by controlling the association of αB-crystallin with ATP6V1A and mTOR and regulating ATP6V1A protein stabilization. GENERAL SIGNIFICANCE: This study uncovers a novel function of αB-crystallin, demonstrating that αB-crystallin can regulate lysosomal ATP6V1A protein stabilization by complexing to ATP6V1A and mTOR. This highlights a novel mechanism by which HSF4 regulates the proteolytic process of organelles during lens development.

A Novel Cx50 Insert Mutation from a Chinese Congenital Cataract Family Impairs Its Cellular Membrane Localization and Function.

Mutations in GJA8 are associated with hereditary autosomal dominant and recessive cataract formation. In this study, a novel insert mutation in GJA8 was identified in a Chinese congenital cataract family and cosegregated with the disease in this pedigree. This insert mutation introduces five additional amino acid residues YAVHY after histidine at the 95 site (p.H95_A96insYAVHY) within the second transmembrane (TM2) domain of Cx50 protein (Cx50-insert). Ectopic expression of Cx50-insert protein impairs the hemichannel functions and gap junction activity compared to wild-type Cx50 protein in human lens epithelial cells. Cx50-insert proteins were mislocated from cytoplasmic membrane to endoplasmic reticulum and lysosome. In mouse lens tissue, our results showed that Cx50 predominant expresses in epithelial cells and fiber cells at the transition zone of lens hinting its roles in lens differentiation. Taken together, these data suggest that the novel insert mutation in the TM2 domain of Cx50 protein, which impairs its trafficking to the cell membrane and gap-junction function, is associated with the cataract formation in this Chinese pedigree.

[Dual-planar osteotomy and lateral column anatomic locking plate fixation in the treatment of adult cubitus varus deformity].

Objective: To explore the effectiveness of distal humeral dual-planar osteotomy followed with distal humeral lateral column anotomical locking plate fixation in the treatment of adult cubitus varus deformity combined with flexion restriction. Methods: The clinical data of 6 adult patients with cubitus varus deformity and flexion restriction treated with dual-planar osteotomy and internal fixation between April 2012 and September 2014 were reviewed. There were 3 males and 3 females with an average age of 22.2 years (range, 18-35 years). All the patients had history of elbow injury in childhood, the age being injured was 6.5 years on average (range, 2-13 years). There was no nerve injury, elbow strength weak, or unstable complications before admission. The preoperative elbow flexion was (107.50±5.24)°, elbow extension was (-12.17±3.48)°; the carring angle was (-19.50±4.46)°, the contralateral elbow carrying angle was (11.50±2.67)°; the length of humerus was (0.42±0.38) cm shorter than the unaffected humerus. The postoperative carrying angles, elbow flexion and extension, and humerus length were recorded. The modified Laupattarakasem criteria in which the comparison of bilateral elbow range of motion excluded was used to evaluate the results. Results: Primary healing of incision was got in all patients and there was no early postoperative complications. All the patients were followed up 19-27 months (mean, 20.8 months). During the follow-up, there was no complication such as loosening and breakage of the internal fixators and all fractures were healed within 3 months after operation. At last follow-up, the elbow flexion was (124.17±5.85)°, the elbow extension was (0.83±2.04)°, the carrying angle was (12.00±4.19)°, and the humerus shortening was (1.88±0.45) cm, all showing significant difference when compared with preoperative ones ( t=-6.742, P=0.001; t=-11.068, P=0.000; t=-20.400, P=0.000; t=-13.914, P=0.000). According to the modified Laupattarakasem criteria, 1 case was excellent, 4 were good, and 1 was fair with an excellent and good rate of 83.3%. Conclusion: Normal carrying angle can be restored and elbow flexion can be increased by coronal closing wedge and sagittal trapezoid dual-planar osteotomy, single lateral anatomical locking plate fixation is available for early mobilization.

[Lateral closing wedge osteotomy and modified cross pinning with external tension band fixation in the treatment of cubitus varus deformity in childrenLateral closing wedge osteotomy and modified cross pinning with external tension band fixation in the treatment of cubitus varus deformity in children].

OBJECTIVE: To explore the therapeutic effects of distal humeral lateral closing wedge osteotomy followed by modified pinning combined with external tension band fixation in the treatment of cubitus varus deformity in children. METHODS: Total 26 adult patients with cubitus varus deformity were treated by operation from March 2011 to June 2015, 15 patients were boys and the other 11 patients were girls, ranging in age from 4 to 13 years, with an average of 7.8 years. The cubitus varus angel ranged from 11 degrees to 24 degrees, with a mean(17.50±6.73) degrees, 3 patients complicated more than 10 degrees constriction of flexion. Lateral closing wedge osteotomy retaining the medial 3 to 4 mm intact cortex by lateral elbow approach was applied in these 26 patients. The wedge defect were closed and fixed by crossing pinning. The lateral column compression was achieved with external tension band(the crossing pins were bended laterally and the pin ends were hooked mutually). The pre-operative, post-oparetive and contralateral carrying angles were compared and Laupattarakasem criteria was used to evaluate the results at follow-up. RESULTS: All the patients got bony union 2 months after operation and there was no infection or nerve palsy. The average follow-up period was 18.8 months (ranged, 13 to 29 months). The carrying angle was restored to(11.50±3.17) degrees(ranged, 8 to 14 degrees). According to the Laupattarakasem evaluation criteria, 14 patients got an excellent result, 13 good and 1 fair. CONCLUSIONS: Normal carrying angle and elbow flexion could be restored by lateral closing wedge osteotomy, and stable fixation could be achieved with crossing pinning and external tension band, which is available for early mobilization.