Immortalized myogenic cells from congenital muscular dystrophy type1A patients recapitulate aberrant caspase activation in pathogenesis: a new tool for MDC1A research

BACKGROUND: Congenital muscular dystrophy Type 1A (MDC1A) is a severe, recessive disease of childhood onset that is caused by mutations in the LAMA2 gene encoding laminin-alpha2. Studies with both mouse models and primary cultures of human MDC1A myogenic cells suggest that aberrant activation of cell death is a significant contributor to pathogenesis in laminin-alpha2-deficiency. METHODS: To overcome the limited population doublings of primary cultures, we generated immortalized, clonal lines of human MDC1A myogenic cells via overexpression of both CDK4 and the telomerase catalytic component (human telomerase reverse transcriptase (hTERT)). RESULTS: The immortalized MDC1A myogenic cells proliferated indefinitely when cultured at low density in high serum growth medium, but retained the capacity to form multinucleate myotubes and express muscle-specific proteins when switched to low serum medium. When cultured in the absence of laminin, myotubes formed from immortalized MDC1A myoblasts, but not those formed from immortalized healthy or disease control human myoblasts, showed significantly increased activation of caspase-3. This pattern of aberrant caspase-3 activation in the immortalized cultures was similar to that found previously in primary MDC1A cultures and laminin-alpha2-deficient mice. CONCLUSIONS: Immortalized MDC1A myogenic cells provide a new resource for studies of pathogenetic mechanisms and for screening possible therapeutic approaches in laminin-alpha2-deficiency

Hierarchical Porous Carbon Materials Prepared by Direct Carbonization of Metal-Organic Frameworks as an Electrode Material for Supercapacitors

© 2020 Korean Chemical Society and Wiley-VCH GmbH. We report the synthesis and characterization of hierarchical porous carbons (HPCs) prepared by direct carbonization of zinc-based metal-organic frameworks (MOFs), and their electrochemical performance as an electrode material for supercapacitors. All the HPCs showed high porosity (Brunauer-Emmett-Teller (BET) surface areas 1000-1820 m(2)/g) with micro-, meso-, and macropores. The HPC-based electrodes exhibited a high-specific capacitance in the range of 164-203 F/g (scan rate: 10 mV/s), which suggests that these porous carbons may be useful for fabricating supercapacitors. Among the HPCs, HPC-4 with the largest surface area as well as with similar to 1% nitrogen content exhibited the highest specific capacitance, which is comparable with those of other reported carbon materials. This work suggests that the hierarchical porosity and nitrogen doping in HPCs may enhance their conductivity and specific capacitance.11Nsciescopuskc

Tandem catalysis with a bifunctional site-isolated Lewis acid–Brønsted base metal–organic framework, NH2-MIL-101(Al)

A metal-organic framework (MOF), NH2-MIL-101(Al), which acts as a bifunctional, site-isolated Lewis acid-Bronsted base heterogeneous catalyst, catalyzes a tandem Meinwald rearrangement-Knoevenagel condensation reaction with remarkable substrate selectivity.open119589sciescopu

Metal-Ion Metathesis in Metal-Organic Frameworks: A Synthetic Route to New Metal-Organic Frameworks

A porous metalorganic framework, Mn(H3O)(Mn4Cl)(3)(hmtt)(8)] (POST-65), was prepared by the reaction of 5,5',10,10',15,15'-hexamethyltruxene-2,7,12-tricarboxylic acid (H(3)hmtt) with MnCl2 under solvothermal conditions. POST-65(Mn) was subjected to post-synthetic modification with Fe, Co, Ni, and Cu according to an ion-exchange method that resulted in the formation of three isomorphous frameworks, POST-65(Co/Ni/Cu), as well as a new framework, POST-65(Fe). The ion-exchanged samples could not be prepared by regular solvothermal reactions. The complete exchange of the metal ions and retention of the framework structure were verified by inductively coupled plasmaatomic emission spectrometry (ICP-AES), powder X-ray diffraction (PXRD), and BrunauerEmmettTeller (BET) surface-area analysis. Single-crystal X-ray diffractions studies revealed a single-crystal-to-single-crystal (SCSC)-transformation nature of the ion-exchange process. Hydrogen-sorption and magnetization measurements showed metal-specific properties of POST-65