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    ARTICLE

    SOD1G93A Induces a Unique PSAP-Dependent Mitochondrial Apoptosis Pathway via Bax–Bak Interaction

    HAN NIU1,#, XIN CHEN1,#, XUEQI FU1, JINGTIAN ZHANG1, GUODONG LI4, YUXIANG WANG1, JIAYUE SONG1, XUETING MA1, CHEN HU5, XUEMIN XU3, FUQIANG ZHANG2,*, LINLIN ZENG1,*

    BIOCELL, Vol.45, No.4, pp. 963-970, 2021, DOI:10.32604/biocell.2021.015297

    Abstract Amyotrophic lateral syndrome (ALS) is a progressive degenerative disorder characterized by motor neuron death and axon degeneration. Mitochondrial dysfunction plays a key role in the pathogenesis of ALS, the mechanism of which remains poorly understood. The B-cell lymphoma-2 (Bcl-2) family of proteins that control and mediate mitochondrial function and apoptosis, including the pro-apoptotic members Bcl2-Associated X (Bax), are involved in ALS development. The death receptor 6 (DR6) regulates motor neuron death in ALS, and DR6 antibodies can prevent axon degeneration and motor neuron damage by blocking DR6. Previous studies demonstrated that PSAP localized to mitochondria More >

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