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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 and was required for DR6-induced… More >

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