ARC (also known as NOL3 - Nucleolar protein 3) has two isoforms. Isoform 1 is involved in RNA splicing. Isoform 2 functions as an apoptosis repressor that blocks multiple modes of cell death. ARC inhibits extrinsic apoptotic pathways two different ways. The first is by interacting with FAS and FADD upon FAS activation blocking death-inducing signaling complex (DISC) assembly. The second way is by interacting with CASP8 in a mitochondria localization-and phosphorylation-dependent manner, which in turn limits the amount of soluble CASP8 avaliable for DISC-mediated activation. ARC also inhibits intrinsic apoptotic pathways in response to a wide range of stresses through interactions with BAX. This results in BAX inactivation and prevents mitochondrial dysfunction and release of pro-apoptotic factors. Mutations affecting the gene can results in myoclonus, familiam cortical.
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Protein Aliases: apoptosis repressor ARC; Apoptosis repressor with CARD; apoptosis repressor with caspase recruitment domain (CARD); Muscle-enriched cytoplasmic protein; Myp; NOL3; Nop30; Nucleolar protein 3; nucleolar protein 3 (apoptosis repressor with CARD domain); Nucleolar protein of 30 kDa
Gene Aliases: ARC; B430311C09Rik; FCM; MYC; MYP; NOL3; NOP; NOP30