AFG3L2 Antibody (K-15): sc-84686

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AFG3L2 (ATPase family gene 3-like 2), also known as Paraplegin-like protein, is a multi-pass membrane metalloprotease that contains one AAA (ATPase associated with diverse cellular activities) domain, a zinc-dependent binding motif, an RNA-binding region and an ATP/GTP binding site. Localizing to the mitochondrial membrane, AFG3L2 is ubiquitously expressed with highest expression levels in skeletal muscle and heart. AFG3L2 shares 69% similarity with the yeast Afg3 protein and 49% similarity with Paraplegin, a protein of mitochondria that is thought to be involved in signal transduction and chaperone-like activities. In mitochondria, AFG3L2 forms a complex with Paraplegin that is believed to regulate essential protein quality control. Mutations in the gene encoding either one of these proteins can result in hereditary spastic paraplegia, a degenerative spinal cord disorder that is characterized by muscle spasms, stiffness in the legs and, in some cases, incontinence.