Anti-AFG3L2

0.5mg/ml if reconstituted with 0.2ml sterile DI water. AFG3L2 is the catalytic subunit of the m-AAA protease, an ATP-dependent proteolytic complex of the mitochondrial inner membrane that degrades misfolded proteins and regulates ribosome assembly. In humans, it is encoded by the AFG3L2 gene. This gene encodes a protein localized in mitochondria and closely related to paraplegin. The paraplegin gene is responsible for an autosomal recessive form of hereditary spastic paraplegia. And this gene is a candidate gene for other hereditary spastic paraplegias or neurodegenerative disorders as well as spastic ataxia-neuropathy syndrome. Protein function: ATP-dependent protease which is essential for axonal development. [The UniProt Consortium]