Proteintech, 68517-1-Ig-100uL, AFG3L2 Monoclonal antibody

68517-1-Ig targets AFG3L2 in WB, IF/ICC, FC (Intra), ELISA applications and shows reactivity with human, mouse, rat, pig, rabbit samples.
Background Information
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(PMID:17101804). Human AFG3L2 is an 80-kDa protein encoded by a 17-exon gene and highly and selectively expressed in human cerebellar Purkinje cells(PMID:20208537) and it can exsit as a truncated 65 kDa protein(PMID:18337413). Defects in AFG3L2 are the cause of spinocerebellar ataxia type 28 (SCA28) and spastic ataxia autosomal recessive type 5 (SPAX5).