The AFG3L2 Antibody from MyBioSource.com is a Rabbit Polyclonal antibody to AFG3L2. This antibody recognizes Human, Mouse, and Rat antigen. The AFG3L2 Antibody has been shown to work in the following applications: Immunohistochemistry, and Western Blot.
Description
Function: ATP-dependent protease which is essential for axonal and neuron development. In neurons, mediates degradation of SMDT1/EMRE before its assembly with the uniporter complex, limiting the availability of SMDT1/EMRE for MCU assembly and promoting efficient assembly of gatekeeper subunits with MCU (PubMed:27642048). Required for the maturation of paraplegin (SPG7) after its cleavage by mitochondrial-processing peptidase (MPP), converting it into a proteolytically active mature form (By similarity).
Subunit Structure: Homooligomer. Forms heterooligomers with SPG7 and AFG3L1 (PubMed:17101804). Interacts with SPG7; the interaction is required for the efficient assembly of mitochondrial complex I (PubMed:14623864, PubMed:26387735). Interacts with AFG3L1 (By similarity). Interacts with MAIP1 (PubMed:27499296, PubMed:27642048).
Post-translational Modifications: Upon import into the mitochondrion, the N-terminal transit peptide is cleaved to generate an intermediate form which undergoes autocatalytic proteolytic processing to generate the proteolytically active mature form.
Similarity: In the N-terminal section; belongs to the AAA ATPase family.In the C-terminal section; belongs to the peptidase M41 family