AFG3L2 Antibody (N-term) [APG01599G]

AFG3L2 Antibody (N-term) [APG01599G] | Gentaur UK, US & Europe Distribution

Product Category: Polyclonal Antibodies

Host: Rabbit

Species Reactivity: H

Specificity: This AFG3L2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 52-80 amino acids from the N-terminal region of human AFG3L2.

Cellular Localisation: Mitochondrion. Mitochondrion inner membrane {ECO:0000250|UniProtKB:Q8JZQ2}; Multi-pass membrane protein

Molecular Weight: 88584

Clone: Polyclonal

Gene Name: AFG3L2 (HGNC:315)

Gene ID: 10939

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 paraplegin (SPG7) maturation (PubMed:30252181) . After its cleavage by mitochondrial-processing peptidase (MPP), it converts paraplegin into a proteolytically active mature form (By similarity) . Required for the maturation of PINK1 into its 52kDa mature form after its cleavage by mitochondrial-processing peptidase (MPP) (PubMed:22354088, PubMed:30252181) . Involved in the regulation of OMA1-dependent processing of OPA1 (PubMed:32600459, PubMed:30252181) .

Summary: Tissue Location: Ubiquitous. Highly expressed in the cerebellar Purkinje cells.

Form: Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Storage: Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.

Application: WB, IHC-P

Dilution: WB--1:1000 IHC-P--1:100~500

Synonyms: AFG3-like protein 2, 3424-, Paraplegin-like protein, AFG3L2