Target：AFG3L2

Fields：Spinocerebellar ataxia

Gene Name：AFG3L2

Protein Name：AFG3L2

Human Gene Id：10939

Human Swiss Prot No：Q9Y4W6

Mouse Gene Id：69597

Mouse Swiss Prot No：Q8JZQ2

Immunogen：Synthesized peptide derived from human AFG3L2. at AA range: 744-793

Specificity：AFG3L2 Polyclonal Antibody detects endogenous levels of AFG3L2

Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Source：Polyclonal, Rabbit,IgG

Dilution：WB 1:500-2000, ELISA 1:10000-20000

Purification：The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration：1 mg/ml

Storage Stability：-15°C to -25°C/1 year(Do not lower than -25°C)

Other Name：AFG3-like protein 2 (EC 3.4.24.-) (Paraplegin-like protein)

Observed Band(KD)：88kD

Background： 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. This gene is a candidate gene for other hereditary spastic paraplegias or neurodegenerative disorders. [provided by RefSeq, Jul 2008],

Function：cofactor:Binds 1 zinc ion per subunit .,function:Putative ATP-dependent protease.,similarity:In the C-terminal section; belongs to the peptidase M41 family.,similarity:In the N-terminal section; belongs to the AAA ATPase family.,tissue specificity:Ubiquitous.,

Subcellular Location：Mitochondrion . Mitochondrion inner membrane ; Multi-pass membrane protein .

Expression：Ubiquitous. Highly expressed in the cerebellar Purkinje cells.