Molecular weight (DA)
88kD
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.
Dilution rate
WB 1:500-2000, ELISA 1:10000-20000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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.,
Other name
AFG3-like protein 2 (EC 3.4.24.-) (Paraplegin-like protein)
Fields
>>Spinocerebellar ataxia
Human protein sequence Database
Q9Y4W6
Mouse protein sequence database
Q8JZQ2
Rat protein sequence database
Cellular localization
Mitochondrion . Mitochondrion inner membrane ; Multi-pass membrane protein .
Tissue expression
Ubiquitous. Highly expressed in the cerebellar Purkinje cells.
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