AFG3L2 Antibody from MYBIOSOURCE INC.
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| Antigenic Specificity | AFG3L2 |
| Clone | polyclonal |
| Host Species | Rabbit |
| Reactive Species | human, mouse, rat. |
| Isotype | n/a |
| Format | immunogen affinity purified |
| Size | 0.1 mg |
| Concentration | n/a |
| Applications | Western Blot (WB) |
| Reviews / Ratings | If you have used this antibody, please help fellow researchers by submitting reviews to pAbmAbs and antYbuddY. |
| Description | Rabbit IgG polyclonal antibody for AFG3-like protein 2 (AFG3L2) detection. Background: 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. In humans, it is encoded by the AFG3L2 gene. 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. And this gene is a candidate gene for other hereditary spastic paraplegias or neurodegenerative disorders as well as spastic ataxia-neuropathy syndrome. |
| Immunogen | Immunogen: E Coli-derived human AFG3L2 recombinant protein (Position: R168-D250). Human AFG3L2 shares 100% amino acid (aa) sequence identity with mouse AFG3L2. |
| Other Names | [AFG3 like protein 2; AFG3-like protein 2; AFG3L2; SCA28; Spinocerebellar ataxia 28; Q9Y4W6; AFG3-like protein 2; AFG3 like matrix AAA peptidase subunit 2], [AFG3L2; AFG3L2; SCA28; SPAX5] |
| Gene, Accession # | [AFG3L2], Gene ID: 10939, NCBI: NP_006787.2, UniProt: Q9Y4W6 |
| Catalog # | MBS178415 |
| Price | $280 |
| Order / More Info | AFG3L2 Antibody from MYBIOSOURCE INC. |
| Product Specific References | 1. Entrez Gene: AFG3 ATPase family gene 3-like 2 (S. cerevisiae). 2. Koppen, M., Metodiev, M. D., Casari, G., Rugarli, E. I., Langer, T. Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia. Molec. Cell. Biol. 27: 758-767, 2007. 3. Pierson TM, Adams D, Bonn F, Martinelli P, Cherukuri PF, Teer JK, Hansen NF, Cruz P, Mullikin For The Nisc Comparative Sequencing Program JC, Blakesley RW, Golas G, Kwan J, Sandler A, Fuentes Fajardo K, Markello T, Tifft C, Blackstone C, Rugarli EI, Langer T, Gahl WA, Toro C (October 2011). Whole-exome sequencing identifies homozygous AFG3L2 mutations in a spastic ataxia-neuropathy syndrome linked to mitochondrial m-AAA proteases. PLoS Genet. 7 (10): e1002325. |
MYBIOSOURCE INC.
MYBIOSOURCE INC.
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P: 1.858.633.0165
P: 1.888.MBS.0165 (1.888.627.0165) (US & Canada)
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