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Target antigen
AFG3L2
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Clonality
Polyclonal antibody
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Clone
Polyclonal antibody
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Raised in
rabbit
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Type of the antibody
IgG polyclonal antibody
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Product form
freeze-dried
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Reacts with species
mouse, rat Theoretical reactivity:human
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Analyses
WB
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Immunogen
E. coli-derived human AFG3L2 recombinant protein (Position: R168-D250). Human AFG3L2 shares 100% amino acid (aa) sequence identity with mouse AFG3L2.
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Product configuration
Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3.
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Purification
Immunogen affinity purified.
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Solubilization
The powdered antibody should be dissolved in 0.2 ml of distilled water to achieve final concentration of 500ug/ml
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Storage condtions
Keep the AFG3L2 Antibodyat minus twenty degrees Celsius for 1 year. The ready-to-use solutions can be stored at four degrees Celsius for a month. Our specialsits recommend to freeze the aliquotes at minus twenty degrees Celsius for long-term application. Multiple procedures of freezing and thawing influence the specifity and reactivity of the antibody in a negative way.
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Tips
The AFG3L2 Antibody did not cross-reacted with other proteins during the test procedures. This antobdy is intended to be used for research analyses and it is not applicale for in vitro diagnostics.
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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.
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Related articles
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.
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Gene Name
AFG3L2
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Protein Name
AFG3-like protein 2
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Gene Full Name
AFG3 like matrix AAA peptidase subunit 2
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Synonyms
AFG3 like protein 2 | AFG3-like protein 2 | AFG3L2 | SCA28 | Spinocerebellar ataxia 28 | Q9Y4W6
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Uniprot ID
Q9Y4W6
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Entrez GeneID
10939
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Properties
If you buy Antibodies supplied by boster they should be stored frozen at - 24°C for long term storage and for short term at + 5°C.
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French translation
anticorps
