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SURF-1 Polyclonal Antibody, 100μg, (ATB-T4471)

$199.00
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Background: This gene encodes a protein localized to the inner mitochondrial membrane and thought to be involved in the biogenesis of the cytochrome c oxidase complex. The protein is a member of the SURF1 family, which includes the related yeast protein SHY1 and rickettsial protein RP733. The gene is located in the surfeit gene cluster, a group of very tightly linked genes that do not share sequence similarity, where it shares a bidirectional promoter with SURF2 on the opposite strand. Defects in this gene are a cause of Leigh syndrome, a severe neurological disorder that is commonly associated with systemic cytochrome c oxidase deficiency. [provided by RefSeq, Jul 2008]Product datasheet: Overview Product Description SURF-1 Polyclonal Antibody, 100μg, (ATB-T4471) Image Species Reactivities Human,Mouse,Rat Immunogen Synthesized peptide derived from the Internal region of human SURF-1. Properties Form Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. Storage Instructions-20°C/1 year References: SURF1 deficiency causes demyelinating Charcot-Marie-Tooth disease. Echaniz-Laguna A, et al. Neurology, 2013 Oct 22. PMID 24027061 Free PMC Article SURF1-associated Leigh syndrome: a case series and novel mutations. Lee IC, et al. Hum Mutat, 2012 Aug. PMID 22488715 Adaptation of respiratory chain biogenesis to cytochrome c oxidase deficiency caused by SURF1 gene mutations. Kovářová N, et al. Biochim Biophys Acta, 2012 Jul. PMID 22465034 Two Japanese patients with Leigh syndrome caused by novel SURF1 mutations. Tanigawa J, et al. Brain Dev, 2012 Nov. PMID 22410471 [Syndrome Leigh caused by mutations in the SURF1 gene: clinical and molecular-genetic characteristics]. Tsygankova PG, et al. Zh Nevrol Psikhiatr Im S S Korsakova, 2010. PMID 20436434

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SURF-1 Polyclonal Antibody, 100μg, (ATB-T4471)
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