产品名称
COX10 Rabbit Polyclonal Antibody
别名
COX10; Protoheme IX farnesyltransferase; mitochondrial; Heme O synthase
蛋白名称
Protoheme IX farnesyltransferase mitochondrial
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=1352
Human Swissprot No.
Q12887
Human Swissprot Link
http://www.uniprot.org/uniprotkb/Q12887/entry
Mouse Swissprot No.
Q8CFY5
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q8CFY5
免疫原
The antiserum was produced against synthesized peptide derived from human COX10. AA range:98-147
特异性
COX10 Polyclonal Antibody detects endogenous levels of COX10 protein.
稀释度
WB 1:500 - 1:2000. ELISA: 1:10000. Not yet tested in other applications.
宿主
Polyclonal, Rabbit,IgG
背景介绍
Cytochrome c oxidase (COX), the terminal component of the mitochondrial respiratory chain, catalyzes the electron transfer from reduced cytochrome c to oxygen. This component is a heteromeric complex consisting of 3 catalytic subunits encoded by mitochondrial genes and multiple structural subunits encoded by nuclear genes. The mitochondrially-encoded subunits function in electron transfer, and the nuclear-encoded subunits may function in the regulation and assembly of the complex. This nuclear gene encodes heme A:farnesyltransferase, which is not a structural subunit but required for the expression of functional COX and functions in the maturation of the heme A prosthetic group of COX. This protein is predicted to contain 7-9 transmembrane domains localized in the mitochondrial inner membrane. A gene mutation, which results in the substitution of a lys
细胞定位
Mitochondrion membrane; Multi-pass membrane protein.
信号通路
Oxidative phosphorylation;Porphyrin and chlorophyll metabolism;
功能
disease:Defects in COX10 are a cause of cytochrome c oxidase deficiency (COX deficiency) [MIM:220110]. COX deficiency is a clinically heterogeneous disorder. The clinical features are ranging from isolated myopathy to severe multisystem disease, with onset from infancy to adulthood.,disease:Defects in COX10 are a cause of Leigh syndrome (LS) [MIM:256000]. LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.,function:Converts protoheme IX and farnesyl diphosphate to heme O.,similarity:Belongs to the ubiA prenyltransferase family.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
