产品名称
SUOX Rabbit Polyclonal Antibody
存储缓冲液
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.
Human Gene Link
https://www.uniprot.org/uniprot/6821
Human Swissprot No.
P51687
Human Swissprot Link
https://www.uniprot.org/uniprotkb/P51687/entry
Mouse Gene Link
https://www.uniprot.org/uniprot/211389
Mouse Swissprot No.
Q8R086
Mouse Swissprot Link
https://www.uniprot.org/uniprotkb/Q8R086
Rat Gene Link
https://www.uniprot.org/uniprot/81805
Rat Swissprot Link
https://www.uniprot.org/uniprotkb/Q07116
免疫原
Synthesized peptide derived from human SUOX AA range: 64-114
特异性
This antibody detects endogenous levels of SUOX at Human/Mouse/Rat
宿主
Polyclonal, Rabbit,IgG
背景介绍
Sulfite oxidase is a homodimeric protein localized to the intermembrane space of mitochondria. Each subunit contains a heme domain and a molybdopterin-binding domain. The enzyme catalyzes the oxidation of sulfite to sulfate, the final reaction in the oxidative degradation of the sulfur amino acids cysteine and methionine. Sulfite oxidase deficiency results in neurological abnormalities which are often fatal at an early age. Alternative splicing results in multiple transcript variants encoding identical proteins. [provided by RefSeq, Jul 2008],
细胞定位
Mitochondrion intermembrane space .
功能
catalytic activity:Sulfite + O(2) + H(2)O = sulfate + H(2)O(2).,cofactor:Binds 1 protoheme group.,cofactor:Molybdenum (molybdopterin).,disease:Defects in SUOX are the cause of isolated sulfite oxidase deficiency (ISOD) [MIM:272300]; also known as sulfocysteinuria. ISOD is characterized by neurological abnormalities including multicystic leukoencephalopathy with brain atrophy. Patients often suffer from seizures. Often leads to death at an early age.,pathway:Energy metabolism; sulfur metabolism.,similarity:Contains 1 cytochrome b5 heme-binding domain.,subunit:Homodimer.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
