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
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺
The antibody was affinity-purified from mouse serum by affinity-chromatography using specific immunogen.
背景
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],
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.,
