Immunogen
Synthesized peptide derived from human SUOX AA range: 64-114
Specificity
This antibody detects endogenous levels of SUOX at Human/Mouse/Rat
Source
Polyclonal, Rabbit,IgG
Formulation
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Dilution rate
WB 1:500-2000
Purification process (Immunogen)
The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
Background
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],
Function
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.,
Fields
>>Sulfur metabolism;>>Metabolic pathways
Human protein sequence Database
P51687
Mouse protein sequence database
Q8R086
Rat protein sequence database
Q07116
Cellular localization
Mitochondrion intermembrane space .