Rabbit anti-Rat Sulfite Oxidase (SUOX) Polyclonal Antibody | anti-SUOX antibody
Biotin-Linked Antibody to Sulfite Oxidase (SUOX)
Immunocytochemistry in formalin fixed cells: 1:100-500
Immunohistochemistry in formalin fixed frozen section: 1:100-500
Immunohistochemistry in paraffin section: 1:50-200
Enzyme-linked Immunosorbent Assay: 1:100-200
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
SUOX: Defects in SUOX are the cause of isolated sulfite oxidase deficiency (ISOD); 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.
Protein type: Energy Metabolism - sulfur; Oxidoreductase; EC 1.8.3.1; Mitochondrial
Chromosomal Location of Human Ortholog: 12q13.2
Cellular Component: mitochondrial matrix; mitochondrial intermembrane space
Molecular Function: molybdopterin cofactor binding; electron carrier activity; molybdenum ion binding; heme binding; sulfite oxidase activity
Biological Process: sulfur amino acid metabolic process; sulfur amino acid catabolic process
Disease: Sulfocysteinuria