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: EC 1.8.3.1; Energy Metabolism - sulfur; Mitochondrial; Oxidoreductase
Chromosomal Location of Human Ortholog: 12q13.2
Cellular Component: mitochondrial matrix
Molecular Function: protein binding; sulfite oxidase activity
Disease: Sulfocysteinuria
Product Notes
The Human SUOX suox (Catalog #
AAA9428123) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA9428123 ELISA Kit recognizes Human SUOX.
It is sometimes possible for the material contained within the vial of
"Sulfite oxidase, ELISA Kit" to become dispersed throughout the inside of
the vial, particularly around the seal of said vial, during shipment and storage. We always
suggest centrifuging these vials
to consolidate all of the liquid away from the lid and to the bottom of the vial prior to
opening. Please be advised that
certain products may require dry ice for shipping and that, if this is the case, an
additional dry ice fee may also be
required.
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industry standard. AAA Biotech
and its authorized distribution partners reserve the right to refuse to fulfill any order if
we have any indication that a
purchaser may be intending to use a product outside of our accepted criteria.
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aspect of this datasheet at
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