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Sulfite oxidase Polyclonal Antibody
Purified Rabbit Polyclonal Antibody (Pab)
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- BACKGROUND
Application 
| IHC-P, IHC-F, IF, ICC, E |
|---|---|
| Primary Accession | P51687 |
| Reactivity | Rat, Dog |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 52 KDa |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human Sulfite oxidase |
| Epitope Specificity | 351-450/545 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SUBCELLULAR LOCATION | Mitochondrion intermembrane space. |
| SIMILARITY | Contains 1 cytochrome b5 heme-binding domain. |
| SUBUNIT | Homodimer. |
| 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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] |
| Gene ID | 6821 |
|---|---|
| Other Names | Sulfite oxidase, mitochondrial, 1.8.3.1, SUOX |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,ICC=1:100-500,IF=1:100-500,ELISA=1:5000-10000 |
| Storage | Store at -20 ℃ for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 ℃. |
| Name | SUOX |
|---|---|
| Function | Catalyzes the oxidation of sulfite to sulfate, the terminal reaction in the oxidative degradation of sulfur-containing amino acids. |
| Cellular Location | Mitochondrion intermembrane space {ECO:0000250|UniProtKB:Q07116} |
Research Areas
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$ 385.00
Cat# AP54260
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