Human SUOX Recombinant Protein (RPPB2299)
- SKU:
- RPPB2299
- Product type:
- Recombinant Protein
- Size:
- 25ug
- Species:
- Human
- Target:
- SUOX
- Synonyms:
- EC 1831
- mitochondrial
- Source:
- Escherichia Coli
- Uniprot:
- P51687
Description
| Product Name: | Human SUOX Recombinant Protein |
| Product Code: | RPPB2299 |
| Size: | 25µg |
| Species: | Human |
| Target: | SUOX |
| Synonyms: | Sulfite Oxidase, EC 1.8.3.1, Sulfite oxidase,mitochondrial. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered colorless solution. |
| Formulation: | SUOX protein solution (1mg/ml) containing Phosphatebuffered saline (pH7.4), 30% glycerol and 1mM DTT. |
| Stability: | Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles. |
| Purity: | Greater than 90% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGSSHHHHHH SSGLVPRGSH MGSESTHIYT KEEVSSHTSP ETGIWVTLGS EVFDVTEFVD LHPGGPSKLMLAAGGPLEPF WALYAVHNQS HVRELLAQYK IGELNPEDKV APTVETSDPY ADDPVRHPAL KVNSQRPFNAEPPPELLTEN YITPNPIFFT RNHLPVPNLD PDTYRLHVVG APGGQSLSLS LDDLHNFPRY EITVTLQCAGNRRSEMTQVK EVKGLEWRTG AISTARWAGA RLCDVLAQAG HQLCETEAHV CFEGLDSDPT GTAYGASIPLARAMDPEAEV LLAYEMNGQP LPRDHGFPVR VVVPGVVGAR HVKWLGRVSV QPEESYSHWQ RRDYKGFSPSVDWETVDFDS APSIQELPVQ SAITEPRDGE TVESGEVTIK GYAWSGGGRA VIRVDVSLDG GLTWQVAKLDGEEQRPRKAW AWRLWQLKAP VPAGQKELNI VCKAVDDGYN VQPDTVAPIW NLRGVLSNAW HRVHVYVSP |
Sulfite oxidase, also known as SUOX is a homodimeric protein localizedto the intermembrane space of mitochondria. Each subunit includes a heme domainas well as a molybdopterin-binding domain. The SUOX enzyme catalyzes theoxidation of sulfite to sulfate, the last reaction in the oxidative degradationof the sulfur amino acids cysteine and methionine. In addition, the deficiencyof SUOX results in neurological abnormalities which are often fatal at an earlyage.
SUOX Human Recombinant produced in E.Coli is a single, non-glycosylatedpolypeptide chain containing 489 amino acids (80-545 a.a) and having amolecular mass of 53.9kDa.SUOX is fused to a 23 amino acid His-tag at N-terminus& purified by proprietary chromatographic techniques.
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | Protein type:Mitochondrial; Energy Metabolism - sulfur; Oxidoreductase; EC 1.8.3.1 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 |
| NCBI Summary: | 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 Code: | P51687 |
| NCBI GenInfo Identifier: | 152031695 |
| NCBI Gene ID: | 6821 |
| NCBI Accession: | P51687.2 |
| UniProt Related Accession: | P51687 |
| Molecular Weight: | 545 |
| NCBI Full Name: | Sulfite oxidase, mitochondrial |
| NCBI Synonym Full Names: | sulfite oxidase |
| NCBI Official Symbol: | SUOX |
| NCBI Protein Information: | sulfite oxidase, mitochondrial |
| UniProt Protein Name: | Sulfite oxidase, mitochondrial |
| Protein Family: | Sulfite oxidase |
| UniProt Gene Name: | SUOX |
| UniProt Entry Name: | SUOX_HUMAN |
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