Human SDHAF2 Recombinant Protein (RPPB4606)
- SKU:
- RPPB4606
- Product Type:
- Recombinant Protein
- Species:
- Human
- Uniprot:
- Q9NX18
Description
| Product Name: | Human SDHAF2 Recombinant Protein |
| Product Code: | RPPB4606 |
| Size: | 20µg |
| Species: | Human |
| Target: | SDHAF2 |
| Synonyms: | Succinate dehydrogenase assembly factor 2 mitochondrial, SDH assembly factor 2, Succinate dehydrogenase subunit 5, mitochondrial, hSDH5, SDHAF2, C11orf79, PGL2, SDH5. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered colorless solution. |
| Formulation: | SDHAF2 protein solution (0.5mg/ml) containing 20mM Tris-HCl buffer (pH 8.0), 0.2M NaCl, 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 90.0% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGSSHHHHHH SSGLVPRGSH MGSSFRRFYR GDSPTDSQKD MIEIPLPPWQ ERTDESIETK RARLLYESRK RGMLENCILL SLFAKEHLQH MTEKQLNLYD RLINEPSNDW DIYYWATEAK PAPEIFENEV MALLRDFAKN KNKEQRLRAP DLEYLFEKPR |
Succinate Dehydrogenase Complex Assembly Factor 2 (SDHAF2) is a mitochondrial protein required for the flavination of a succinate dehydrogenase complex subunit essential for activity of the complex. DHAF2 is necessary for insertion of FAD cofactor into SDHA, the catalytic subunit of succinate dehydrogenase (SDH). SDH is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). SDHAF2 gene defects are the cause of hereditary paragangliomas type 2 (PGL2), otherwise known as familial non-chromaffin paragangliomas type 2.
SDHAF2 Human Recombinant produced in E.Coli is a single, non-glycosylated polypeptide chain containing 160 amino acids (30-166 a.a) and having a molecular mass of 19kDa.SDHAF2 is fused to a 23 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.
| UniProt Protein Function: | SDH5: a subunit of succinate dehydrogenase (SDH) complex II of the mitochondrial electron transport chain. SDH5 is required for insertion of FAD cofactor into SDHA, the catalytic subunit of succinate dehydrogenase (SDH) complex II. SDH is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Mutated in some cancers. Germline mutations cause hereditary paragangliomas type 2, or non-chromaffin paragangliomas type 2. Paragangliomas are rare tumors that arise from components of the neuroendocrine system. |
| UniProt Protein Details: | Chromosomal Location of Human Ortholog: 11q12.2 Cellular Component: mitochondrion; mitochondrial matrix Molecular Function:protein binding Biological Process: mitochondrial electron transport, succinate to ubiquinone; protein-FAD linkage; protein amino acid dephosphorylation Disease: Paragangliomas 2 |
| NCBI Summary: | This gene encodes a mitochondrial protein needed for the flavination of a succinate dehydrogenase complex subunit required for activity of the complex. Mutations in this gene are associated with paraganglioma.[provided by RefSeq, Jul 2010] |
| UniProt Code: | Q9NX18 |
| NCBI GenInfo Identifier: | 8923450 |
| NCBI Gene ID: | 54949 |
| NCBI Accession: | NP_060311.1 |
| UniProt Related Accession: | Q9NX18 |
| Molecular Weight: | 19,599 Da |
| NCBI Full Name: | succinate dehydrogenase assembly factor 2, mitochondrial |
| NCBI Synonym Full Names: | succinate dehydrogenase complex assembly factor 2 |
| NCBI Official Symbol: | SDHAF2�� |
| NCBI Official Synonym Symbols: | PGL2; SDH5; C11orf79�� |
| NCBI Protein Information: | succinate dehydrogenase assembly factor 2, mitochondrial; SDH assembly factor 2; SDHAF2; hSDH5; succinate dehydrogenase subunit 5, mitochondrial |
| UniProt Protein Name: | Succinate dehydrogenase assembly factor 2, mitochondrial |
| Protein Family: | Succinate dehydrogenase assembly factor |
| UniProt Gene Name: | SDHAF2�� |
| UniProt Entry Name: | SDHF2_HUMAN |
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