Human SURF1 Recombinant Protein (RPPB4881)
- SKU:
- RPPB4881
- Product Type:
- Recombinant Protein
- Species:
- Human
- Uniprot:
- Q15526
Description
| Product Name: | Human SURF1 Recombinant Protein |
| Product Code: | RPPB4881 |
| Size: | 25µg |
| Species: | Human |
| Target: | SURF1 |
| Synonyms: | SURF1, SURF-1, Surfeit locus protein 1, Surfeit 1. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered colorless solution. |
| Formulation: | The Surfeit-1 solution contains 20 mM Tris-HCl buffer (pH 8.0), 0.1M Nacl, 1mM DTT and 20% glycerol. |
| 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 95.0% as determined by SDS-PAGE. |
| Amino Acid Sequence: | MGSSHHHHHH SSGLVPRGSH MQVQRRKWKL NLIAELESRV LAEPVPLPAD PMELKNLEYR PVKVRGCFDH SKELYMMPRT MVDPVREARE GGLISSSTQS GAYVVTPFHC TDLGVTILVN RGFVPRKKVN PETRQKGQIE GEVDLIGMVR LTETRQPFVP ENNPERNHWH YRDLEAMARI TGAEPIFIDA NFQSTVPGGP IGGQTRVTLR NEHLQ |
SURF1 plays a role in the biogenesis of the COX complex. SURF1 is found in the inner mitochondrial membrane and takes part in the biogenesis of the cytochrome c oxidase complex. SURF1 is shares a bidirectional promoter with SURF2 on the opposite strand. Defects in this SURF1 cause Leigh syndrome, a rigorous neurological disorder that is generally associated with systemic cytochrome c oxidase deficiency.
Surfeit-1 Human Recombinant produced in E.Coli is a single, non-glycosylated, polypeptide chain containing 215 amino acids (80-273 a.a.) and having a molecular mass of 24.3 kDa. The Surfeit-1 is fused to a 20 amino acid His Tag at N-terminus and purified by proprietary chromatographic techniques.
| UniProt Protein Function: | SURF1: Probably involved in the biogenesis of the COX complex. Defects in SURF1 are a cause of Leigh syndrome (LS). LS is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions that is commonly associated with systemic cytochrome c oxidase (COX) deficiency. Belongs to the SURF1 family. 2 isoforms of the human protein are produced by alternative splicing. |
| UniProt Protein Details: | Protein type:Oxidoreductase; Membrane protein, multi-pass; Membrane protein, integral; Mitochondrial Chromosomal Location of Human Ortholog: 9q34.2 Cellular Component: integral to membrane; mitochondrial respiratory chain Molecular Function:protein binding; cytochrome-c oxidase activity Biological Process: ATP biosynthetic process; respiratory chain complex IV assembly; aerobic respiration; oxidative phosphorylation Disease: Leigh Syndrome |
| NCBI Summary: | This gene encodes a protein localized to the inner mitochondrial membrane and thought to be involved in the biogenesis of the cytochrome c oxidase complex. The protein is a member of the SURF1 family, which includes the related yeast protein SHY1 and rickettsial protein RP733. The gene is located in the surfeit gene cluster, a group of very tightly linked genes that do not share sequence similarity, where it shares a bidirectional promoter with SURF2 on the opposite strand. Defects in this gene are a cause of Leigh syndrome, a severe neurological disorder that is commonly associated with systemic cytochrome c oxidase deficiency. [provided by RefSeq, Jul 2008] |
| UniProt Code: | Q15526 |
| NCBI GenInfo Identifier: | 2498973 |
| NCBI Gene ID: | 6834 |
| NCBI Accession: | Q15526.1 |
| UniProt Secondary Accession: | Q15526,Q5T8T3, Q5T8T4, |
| UniProt Related Accession: | Q15526 |
| Molecular Weight: | 300 |
| NCBI Full Name: | Surfeit locus protein 1 |
| NCBI Synonym Full Names: | surfeit 1 |
| NCBI Official Symbol: | SURF1�� |
| NCBI Protein Information: | surfeit locus protein 1 |
| UniProt Protein Name: | Surfeit locus protein 1 |
| Protein Family: | SURF1-like protein |
| UniProt Gene Name: | SURF1�� |
| UniProt Entry Name: | SURF1_HUMAN |
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