Human TIMM8A Recombinant Protein (RPPB4962)
- SKU:
- RPPB4962
- Product type:
- Recombinant Protein
- Size:
- 20ug
- Species:
- Human
- Target:
- TIMM8A
- Synonyms:
- Mitochondrial import inner membrane translocase subunit Tim8 A
- TIMM8A
- Translocase of Inner Mitochondrial Membrane 8 Homolog A
- DDP
- Source:
- Escherichia Coli
- Uniprot:
- O60220
Description
| Product Name: | Human TIMM8A Recombinant Protein |
| Product Code: | RPPB4962 |
| Size: | 20µg |
| Species: | Human |
| Target: | TIMM8A |
| Synonyms: | Mitochondrial import inner membrane translocase subunit Tim8 A, TIMM8A, Translocase of Inner Mitochondrial Membrane 8 Homolog A, DDP, DDP1, DFN1, MTS, TIM8, Deafness dystonia protein 1, X-linked deafness dystonia protein. |
| Source: | Escherichia Coli |
| Physical Appearance: | Sterile Filtered colorless solution. |
| Formulation: | The TIMM8A solution (0.25mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 0.15M 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 MGSMDSSSSS SAAGLGAVDP QLQHFIEVET QKQRFQQLVH QMTELCWEKC MDKPGPKLDS RAEACFVNCV ERFIDTSQFI LNRLEQTQKS KPVFSESLSD |
Translocase of Inner Mitochondrial Membrane 8 Homolog A (TIMM8A) takes part in the import and insertion of hydrophobic membrane proteins from the cytoplasm into the mitochondrial. TIMM8A plays a role as a chaperone-like protein which protects the hydrophobic precursors from aggregation and leads them through the mitochondrial intermembrane space. TIMM8A is essential for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Defects in TIMM8A cause Jensen syndrome. TIMM8A and TIMM13, forms a 70 kDa heterohexamer.
TIMM8A Human Recombinant produced in E.coli is a single, non-glycosylated polypeptide chain containing 120 amino acids (1-97) and having a molecular mass of 13.4 kDa.TIMM8A is fused to a 23 amino acid His-Tag at N-terminus and purified by proprietary chromatographic techniques.
| UniProt Protein Function: | Mitochondrial intermembrane chaperone that participates in the import and insertion of some multi-pass transmembrane proteins into the mitochondrial inner membrane. Also required for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Acts as a chaperone-like protein that protects the hydrophobic precursors from aggregation and guide them through the mitochondrial intermembrane space. The TIMM8-TIMM13 complex mediates the import of proteins such as TIMM23, SLC25A12/ARALAR1 and SLC25A13/ARALAR2, while the predominant TIMM9-TIMM10 70 kDa complex mediates the import of much more proteins. Probably necessary for normal neurologic development. |
| NCBI Summary: | This translocase is involved in the import and insertion of hydrophobic membrane proteins from the cytoplasm into the mitochondrial inner membrane. The gene is mutated in Mohr-Tranebjaerg syndrome/Deafness Dystonia Syndrome (MTS/DDS) and it is postulated that MTS/DDS is a mitochondrial disease caused by a defective mitochondrial protein import system. Defects in this gene also cause Jensen syndrome; an X-linked disease with opticoacoustic nerve atrophy and muscle weakness. This protein, along with TIMM13, forms a 70 kDa heterohexamer. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Mar 2009] |
| UniProt Code: | O60220 |
| NCBI GenInfo Identifier: | 4758152 |
| NCBI Gene ID: | 1678 |
| NCBI Accession: | NP_004076.1 |
| UniProt Secondary Accession: | O60220,Q6IRW6, B2R5A6, |
| UniProt Related Accession: | O60220 |
| Molecular Weight: | 10,998 Da |
| NCBI Full Name: | mitochondrial import inner membrane translocase subunit Tim8 A isoform 1 |
| NCBI Synonym Full Names: | translocase of inner mitochondrial membrane 8A |
| NCBI Official Symbol: | TIMM8A |
| NCBI Official Synonym Symbols: | DDP; MTS; DDP1; DFN1; TIM8 |
| NCBI Protein Information: | mitochondrial import inner membrane translocase subunit Tim8 A |
| UniProt Protein Name: | Mitochondrial import inner membrane translocase subunit Tim8 A |
| UniProt Synonym Protein Names: | Deafness dystonia protein 1; X-linked deafness dystonia protein |
| UniProt Gene Name: | TIMM8A |
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