Specification
| Organism | Homo sapiens (Human) |
| Expression Host | E.coli |
| Tag Info | N-terminal GST-tagged |
| Purity | Greater than 85% by SDS-PAGE |
| Uniprot ID | O60220 |
| Gene Names | TIMM8A |
| Alternative Names | Deafness dystonia protein 1 X-linked deafness dystonia protein |
| Expression Region | Full Length(1-97aa ) |
| Molecular Weight | 38 kDa |
| Protein Sequence | MDSSSSSSAAGLGAVDPQLQHFIEVETQKQRFQQLVHQMTELCWEKCMDKPGPKLDSRAEACFVNCVERFIDTSQFILNRLEQTQKSKPVFSESLSD |
| Form | Liquid or Lyophilization |
| Buffer | The default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol if the delivery form is liquid. The lyophilization buffer is Tris/PBS-based buffer, 6% Trehalose, pH 8.0 if the delivery form is lyophilized powder. Please contact us if you have any special requirment. |
| Reconstitution | Please reconstitute protein in deionized sterile water and we recommend that briefly centrifuge thevial prior to opening the vial .We recommend aliquot for long-term storage at -20℃/-80℃. |
Background
| Relevance | 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 70KDA complex mediates the import of much more proteins. Probably necessary for normal neurologic development. |
| Involvement in Disease | Mohr-Tranebjaerg syndrome (MTS) |
| Subcellular Location | Mitochondrion inner membrane, Peripheral membrane protein, Intermembrane side |
| Protein Families | Small Tim family |
| Tissue Specificity | TIMM8A |
QC Data
| Note | Please contact us for QC Data |
| Product Image (Reference Only) |  |