Size: 200ug. Other sizes are also available. Please Inquire.
In Stock: No
Lead time: 10-20 working days
Research Topic: Signal Transduction
Uniprot ID: O60220
Gene Names: TIMM8A
Organism: Homo sapiens (Human)
AA Sequence: MDSSSSSSAAGLGAVDPQLQHFIEVETQKQRFQQLVHQMTELCWEKCMDKPGPKLDSRAEACFVNCVERFIDTSQFILNRLEQTQKSKPVFSESLSD
Expression Region: 1-97aa
Sequence Info: Full Length
Source: E.coli
Tag Info: N-terminal GST-tagged
MW: 38 kDa
Alternative Name(s): Deafness dystonia protein 1 X-linked deafness dystonia protein
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.
Reference: "A novel X-linked gene, DDP, shows mutations in families with deafness (DFN-1), dystonia, mental deficiency and blindness." Jin H., May M., Tranebjaerg L., Kendall E., Fontan G., Jackson J., Subramony S.H., Arena F., Lubs H., Smith S., Stevenson R., Schwartz C., Vetrie D. Nat. Genet. 14:177-180(1996)
Purity: Greater than 90% as determined by SDS-PAGE.
Storage Buffer: Tris-based buffer，50% glycerol
Storage: The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself. Generally, the shelf life of liquid form is 6 months at -20℃/-80℃. The shelf life of lyophilized form is 12 months at -20℃/-80℃.
Notes: Repeated freezing and thawing is not recommended. Store working aliquots at 4℃ for up to one week.