Product Description
Recombinant Human Mitochondrial import inner membrane translocase subunit Tim8 A (TIMM8A) is available at Gentaur for Next week Delivery.
Gene Name: TIMM8A
Alternative Names : Deafness dystonia protein 1 X-linked deafness dystonia protein
Expression Region : 1-97aa
AA Sequence : MDSSSSSSAAGLGAVDPQLQHFIEVETQKQRFQQLVHQMTELCWEKCMDKPGPKLDSRAEACFVNCVERFIDTSQFILNRLEQTQKSKPVFSESLSD
Sequence Info : Full Length
Tag Info : N-terminal GST-tagged
Theoretical MW : 38 kDa
Storage Buffer : Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, pH 8.0.
Endotoxin Level : Not tested-
Biological Activity : Not tested
Storage : Short term: -20°C; Long term: -80°C. Minimize freeze and thaw cycles.
Research Area : Signal Transduction
Restriction : For Research Use Only. Not for use in diagnostic procedures, drug use, or for administration to humans or animals.
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.
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.
Involvement in disease : Mohr-Tranebjaerg syndrome (MTS)
Subcellular location : Mitochondrion inner membrane, Peripheral membrane protein, Intermembrane side
Protein Families : Small Tim family
Tissue Specificity : Highly expressed in fetal and adult brain, followed by fetal lung, liver and kidney. Also expressed in heart, placenta, lung, liver, kidney, pancreas, skeletal muscle and heart.
Paythway :
Uniprot ID : O60220