TIMM8A (Translocase of inner Mitochondrial Membrane 8 Homolog A (yeast), DDP, DDP1, DFN1, MGC12262, MTS)

Size

£685.00

SKU:: 252662
Purity:: Purified
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Mouse
Immunogen:: TIMM8A (NP_004076, 9aa-97aa) partial recombinant protein with GST tag. MW of the GST tag alone is 26kD.
Clone:: 2D12
Format:: Supplied as a liquid in PBS, pH 7.4.
Extra Details:: 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 Applications: Suitable for use in ELISA, Western Blot. Other applications not tested. Recommended Dilution: Optimal dilutions to be determined by the researcher. AA Sequence: AAGLGAVDPQLQHFIEVETQKQRFQQLVHQMTELCWEKCMDKPGPKLDSRAEACFVNCVERFIDTSQFILNRLEQTQKSKPVFSESLSD Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.