TIMM8A protein (His tag) (80R-3619)
Purified recombinant TIMM8A protein (His tag)
|Synonyms||DDP protein, DDP1 protein, DFN1 protein, MTS protein, TIM8 protein, TIMM 8A protein, TIMM-8A protein|
Coomassie blue stained SDS-PAGE of TIMM8A protein (His tag) (80R-3619)
Figure annotation denotes ug of protein loaded and % gel used.
|Residues||1-97 aa: MGSSHHHHHH SSGLVPRGSH MGSMDSSSSS SAAGLGAVDP QLQHFIEVET QKQRFQQLVH QMTELCWEKC MDKPGPKLDS RAEACFVNCV ERFIDTSQFI LNRLEQTQKS KPVFSESLSD|
|Purity||> 90% pure|
|Method of Purification||TIMM8A protein was purified by using conventional chromatography techniques|
|Molecular Weight||13.4 kDa|
|Form & Buffer||Supplied in liquid form in 20mM Tris-HCl buffer (pH 8.0) containing 0.15M NaCl, 30% glycerol, 1mM DTT|
Storage & Safety
|Storage||Can be stored at 4 deg C short term (1-2 weeks). For long term storage, aliquot and store at -20 deg C or -70 deg C. Avoid repeated freezing and thawing cycles.|
|Biological Significance||TIMM8A 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.|
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