Recombinant humaan TIMM8A eiwit

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. Recombinant human TIMM8A proten, fused to His-tag at N-terminus, was expressed in E. coli and purified by using conventional chromatography techniques.

Mitochondrial import inner membrane translocase subunit Tim8 A, Mitochondrial import inner membrane translocase subunit Tim8 A, DDP, DDP1, DFN1, MTS, TIM8

E.coli

Human

His-Tag

SDS-PAGE

0.25 mg/mL (determined by Bradford assay)

> 90% by SDS-PAGE

13.4 kDa (120aa) confirmed by MALDI-TOF

TIMM8A, Mitochondrial import inner membrane translocase subunit Tim8 A, Mitochondrial import inner membrane translocase subunit Tim8 A, DDP, DDP1, DFN1, MTS, TIM8, ATGP2486-10 µg, ATGP2486-20 µg, ATGP2486-50 µg, ATGP2486-100 µg, ATGP2486-250 µg, ATGP2486-500 µg, ATGP2486-1 mg, ATGP2486-10, ATGP2486-20, ATGP2486-50, ATGP2486-100, ATGP2486-250, ATGP2486-500, ATGP2486-1

Rothbauer u., et al. (2001) J. Biol. Chem. 276:37327-37334; ; Roesch K., et al. (2004) Hum. Mol. Genet. 13:2101-2111;

Can be stored at 2°C to 8°C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles.

Liquid in. 20 mM Tris-HCl buffer (pH 8.0) containing 0.15M NaCl, 30% glycerol, 1mM DTT

Signal Transduction

NP_004076

O60220

Human

MGSSHHHHHH SSGLVPRGSH MGSMDSSSSS SAAGLGAVDP QLQHFIEVET QKQRFQQLVH QMTELCWEKC MDKPGPKLDS RAEACFVNCV ERFIDTSQFI LNRLEQTQKS KPVFSESLSD