TIMM8A recombinant protein

TIMM8A, 1-97aa, Human, His tag, E Coli

Cat. Num. AAA204942

• Gene Names: TIMM8A; DDP; MTS; DDP1; DFN1; TIM8
• Applications: SDS-Page
• Purity: > 90% by SDS-PAGE
• Synonyms: TIMM8A; 1-97aa; Human; His tag; E Coli; Mitochondrial import inner membrane translocase subunit Tim8 A; DDP; DDP1; DFN1; MTS; TIM8; TIMM8A recombinant protein

• Host: E Coli
• Purity/Purification: > 90% by SDS-PAGE
• Form/Format: Liquid. In 20mM Tris-HCl Buffer (pH 8.0) containing 0.15M NaCl, 30% glycerol, 1mM DTT
• Concentration: 0.25 mg/ml (determined by Bradford assay) (varies by lot)
• Sequence: MGSSHHHHHH SSGLVPRGSH MGSMDSSSSS SAAGLGAVDP QLQHFIEVET QKQRFQQLVH QMTELCWEKC MDKPGPKLDS RAEACFVNCV ERFIDTSQFI LNRLEQTQKS KPVFSESLSD
• Sequence Length: 97

• Applicable Applications for TIMM8A recombinant protein: SDS-PAGE
• Antigen Species: Human
• Tag Information: His-tag
• Preparation and Storage: Can be stored at 4 degree C short term (1-2 weeks).; For long term storage, aliquot and store at -20 degree C or -70 degree C.; Avoid repeated freezing and thawing cycles.

SDS-Page

Related Product Information for TIMM8A recombinant protein

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.

Product Categories/Family for TIMM8A recombinant protein

Signal Transduction

References

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

NCBI and Uniprot Product Information

• NCBI GI #: 4758152
• NCBI GeneID: 1678
• NCBI Accession #: NP_004076
• NCBI GenBank Nucleotide #: NM_004085.3
• UniProt Accession #: O60220; Q6IRW6; B2R5A6
• Molecular Weight: 13.4kDa (120aa) confirmed by MALDI-TOF
• NCBI Official Full Name: mitochondrial import inner membrane translocase subunit Tim8 A isoform 1
• NCBI Official Synonym Full Names: translocase of inner mitochondrial membrane 8A
• NCBI Official Symbol: TIMM8A
• NCBI Official Synonym Symbols: DDP; MTS; DDP1; DFN1; TIM8
• NCBI Protein Information: mitochondrial import inner membrane translocase subunit Tim8 A
• UniProt Protein Name: Mitochondrial import inner membrane translocase subunit Tim8 A
• Protein Family: Mitochondrial import inner membrane translocase
• UniProt Gene Name: TIMM8A
• UniProt Synonym Gene Names: DDP; DDP1; TIM8A

NCBI Description

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.[provided by RefSeq, Mar 2009]

Uniprot Description

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.