Human TIMM8A Recombinant Protein (RPPB4962)

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SKU:
RPPB4962
Product type:
Recombinant Protein
Size:
20ug
Species:
Human
Target:
TIMM8A
Synonyms:
Mitochondrial import inner membrane translocase subunit Tim8 A
Synonyms:
TIMM8A
Synonyms:
Translocase of Inner Mitochondrial Membrane 8 Homolog A
Synonyms:
DDP
Source:
Escherichia Coli
Uniprot:
O60220
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Description

Product Name:Human TIMM8A Recombinant Protein
Product Code:RPPB4962
Size:20µg
Species:Human
Target:TIMM8A
Synonyms:Mitochondrial import inner membrane translocase subunit Tim8 A, TIMM8A, Translocase of Inner Mitochondrial Membrane 8 Homolog A, DDP, DDP1, DFN1, MTS, TIM8, Deafness dystonia protein 1, X-linked deafness dystonia protein.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered colorless solution.
Formulation:The TIMM8A solution (0.25mg/ml) contains 20mM Tris-HCl buffer (pH 8.0), 0.15M NaCl, 30% glycerol and 1mM DTT.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 90.0% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MGSMDSSSSS SAAGLGAVDP QLQHFIEVET QKQRFQQLVH QMTELCWEKC MDKPGPKLDS RAEACFVNCV ERFIDTSQFI LNRLEQTQKS KPVFSESLSD

Translocase of Inner Mitochondrial Membrane 8 Homolog A (TIMM8A) takes part in the import and insertion of hydrophobic membrane proteins from the cytoplasm into the mitochondrial. TIMM8A plays a role as a chaperone-like protein which protects the hydrophobic precursors from aggregation and leads them through the mitochondrial intermembrane space. TIMM8A is essential for the transfer of beta-barrel precursors from the TOM complex to the sorting and assembly machinery (SAM complex) of the outer membrane. Defects in TIMM8A cause Jensen syndrome. TIMM8A and TIMM13, forms a 70 kDa heterohexamer.

TIMM8A Human Recombinant produced in E.coli is a single, non-glycosylated polypeptide chain containing 120 amino acids (1-97) and having a molecular mass of 13.4 kDa.TIMM8A is fused to a 23 amino acid His-Tag at N-terminus and purified by proprietary chromatographic techniques.

UniProt Protein 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.
NCBI Summary: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 Code:O60220
NCBI GenInfo Identifier:4758152
NCBI Gene ID:1678
NCBI Accession:NP_004076.1
UniProt Secondary Accession:O60220,Q6IRW6, B2R5A6,
UniProt Related Accession:O60220
Molecular Weight:10,998 Da
NCBI Full Name:mitochondrial import inner membrane translocase subunit Tim8 A isoform 1
NCBI 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
UniProt Synonym Protein Names:Deafness dystonia protein 1; X-linked deafness dystonia protein
UniProt Gene Name:TIMM8A  
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