Anti-TIMM8A Antibody (CAB9811)
- SKU:
- CAB9811
- Product type:
- Antibody
- Application:
- WB
- Reactivity:
- Human
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Cell Biology
Frequently bought together:
Description
Antibody Name: | Anti-TIMM8A Antibody |
Antibody SKU: | CAB9811 |
Antibody Size: | 20uL, 50uL, 100uL |
Application: | WB |
Reactivity: | Human, Mouse |
Host Species: | Rabbit |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-97 of human TIMM8A (NP_004076.1). |
Application: | WB |
Recommended Dilution: | WB 1:500 - 1:2000 |
Reactivity: | Human, Mouse |
Positive Samples: | U-251MG, A-549, LO2, 293T, MCF7, HeLa, Mouse liver, Mouse brain |
Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-97 of human TIMM8A (NP_004076.1). |
Purification Method: | Affinity purification |
Storage Buffer: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Isotype: | IgG |
Sequence: | MDSS SSSS AAGL GAVD PQLQ HFIE VETQ KQRF QQLV HQMT ELCW EKCM DKPG PKLD SRAE ACFV NCVE RFID TSQF ILNR LEQT QKSK PVFS ESLS D |
Gene ID: | 1678 |
Uniprot: | O60220 |
Cellular Location: | Intermembrane side, Mitochondrion inner membrane, Peripheral membrane protein |
Calculated MW: | 10kDa |
Observed MW: | 11kDa |
Synonyms: | TIMM8A, DDP, DDP1, DFN1, MTS, TIM8 |
Background: | 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. |
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 |