TIMM8A Antibody (PACO44417)
- SKU:
- PACO44417
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
抗体名: | TIMM8A Antibody (PACO44417) |
抗体コード: | PACO44417 |
サイズ: | 50ul |
宿主種: | Rabbit |
申し込み: | ELISA, IHC |
推奨される希釈: | ELISA:1:2000-1:10000, IHC:1:20-1:200 |
反応性: | Human |
免疫原: | Recombinant Human Mitochondrial import inner membrane translocase subunit Tim8 A protein (1-97AA) |
憲法: | Liquid |
ストレージバッファ: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
精製方法: | Antigen Affinity Purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
Immunohistochemistry of paraffin-embedded human liver cancer using PACO44417 at dilution of 1:100. |
バックグラウンド: | 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. |
シノニム: | Mitochondrial import inner membrane translocase subunit Tim8 A (Deafness dystonia protein 1) (X-linked deafness dystonia protein), TIMM8A, DDP DDP1 TIM8A |
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 |