TIMM8A Antibody

說明書

中文名稱：

TIMM8A兔多克隆抗體
貨號：

CSB-PA023557ESR2HU
規格：

￥440
圖片：
Immunohistochemistry of paraffin-embedded human liver cancer using CSB-PA023557ESR2HU at dilution of 1:100
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品名稱：

Rabbit anti-Homo sapiens (Human) TIMM8A Polyclonal antibody
Uniprot No.：

O60220
基因名：

TIMM8A
別名：

DDP 1 antibody; DDP antibody; DDP1 antibody; Deafness dystonia protein 1 antibody; Deafness/dystonia peptide antibody; DFN 1 antibody; DFN1 antibody; MGC12262 antibody; Mitochondrial import inner membrane translocase subunit Tim8 A antibody; MTS antibody; TIM 8A antibody; TIM8 antibody; TIM8A antibody; TIM8A_HUMAN antibody; TIMM 8A antibody; timm8a antibody; Translocase of inner mitochondrial membrane 8 homolog A antibody; X linked deafness dystonia protein antibody; X-linked deafness dystonia protein antibody
宿主：

Rabbit
反應種屬：

Human
免疫原：

Recombinant Human Mitochondrial import inner membrane translocase subunit Tim8 A protein (1-97AA)
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Polyclonal
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
產品提供形式：

Liquid
應用范圍：

ELISA, IHC

推薦稀釋比：

Application	Recommended Dilution
IHC	1:20-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

功能：

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.
基因功能參考文獻：
1. The results of this study demonistrated that the syndrome of deafness-dystonia is cause by mutation of Timm8a. PMID: 23418071
2. knockdown of the TIMM8A gene by RNA interference did not show an influence on the oxygen respiration rate and the mitochondrial membrane potentia PMID: 21984432
3. Interaction of TIMM8a with the signal transduction adaptor molecule STAM1. PMID: 12745081
4. Intronic mutations in the DDP1 gene can also cause X-linked dystonia-deafness syndrome. PMID: 15710860
5. Bax/Bak-dependent release of DDP/TIMM8a promotes Drp1-mediated mitochondrial fission and mitoptosis during programmed cell death. PMID: 16332536
6. Mutation in TIMM8a is associated with deafness-dystonia (Mohr-Tranebjaerg) syndrome PMID: 16411215
7. A sporadic 42-year-old man with MTS presenting with postlingual deafness, adult-onset progressive dystonia with marked arm tremor, mild spasticity of the legs, and visual disturbance due to a novel mutation in the DDP1 gene. PMID: 17534980
8. mRNA expression demonstrate increased TIMM8A mRNA levels in cultured fibroblasts from a patient with Mohr-Tranebjaerg Syndrome. PMID: 17999202
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相關疾病：

Mohr-Tranebjaerg syndrome (MTS)
亞細胞定位：

Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side.
蛋白家族：

Small Tim family
組織特異性：

Highly expressed in fetal and adult brain, followed by fetal lung, liver and kidney. Also expressed in heart, placenta, lung, liver, kidney, pancreas, skeletal muscle and heart.
數據庫鏈接：

HGNC: 11817

OMIM: 300356

KEGG: hsa:1678

STRING: 9606.ENSP00000361993

UniGene: Hs.447877

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