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TIMM8A Antibody

Datasheet
Code CSB-PA023557ESR2HU
Size US$166
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Image
  • Immunohistochemistry(IHC) 1- TIMM8A Antibody

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

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Product Details

Full Product Name
Rabbit anti-Homo sapiens (Human) TIMM8A Polyclonal antibody
Uniprot No.
O60220
Target Names
TIMM8A
Alternative Names
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
Raised in
Rabbit
Species Reactivity
Human
Immunogen
Recombinant Human Mitochondrial import inner membrane translocase subunit Tim8 A protein (1-97AA)
Immunogen Species
Homo sapiens (Human)
Conjugate
Non-conjugated
Clonality
Polyclonal
Isotype
IgG
Purification Method
Antigen Affinity Purified
Concentration
It differs from different batches. Please contact us to confirm it.
Buffer
PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Form
Liquid
Tested Applications
ELISA, IHC
Recommended Dilution
Application Recommended Dilution
IHC 1:20-1:200
Protocols
ELISA Protocol
Immunohistochemistry (IHC) Protocol
Troubleshooting and FAQs
Antibody FAQs
Storage
Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time
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.
Usage
For Research Use Only. Not for use in diagnostic or therapeutic procedures.

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TIMM8A Antibodies

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Target Background

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.
Gene References into Functions
  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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Involvement in disease
Mohr-Tranebjaerg syndrome (MTS)
Subcellular Location
Mitochondrion inner membrane; Peripheral membrane protein; Intermembrane side.
Protein Families
Small Tim family
Tissue Specificity
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.
Database Links

HGNC: 11817

OMIM: 300356

KEGG: hsa:1678

STRING: 9606.ENSP00000361993

UniGene: Hs.447877

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