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Rabbit Anti-TIMM8A Antibody (CSB-PA023557ESR2HU)
Supplier: CUSABIO BIOTECH CO.
Mitochondrial import inner membrane translocase subunit Tim8 A Antibody,Deafness dystonia protein 1 Antibody,X-linked deafness dystonia protein Antibody,TIMM8A Antibody,DDP Antibody,DDP1 Antibody,TIM8A AntibodyMore alternative names for the antibody
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
Anti-TIMM8A antibody (ab168835)
Anti-TIMM8A antibody (ab168835)
Recommended applications: ELISA, IHC
Recommended dilution: Recommended dilution:IHC:1:20-1:200
Recommended protocols: check protocols
|Catalogue No.|| |
Recombinant human Mitochondrial import inner membrane translocase subunit Tim8 A protein (1-97AA)
|Recommended dilution|| |
|Molecular weight|| |
Antigen Affinity Purified
Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.
|Database links|| |
Entrez Gene:738690( Chimpanzee), Entrez Gene:101135568( Gorilla), Entrez Gene:1678( Human), Entrez Gene:30058( Mouse), Entrez Gene:100443133( Orangutan), Entrez Gene:702885( Rhesus monkey), GenBank:NP_004076( Human), Omim:300356( Human), SwissProt:O60220( Human), SwissProt:Q9WVA2( Mouse), Unigene:447877( Human), Unigene:214504( Mouse)
|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. .
|Protein 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.
|Involvement in disease|| |
Mohr-Tranebjaerg syndrome (MTS) [MIM:304700]: An X-linked recessive disorder characterized by postlingual sensorineural deafness with onset in early childhood, dystonia, spasticity, dysphagia, mental deterioration, paranoia and cortical blindness. . Note=The disease is caused by mutations affecting the gene represented in this entry.
|Protein sequence and domain|| |
Belongs to the small Tim family. The twin CX3C motif contains 4 conserved Cys residues that form 2 disulfide bonds in the mitochondrial intermembrane space. However, during the transit of TIMM8A from cytoplasm into mitochondrion, the Cys residues probably coordinate zinc, thereby preventing folding and allowing its transfer across mitochondrial outer membrane (By similarity).
|Protein cellular localization|| |
Mitochondrion inner membrane ; Peripheral membrane protein ; Intermembrane side .
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|Product type|| |
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