Cell Biology Antibodies 12

Anti-TIMM8A Antibody (CAB9811)

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SKU:
CAB9811
Product Type:
Antibody
Applications:
WB
Reactivity:
Human
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Research Area:
Cell Biology

Description

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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  
Anti-TIMM8A Polyclonal Antibody (CAB9811)Western blot analysis of extracts of various cell lines, using TIMM8A antibody (CAB9811) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.
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