WH0001678M1
Monoclonal Anti-TIMM8A antibody produced in mouse
clone 2F11, purified immunoglobulin, buffered aqueous solution
别名:
Anti-DDP, Anti-DDP1, Anti-DFN1, Anti-MGC12262, Anti-MTS, Anti-translocase of inner mitochondrial membrane 8 homolog A (yeast)
产品名称
Monoclonal Anti-TIMM8A antibody produced in mouse, clone 2F11, purified immunoglobulin, buffered aqueous solution
biological source
mouse
conjugate
unconjugated
antibody form
purified immunoglobulin
antibody product type
primary antibodies
clone
2F11, monoclonal
form
buffered aqueous solution
species reactivity
human
technique(s)
immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable
indirect ELISA: suitable
western blot: 1-5 μg/mL
isotype
IgG2aκ
GenBank accession no.
UniProt accession no.
shipped in
dry ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... TIMM8A(1678)
Application
Monoclonal Anti-TIMM8A antibody produced in mouse has been used in immunoblotting.
Biochem/physiol Actions
Translocase of inner mitochondrial membrane 8A (TIMM8A) is involved in the dynamin-1-like protein (Drp1)-mediated mitochondrial fission during programmed cell death. It interacts with TIMM13 and aids the import of TIMM23 into mitochondria.{152)
General description
Translocase of inner mitochondrial membrane 8A (TIMM8A) 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. TIMM8A gene is located on human chromosome Xq22. It is a 11 kDa protein. It belongs to a family of evolutionary conserved proteins, which are arranged in hetero-oligomeric complexes in the mitochondrial intermembrane space.
Immunogen
TIMM8A (NP_004076, 9 a.a. ~ 97 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
AAGLGAVDPQLQHFIEVETQKQRFQQLVHQMTELCWEKCMDKPGPKLDSRAEACFVNCVERFIDTSQFILNRLEQTQKSKPVFSESLSD
Sequence
AAGLGAVDPQLQHFIEVETQKQRFQQLVHQMTELCWEKCMDKPGPKLDSRAEACFVNCVERFIDTSQFILNRLEQTQKSKPVFSESLSD
Physical form
Solution in phosphate buffered saline, pH 7.4
Legal Information
GenBank is a registered trademark of United States Department of Health and Human Services
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存储类别
10 - Combustible liquids
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
法规信息
常规特殊物品
此项目有
A Spanish sporadic case of deafness-dystonia (Mohr-Tranebjaerg) syndrome with a novel mutation in the gene encoding TIMM8a, a component of the mitochondrial protein translocase complexes
Aguirre LA, et al.
Neuromuscular Disorders, 18(12), 979-981 (2008)
Bax/Bak-dependent release of DDP/TIMM8a promotes Drp1-mediated mitochondrial fission and mitoptosis during programmed cell death
Arnoult D, et al.
Current Biology, 15(23), 2112-2118 (2005)
The C66W mutation in the deafness dystonia peptide 1 (DDP1) affects the formation of functional DDP1.TIM13 complexes in the mitochondrial intermembrane space
Hofmann S, et al.
The Journal of Biological Chemistry, 277(26), 23287-23293 (2002)
Baris Bingol et al.
Nature, 510(7505), 370-375 (2014-06-05)
Cells maintain healthy mitochondria by degrading damaged mitochondria through mitophagy; defective mitophagy is linked to Parkinson's disease. Here we report that USP30, a deubiquitinase localized to mitochondria, antagonizes mitophagy driven by the ubiquitin ligase parkin (also known as PARK2) and
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