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WH0001678M1

Sigma-Aldrich

Monoclonal Anti-TIMM8A antibody produced in mouse

clone 2F11, purified immunoglobulin, buffered aqueous solution

Synonym(s):

Anti-DDP, Anti-DDP1, Anti-DFN1, Anti-MGC12262, Anti-MTS, Anti-translocase of inner mitochondrial membrane 8 homolog A (yeast)

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100 μG
$406.00

$406.00

Ships within 2 weeks. (Orders outside of US and Europe, please allow an additional 1-2 weeks for delivery)
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100 μG
$406.00

About This Item

UNSPSC Code:
12352203
NACRES:
NA.41

$406.00

Ships within 2 weeks. (Orders outside of US and Europe, please allow an additional 1-2 weeks for delivery)

biological source

mouse

Quality Level

100

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.

NM_004085

UniProt accession no.

O60220

shipped in

dry ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... TIMM8A(1678)

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

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)

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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Storage Class

10 - Combustible liquids

flash_point_f

Not applicable

flash_point_c

Not applicable

ppe

Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)

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Certificates of Analysis (COA)

Lot/Batch Number
06069-2F11

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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)
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)
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)
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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