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TIMM8A

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

Full Name

translocase of inner mitochondrial membrane 8 homolog A (yeast)

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.

Biological Process

Biological Process nervous system developmentSource:ProtInc1 Publication
Biological Process protein insertion into mitochondrial inner membraneSource:BHF-UCL1 Publication

Cellular Location

Mitochondrion inner membrane

Involvement in disease

Mohr-Tranebjaerg syndrome (MTS):
An X-linked recessive disorder characterized by postlingual sensorineural deafness with onset in early childhood, dystonia, spasticity, dysphagia, mental deterioration, paranoia and cortical blindness.

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Anti-TIMM8A antibodies

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Mouse Anti-TIMM8A Recombinant Antibody (2F11) (CBMAB-A9102-LY)

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Target: TIMM8A

Host: Mouse

Antibody Isotype: IgG2a, κ

Specificity: Human

Clone: 2F11

Application*: WB, E

Mouse Anti-TIMM8A Recombinant Antibody (CBYJT-3181) (CBMAB-T2455-YJ)

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Target: TIMM8A

Host: Mouse

Antibody Isotype: IgG2a, κ

Specificity: Human

Clone: CBYJT-3181

Application*: E, IH, WB

Mouse Anti-TIMM8A Recombinant Antibody (CBYJT-3180) (CBMAB-T2454-YJ)

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Target: TIMM8A

Host: Mouse

Antibody Isotype: IgG2a, κ

Specificity: Human

Clone: CBYJT-3180

Application*: E, WB

Mouse Anti-TIMM8A Recombinant Antibody (CBYJT-3179) (CBMAB-T2453-YJ)

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Target: TIMM8A

Host: Mouse

Antibody Isotype: IgG2a, κ

Specificity: Human

Clone: CBYJT-3179

Application*: E, WB

Mouse Anti-TIMM8A (C-terminus) Recombinant Antibody (CBFYM-3157) (CBMAB-M3354-FY)

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Target: TIMM8A

Host: Mouse

Antibody Isotype: IgG1

Specificity: Mouse, Rat, Human

Clone: CBFYM-3157

Application*: WB, IP

Mouse Anti-TIMM8A Recombinant Antibody (9G347) (CBMAB-D2276-YC)

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Target: TIMM8A

Host: Mouse

Antibody Isotype: IgG

Specificity: Human

Clone: 9G347

Application*: E, IH, WB

Human TIMM8A ELISA Kit (V2LY-0626-LY5931)

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Target: TIMM8A

Sensitivity: 0.0096 ng/mL

Detection Range: 0.02-3.5 ng/mL

Sample Type: Serum, Plasma, cell culture supernates

Specificity: Human

Assay Type: Sandwich

Reactivity: Human

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(P): Predicted

* Abbreviations

AActivation
AGAgonist
APApoptosis
BBlocking
BABioassay
BIBioimaging
CImmunohistochemistry-Frozen Sections
CIChromatin Immunoprecipitation
CTCytotoxicity
CSCostimulation
DDepletion
DBDot Blot

EELISA
ECELISA(Cap)
EDELISA(Det)
ESELISpot
EMElectron Microscopy
FFlow Cytometry
FNFunction Assay
GSGel Supershift
IInhibition
IAEnzyme Immunoassay
ICImmunocytochemistry
IDImmunodiffusion
IEImmunoelectrophoresis

IFImmunofluorescence
IGImmunochromatography
IHImmunohistochemistry
IMImmunomicroscopy
IOImmunoassay
IPImmunoprecipitation
ISIntracellular Staining for Flow Cytometry
LALuminex Assay
LFLateral Flow Immunoassay
MMicroarray
MCMass Cytometry/CyTOF
MDMeDIP

MSElectrophoretic Mobility Shift Assay
NNeutralization
PImmunohistologyp-Paraffin Sections
PAPeptide Array
PEPeptide ELISA
PLProximity Ligation Assay
RRadioimmunoassay
SStimulation
SESandwich ELISA
SHIn situ hybridization
TCTissue Culture
WBWestern Blot

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