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MIPEP

The product of this gene performs the final step in processing a specific class of nuclear-encoded proteins targeted to the mitochondrial matrix or inner membrane. This protein is primarily involved in the maturation of oxidative phosphorylation (OXPHOS)-related proteins. This gene may contribute to the functional effects of frataxin deficiency and the clinical manifestations of Friedreich ataxia. [provided by RefSeq]

Full Name

mitochondrial intermediate peptidase

Function

Cleaves proteins, imported into the mitochondrion, to their mature size.

Biological Process

Peptide metabolic process Source: GO_Central
Protein processing involved in protein targeting to mitochondrion Source: GO_Central
Proteolysis Source: GO_Central

Cellular Location

Mitochondrion matrix

Involvement in disease

Combined oxidative phosphorylation deficiency 31 (COXPD31):
An autosomal recessive, severe mitochondrial disease with multisystemic manifestations appearing soon after birth or in early infancy. Clinical features include left ventricular non-compaction, global developmental delay, severe hypotonia, seizures, cataract, and abnormal movements. Death may occur in early childhood.

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Anti-MIPEP antibodies

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Mouse Anti-MIPEP (AA 611-713) Recombinant Antibody (CBFYM-2236) (CBMAB-M2419-FY)

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Target: MIPEP

Host: Mouse

Antibody Isotype: IgG2a, κ

Specificity: Human

Clone: CBFYM-2236

Application*: E, WB

Mouse Anti-MIPEP Recombinant Antibody (CBFYM-0066) (CBMAB-M0074-FY)

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Target: MIPEP

Host: Mouse

Antibody Isotype: IgG1, κ

Specificity: Human

Clone: CBFYM-0066

Application*: WB

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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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