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CLPP

The protein encoded by this gene belongs to the peptidase family S14 and hydrolyzes proteins into small peptides in the presence of ATP and magnesium. The protein is transported into mitochondrial matrix and is associated with the inner mitochondrial membrane. [provided by RefSeq, Jul 2008]
Full Name
Caseinolytic Mitochondrial Matrix Peptidase Proteolytic Subunit
Function
Protease component of the Clp complex that cleaves peptides and various proteins in an ATP-dependent process. Has low peptidase activity in the absence of CLPX. The Clp complex can degrade CSN1S1, CSN2 and CSN3, as well as synthetic peptides (in vitro) and may be responsible for a fairly general and central housekeeping function rather than for the degradation of specific substrates (PubMed:11923310, PubMed:15522782).

Cleaves PINK1 in the mitochondrion (PubMed:22354088).
Biological Process
Membrane protein proteolysis Source: UniProtKB
Protein quality control for misfolded or incompletely synthesized proteins Source: GO_Central
Proteolysis involved in cellular protein catabolic process Source: UniProtKB
Cellular Location
Mitochondrion matrix
Involvement in disease
Perrault syndrome 3 (PRLTS3):
A sex-influenced disorder characterized by sensorineural deafness in both males and females, and ovarian dysgenesis in females. Affected females have primary amenorrhea, streak gonads, and infertility, whereas affected males show normal pubertal development and are fertile. A spectrum of additional clinical features, including cerebellar ataxia, learning disability, and peripheral neuropathy, have been described in some PRLTS3 affected individuals.

Anti-CLPP antibodies

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Target: CLPP
Host: Mouse
Antibody Isotype: IgG3
Specificity: Human, Pig
Clone: 2E1D9
Application*: E, C, WB
Target: CLPP
Host: Mouse
Antibody Isotype: IgG1
Specificity: Dog, Human, Monkey
Clone: 1E2
Application*: WB, F
Target: CLPP
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human, Mouse, Rat
Clone: CBWJC-2821
Application*: WB, IP, F
Target: CLPP
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Dog, Rat, Monkey
Clone: CBWJC-2820
Application*: WB, IH, IF, F
Target: CLPP
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Monkey
Clone: CBWJC-2819
Application*: WB, IF, F
Target: CLPP
Host: Mouse
Antibody Isotype: IgG3
Specificity: Human, Pig
Clone: CBXC-2740
Application*: E, IH, WB
Target: CLPP
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human, Dog, Rat, Monkey
Clone: CBXC-1953
Application*: WB, IF, F
Target: CLPP
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human, Dog, Rat, Monkey
Clone: CBXC-1963
Application*: WB, IF, F
Target: CLPP
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBXC-1944
Application*: WB, IH, F
Target: CLPP
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBFYC-1958
Application*: F, IF, P, WB
Target: CLPP
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: CBFYC-1957
Application*: F, IF, WB
Target: CLPP
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBFYC-1956
Application*: F, IH, WB
Target: CLPP
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBFYC-1955
Application*: E, WB
Target: CLPP
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human, Dog, Mouse, Rat
Clone: CBFYC-1954
Application*: F, IF, WB
Target: CLPP
Host: Mouse
Antibody Isotype: IgG3
Specificity: Human
Clone: 555
Application*: ELISA, IHC, WB
Target: CLPP
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBCNC-027
Application*: E, WB
Target: CLPP
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBWJC-2817
Application*: E, WB
Target: CLPP
Host: Mouse
Antibody Isotype: IgG2b, κ
Specificity: Human
Clone: 3E2
Application*: E, P, WB
Target: CLPP
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 1G9
Application*: F, P, WB
Target: CLPP
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human, Rat, Dog
Clone: 1F6
Application*: WB, F, IH, IC/IF, P
Target: CLPP
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 1E8
Application*: F, IF, WB
Target: CLPP
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: 1D8
Application*: F, IF, WB
Target: CLPP
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 1C7
Application*: F, C, WB
Target: CLPP
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: 10B2264
Application*: E, WB
For Research Use Only. Not For Clinical Use.
(P): Predicted
* Abbreviations
IFImmunofluorescence
IHImmunohistochemistry
IPImmunoprecipitation
WBWestern Blot
EELISA
MMicroarray
CIChromatin Immunoprecipitation
FFlow Cytometry
FNFunction Assay
IDImmunodiffusion
RRadioimmunoassay
TCTissue Culture
GSGel Supershift
NNeutralization
BBlocking
AActivation
IInhibition
DDepletion
ESELISpot
DBDot Blot
MCMass Cytometry/CyTOF
CTCytotoxicity
SStimulation
AGAgonist
APApoptosis
IMImmunomicroscopy
BABioassay
CSCostimulation
EMElectron Microscopy
IEImmunoelectrophoresis
PAPeptide Array
ICImmunocytochemistry
PEPeptide ELISA
MDMeDIP
SHIn situ hybridization
IAEnzyme Immunoassay
SEsandwich ELISA
PLProximity Ligation Assay
ECELISA(Cap)
EDELISA(Det)
BIBioimaging
IOImmunoassay
LFLateral Flow Immunoassay
LALuminex Assay
CImmunohistochemistry-Frozen Sections
PImmunohistologyp-Paraffin Sections
ISIntracellular Staining for Flow Cytometry
MSElectrophoretic Mobility Shift Assay
RIRNA Binding Protein Immunoprecipitation (RIP)
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