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Mouse Anti-CLPP Recombinant Antibody (CBWJC-2817) (CBMAB-C3934WJ)

This product is a Mouse antibody that recognizes CLPP. This antibody CBWJC-2817 can be used for immunoassay techniques such as: ELISA, WB.
See all CLPP antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBWJC-2817
Antibody Isotype
IgG2b
Application
ELISA, WB

Basic Information

Specificity
Human
Antibody Isotype
IgG2b
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Caseinolytic Mitochondrial Matrix Peptidase Proteolytic Subunit
Introduction
CLPP (Caseinolytic Mitochondrial Matrix Peptidase Proteolytic Subunit) is a Protein Coding gene. Diseases associated with CLPP include Perrault Syndrome 3 and Perrault Syndrome. Among its related pathways are Glucose / Energy Metabolism and Neuroscience. Gene Ontology (GO) annotations related to this gene include identical protein binding and peptidase activity.
Entrez Gene ID
8192
UniProt ID
Q16740
Alternative Names
DFNB81;PRLTS3;ClpP
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.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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