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Mouse Anti-CLPX Recombinant Antibody (11C11) (CBMAB-BR135LY)

The product is antibody recognizes CLPX. The antibody 11C11 immunoassay techniques such as: WB.
See all CLPX antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
11C11
Antibody Isotype
IgG2b
Application
WB: 0.1-0.5 μg/ml

Basic Information

Immunogen
E. coli-derived human CLPX recombinant protein (Position: A337-E574)
Specificity
Human
Antibody Isotype
IgG2b
Clonality
Monoclonal
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!]

Format
Lyophilized
Preservative
0.05 mg sodium azide
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
caseinolytic mitochondrial matrix peptidase chaperone subunit
Introduction
The protein encoded by this gene is part of a protease found in mitochondria. This protease is ATP-dependent and targets specific proteins for degradation. The protease consists of two heptameric rings of the CLPP catalytic subunit sandwiched between two hexameric rings of the chaperone subunit encoded by this gene. Targeted proteins are unwound by this protein and then passed on to the CLPP subunit for degradation. Two transcript variants, one protein-coding and the other non-protein coding, have been found for this gene. [provided by RefSeq, Nov 2015]
Entrez Gene ID
10845
UniProt ID
O76031
Alternative Names
ATP-dependent Clp protease ATP-binding subunit clpX-like, mitochondrial; CLPX
Function
ATP-dependent specificity component of the Clp protease complex. Hydrolyzes ATP (PubMed:28874591).

Targets specific substrates for degradation by the Clp complex (PubMed:11923310, PubMed:22710082).

Can perform chaperone functions in the absence of CLPP. Enhances the DNA-binding activity of TFAM and is required for maintaining a normal mitochondrial nucleoid structure (PubMed:22841477).

ATP-dependent unfoldase that stimulates the incorporation of the pyridoxal phosphate cofactor into 5-aminolevulinate synthase, thereby activating 5-aminolevulinate (ALA) synthesis, the first step in heme biosynthesis (PubMed:28874591).

Important for efficient erythropoiesis through upregulation of heme biosynthesis (PubMed:25957689, PubMed:28874591).
Biological Process
ATP metabolic process Source: UniProtKB
Protein catabolic process Source: GO_Central
Protein folding Source: InterPro
Proteolysis Source: GO_Central
Proteolysis involved in cellular protein catabolic process Source: UniProtKB
Cellular Location
Mitochondrion; Mitochondrion nucleoid
Involvement in disease
Protoporphyria, erythropoietic, 2 (EPP2):
An autosomal dominant form of porphyria with onset in infancy. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. Erythropoietic protoporphyria is marked by excessive protoporphyrin in erythrocytes, plasma, liver and feces, and by widely varying photosensitive skin changes ranging from a burning or pruritic sensation to erythema, edema and wheals.
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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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