Rabbit Anti-CLPX Recombinant Antibody (CBWJC-2045) (CBMAB-C2682WJ)

This product is a Rabbit antibody that recognizes CLPX. This antibody CBWJC-2045 can be used for immunoassay techniques such as: WB, ICC.
See all CLPX antibodies

Datasheet

Summary

Host Animal

Rabbit

Specificity

Human, Mouse, Rat

Clone

CBWJC-2045

Antibody Isotype

IgG

Application

WB, ICC

Basic Information

Specificity

Human, Mouse, Rat

Antibody Isotype

IgG

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!]

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 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

Human	10845
Mouse	270166
Rat	300786

UniProt ID

Human	O76031
Mouse	Q9JHS4
Rat	Q5U2U0

Alternative Names

Caseinolytic Mitochondrial Matrix Peptidase Chaperone Subunit; ClpX (Caseinolytic Protease X, E. Coli) Homolog; ClpX Caseinolytic Peptidase X Homolog (E. Coli); ClpX Caseinolytic Protease X Homolog (E. Coli);

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.

References

Rondelli, C. M., Perfetto, M., Danoff, A., Bergonia, H., Gillis, S., O'Neill, L., ... & Yien, Y. Y. (2021). The ubiquitous mitochondrial protein unfoldase CLPX regulates erythroid heme synthesis by control of iron utilization and heme synthesis enzyme activation and turnover. Journal of Biological Chemistry, 297(2).

Nomura, K., Kitagawa, Y., Aihara, M., Ohki, Y., Furuyama, K., & Hirokawa, T. (2021). Heme‐dependent recognition of 5‐aminolevulinate synthase by the human mitochondrial molecular chaperone ClpX. FEBS letters.

Key, J., Torres-Odio, S., Bach, N. C., Gispert, S., Koepf, G., Reichlmeir, M., ... & Auburger, G. (2021). Inactivity of Peptidase ClpP Causes Primary Accumulation of Mitochondrial Disaggregase ClpX with Its Interacting Nucleoid Proteins, and of mtDNA. Cells, 10(12), 3354.

Serpeloni, F., Nätt, D., Assis, S. G. D., Wieling, E., & Elbert, T. (2020). Experiencing community and domestic violence is associated with epigenetic changes in DNA methylation of BDNF and CLPX in adolescents. Psychophysiology, 57(1), e13382.

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Protocols

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Associated Products

Related Products

Mouse Anti-CLPX Recombinant Antibody (11C11)

Isotype control

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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