Rabbit Anti-CLPX Recombinant Antibody (CBWJC-2045) (V2LY-1206-LY292)

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Basic Information

Host Animal
Rabbit
Clone
CBWJC-2045
Application
IF, IHC, WB
Immunogen
Synthetic peptide within human CLPX aa 584-633/633.
Host Species
Rabbit
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG
Clonality
Monoclonal Antibody
Application Notes
ApplicationNote
WB1:500
IHC1:200-1:1,000
IF(ICC)1:50

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

Format
Liquid
Buffer
TBS, pH 7.4, 0.05% BSA, 40% glycerol
Preservative
0.05% sodium azide
Concentration
1 mg/ml
Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.
More Infomation

Target

Full Name
caseinolytic mitochondrial matrix peptidase chaperone subunit
Entrez Gene ID
Human10845
Mouse270166
Rat300786
UniProt ID
HumanO76031
MouseQ9JHS4
RatQ5U2U0
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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