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Mouse Anti-COASY Recombinant Antibody (CBYY-C2970) (CBMAB-C4413-YY)

This product is mouse antibody that recognizes COASY. The antibody CBYY-C2970 can be used for immunoassay techniques such as: FC, WB
See all COASY antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBYY-C2970
Antibody Isotype
IgG2b
Application
FC, WB

Basic Information

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
Immunoaffinity purified
Buffer
0.58 mg/mL
Preservative
50% glycerol, 1% BSA, PBS, pH 7.3
Concentration
Liquid
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
Coenzyme A Synthase
Entrez Gene ID
80347
UniProt ID
Q13057
Alternative Names
Collagen Type XI Alpha 2 Chain; Collagen, Type XI, Alpha 2; Pro-A2 Chain Of Collagen Type XI; Collagen Alpha-2(XI) Chain; OSMEDA; OSMEDB; DFNA13;
Function
Bifunctional enzyme that catalyzes the fourth and fifth sequential steps of CoA biosynthetic pathway. The fourth reaction is catalyzed by the phosphopantetheine adenylyltransferase, coded by the coaD domain; the fifth reaction is catalyzed by the dephospho-CoA kinase, coded by the coaE domain. May act as a point of CoA biosynthesis regulation.
Biological Process
Coenzyme A biosynthetic process Source: UniProtKB
Cellular Location
Mitochondrion matrix; Cytoplasm. The protein is mainly present in the mitochondrial matrix, probably anchored to the inner mitochondrial membrane, but is also present in cell lysate.
Involvement in disease
Neurodegeneration with brain iron accumulation 6 (NBIA6):
A neurodegenerative disorder associated with iron accumulation in the brain, primarily in the basal ganglia. It is characterized by progressive motor and cognitive dysfunction beginning in childhood or young adulthood. Patients show extrapyramidal motor signs, such as spasticity, dystonia, and parkinsonism.
Pontocerebellar hypoplasia 12 (PCH12):
A form of pontocerebellar hypoplasia, a disorder characterized by structural defects of the pons and cerebellum, evident upon brain imaging. PCH12 is an autosomal recessive form characterized by onset in utero and death in infancy. Brain imaging shows microcephaly, cerebellar hypoplasia, micrognathia, and multiple contractures.
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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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