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Mouse Anti-COASY Recombinant Antibody (3B3) (CBMAB-C5707-LY)

This product is antibody recognizes COASY. The antibody 3B3 immunoassay techniques such as: IF, FC, IHC-F, WB.
See all COASY antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
3B3
Antibody Isotype
IgG2a
Application
IF, FC, IHC-F, WB

Basic Information

Immunogen
COASY antibody was raised in Mouse using full length human recombinant protein of human COASY produced in HEK293T cell as the immunogen
Specificity
Human
Antibody Isotype
IgG2a
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
Liquid
Buffer
1% BSA, 50% glycerol
Preservative
0.02% sodium azide
Concentration
0.5-1 mg/ml
Purity
> 95% Purity determined 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.

Target

Full Name
Coenzyme A Synthase
Introduction
COASY (Coenzyme A Synthase) is a Protein Coding gene. Diseases associated with COASY include Neurodegeneration With Brain Iron Accumulation 6 and Coasy Protein-Associated Neurodegeneration. Among its related pathways are Metabolism of water-soluble vitamins and cofactors and Metabolism. Gene Ontology (GO) annotations related to this gene include dephospho-CoA kinase activity and pantetheine-phosphate adenylyltransferase activity.
Entrez Gene ID
80347
UniProt ID
Q13057
Alternative Names
Coenzyme A Synthase; CoA Synthase; POV-2; NBP; Bifunctional Phosphopantetheine Adenylyl Transferase/Dephospho CoA Kinase; Phosphopantetheine Adenylyltransferase / Dephosphocoenzyme A Kinase; Bifunctional Coenzyme A Synthase;
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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