catalog number :
MBS713333
products full name :
Rabbit anti-human Coenzyme A synthase polyclonal Antibody
products short name :
Coenzyme A synthase
products name syn :
Coenzyme A synthase; COASY; DPCK; FLJ35179; NBP; PPAT; UKR1; pOV-2
other names :
Coenzyme A synthase; Bifunctional coenzyme A synthase; bifunctional coenzyme A synthase; nucleotide binding protein; phosphopantetheine adenylyltransferase / dephosphocoenzyme A kinase; bifunctional phosphopantetheine adenylyl transferase/dephospho CoA kinase; CoA synthase; NBP; POV-2Including the following 2 domains:Phosphopantetheine adenylyltransferase (EC:2.7.7.3)Alternative name(s):Dephospho-CoA pyrophosphorylase; Pantetheine-phosphate adenylyltransferase; PPAT
other gene names :
COASY; COASY; NBP; DPCK; PPAT; UKR1; NBIA6; pOV-2; CoA synthase; PPAT; DPCK; DPCOAK
uniprot entry name :
COASY_HUMAN
reactivity :
Human, Mouse, Rat
purity :
Antigen Affinity Purified
tested application :
ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
other info1 :
Immunogen: Human COASY
other info2 :
Storage Buffer: PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20 degree C, Avoid freeze / thaw cycles.
ncbi acc num :
AAH67254.1
ncbi mol weight :
65,339 Da
ncbi pathways :
Coenzyme A Biosynthesis Pathway (106255); Coenzyme A Biosynthesis, Pantothenate = CoA Pathway (413397); Coenzyme A Biosynthesis, Pantothenate = CoA Pathway (468312); Defective AMN Causes Hereditary Megaloblastic Anemia 1 Pathway (906000); Defective BTD Causes Biotidinase Deficiency Pathway (906015); Defective CD320 Causes Methylmalonic Aciduria Pathway (906012); Defective CUBN Causes Hereditary Megaloblastic Anemia 1 Pathway (906001); Defective GIF Causes Intrinsic Factor Deficiency Pathway (906004); Defective HLCS Causes Multiple Carboxylase Deficiency Pathway (906014); Defective LMBRD1 Causes Methylmalonic Aciduria And Homocystinuria Type CblF Pathway (906003)
ncbi summary :
Coenzyme A (CoA) functions as a carrier of acetyl and acyl groups in cells and thus plays an important role in numerous synthetic and degradative metabolic pathways in all organisms. In eukaryotes, CoA and its derivatives are also involved in membrane trafficking and signal transduction. This gene encodes the bifunctional protein coenzyme A synthase (CoAsy) which carries out the last two steps in the biosynthesis of CoA from pantothenic acid (vitamin B5). The phosphopantetheine adenylyltransferase domain of this bifunctional protein catalyzes the conversion of 4'-phosphopantetheine into dephospho-coenzyme A (dpCoA) while its dephospho-CoA kinase domain completes the final step by phosphorylating dpCoA to form CoA. Mutations in this gene are associated with neurodegeneration with brain iron accumulation (NBIA). Alternative splicing results in multiple isoforms. [provided by RefSeq, Apr 2014]
uniprot summary :
PPAT: bifunctional cytoplasmic 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. Protein type: Transferase; EC 2.7.7.3; Kinase, other; EC 2.7.1.24; Cofactor and Vitamin Metabolism - pantothenate and CoA biosynthesis. Chromosomal Location of Human Ortholog: 17q12-q21. Cellular Component: nucleoplasm; mitochondrial outer membrane; mitochondrial matrix; cytoplasm. Molecular Function: protein binding; dephospho-CoA kinase activity; pantetheine-phosphate adenylyltransferase activity; ATP binding. Biological Process: coenzyme A biosynthetic process; coenzyme biosynthetic process; vitamin metabolic process; pantothenate metabolic process; phosphorylation; water-soluble vitamin metabolic process. Disease: Neurodegeneration With Brain Iron Accumulation 6