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Hydroxymethylglutaryl-coenzyme A synthase (EC 2.3.3.10) (HMG-CoA synthase) catalyzes the condensation of acetyl-CoA with acetoacetyl-CoA to produce HMG-CoA and CoA [1].

In vertebrates there are two isozymes located in different subcellular compartments: a cytosolic form which is the starting point of the mevalonate pathway which leads to cholesterol and other sterolic and isoprenoid compounds and a mitochondrial form responsible for ketone body biosynthesis. HMG-CoA is also found in other eukaryotes such as insect, plants and fungi.

A cysteine is known to act as the catalytic nucleophile in the first step of the reaction, the acetylation of the enzyme by acetyl-CoA. We have used the conserved region around this active site residue as a signature pattern.

April 2003 / Text revised.

PROSITE method (with tools and information) covered by this documentation:

HMG_COA_SYNTHASE, PS01226; Hydroxymethylglutaryl-coenzyme A synthase active site  (PATTERN)

Consensus pattern: N-x-[DN]-[IV]-E-G-[IV]-D-x(2)-N-A-C-[FY]-x-G C is the active site residue Sequences known to belong to this class detected by the pattern: ALL Other sequence(s) detected in Swiss-Prot: NONE

• Retrieve an alignment of Swiss-Prot true positive hits:   Clustal format, color, condensed view  / Clustal format, color  / Clustal format, plain text  / Fasta format • Retrieve the sequence logo from the alignment • Taxonomic tree view of all Swiss-Prot/TrEMBL entries matching PS01226 • Retrieve a list of all Swiss-Prot/TrEMBL entries matching PS01226 • Scan Swiss-Prot/TrEMBL entries against PS01226 • view ligand binding statistics

Matching PDB structures: 2WYA [ALL]

1	Authors	Rokosz L.L., Boulton D.A., Butkiewicz E.A., Sanyal G., Cueto M.A., Lachance P.A., Hermes J.D.
	Title	Human cytoplasmic 3-hydroxy-3-methylglutaryl coenzyme A synthase: expression, purification, and characterization of recombinant wild-type and Cys129 mutant enzymes.
	Source	Arch. Biochem. Biophys. 312:1-13(1994).
	PubMed ID	7913309

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