Any feedback?
Please rate this page
(literature.php)
(0/150)

BRENDA support

Literature summary extracted from

  • Shafqat, N.; Turnbull, A.; Zschocke, J.; Oppermann, U.; Yue, W.W.
    Crystal structures of human HMG-CoA synthase isoforms provide insights into inherited ketogenesis disorders and inhibitor design (2010), J. Mol. Biol., 398, 497-506.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
2.3.3.10 expressed in Escherichia coli BL21(DE3) cells Homo sapiens

Crystallization (Commentary)

EC Number Crystallization (Comment) Organism
2.3.3.10 in complex with (S)-3-hydroxy-3-methylglutaryl-CoA, sitting drop vapor diffusion method, using 0.2 M ammonium sulfate, 0.1 M Bis-Tris (pH 6.5), 25% (w/v) PEG 3350, at 20°C Homo sapiens
2.3.3.10 in complex with Co-A, sitting drop vapor diffusion method, using 0.1 M Bis-Tris (pH 6.5), 25% (w/v) polyethylene glycol (PEG) 3350, 0.2 M ammonium sulfate, at 4°C Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
2.3.3.10 F174L the mutant shows 10000fold decrease of activity compared to the wild type enzyme Homo sapiens
2.3.3.10 G169D the mutant is associated with HMGCS2 deficiency Homo sapiens
2.3.3.10 G212R inactive Homo sapiens
2.3.3.10 G388R the mutant is associated with HMGCS2 deficiency Homo sapiens
2.3.3.10 I407T the mutant is associated with HMGCS2 deficiency Homo sapiens
2.3.3.10 I56N the mutant is associated with HMGCS2 deficiency Homo sapiens
2.3.3.10 K243E the mutant is associated with HMGCS2 deficiency Homo sapiens
2.3.3.10 L266S the mutant is associated with HMGCS2 deficiency Homo sapiens
2.3.3.10 M307T the mutant is associated with HMGCS2 deficiency Homo sapiens
2.3.3.10 R188H the mutant is associated with HMGCS2 deficiency Homo sapiens
2.3.3.10 R424X the truncated mutant is nonfunctional Homo sapiens
2.3.3.10 R500H inactive Homo sapiens
2.3.3.10 R505Q the mutant is associated with HMGCS2 deficiency Homo sapiens
2.3.3.10 T233A the mutant is associated with HMGCS2 deficiency Homo sapiens
2.3.3.10 V54M the mutant is associated with HMGCS2 deficiency Homo sapiens
2.3.3.10 Y167C the mutant is associated with HMGCS2 deficiency Homo sapiens
2.3.3.10 Y84X the truncated mutant is nonfunctional Homo sapiens

Localization

EC Number Localization Comment Organism GeneOntology No. Textmining
2.3.3.10 cytosol isoform HMGCS1 Homo sapiens 5829
-
2.3.3.10 mitochondrion isoform HMGCS2 Homo sapiens 5739
-

Organism

EC Number Organism UniProt Comment Textmining
2.3.3.10 Homo sapiens P54868 isoform HMGCS2
-
2.3.3.10 Homo sapiens Q01581 isoform HMGCS1
-

Purification (Commentary)

EC Number Purification (Comment) Organism
2.3.3.10 Ni-Sepharose resin column chromatography and Superdex 200 gel filtration Homo sapiens

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2.3.3.10 acetyl-CoA + acetoacetyl-CoA + H2O
-
 Homo sapiens (S)-3-hydroxy-3-methylglutaryl-CoA + CoA
-
 ?
2.3.3.10 additional information the enzyme catalyzes a three-step ping-pong reaction involving an acylation, a condensation, and a hydrolysis Homo sapiens ?
-
 ?

Synonyms

EC Number Synonyms Comment Organism
2.3.3.10 3-hydroxy-3-methylglutaryl coenzyme A synthase
-
 Homo sapiens
2.3.3.10 HMG-CoA synthase
-
 Homo sapiens
2.3.3.10 HMGCS
-
 Homo sapiens
2.3.3.10 HMGCS1 isoform Homo sapiens
2.3.3.10 HMGCS2 isoform Homo sapiens