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Literature summary for 2.3.3.10 extracted from
- Crystal structures of human HMG-CoA synthase isoforms provide insights into inherited ketogenesis disorders and inhibitor design (2010), J. Mol. Biol., 398, 497-506.
Cloned(Commentary)
| Cloned (Comment) | Organism |
|---|---|
| expressed in Escherichia coli BL21(DE3) cells | Homo sapiens |
Crystallization (Commentary)
| Crystallization (Comment) | Organism |
|---|---|
| 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 |
| 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
| Protein Variants | Comment | Organism |
|---|---|---|
| F174L | the mutant shows 10000fold decrease of activity compared to the wild type enzyme | Homo sapiens |
| G169D | the mutant is associated with HMGCS2 deficiency | Homo sapiens |
| G212R | inactive | Homo sapiens |
| G388R | the mutant is associated with HMGCS2 deficiency | Homo sapiens |
| I407T | the mutant is associated with HMGCS2 deficiency | Homo sapiens |
| I56N | the mutant is associated with HMGCS2 deficiency | Homo sapiens |
| K243E | the mutant is associated with HMGCS2 deficiency | Homo sapiens |
| L266S | the mutant is associated with HMGCS2 deficiency | Homo sapiens |
| M307T | the mutant is associated with HMGCS2 deficiency | Homo sapiens |
| R188H | the mutant is associated with HMGCS2 deficiency | Homo sapiens |
| R424X | the truncated mutant is nonfunctional | Homo sapiens |
| R500H | inactive | Homo sapiens |
| R505Q | the mutant is associated with HMGCS2 deficiency | Homo sapiens |
| T233A | the mutant is associated with HMGCS2 deficiency | Homo sapiens |
| V54M | the mutant is associated with HMGCS2 deficiency | Homo sapiens |
| Y167C | the mutant is associated with HMGCS2 deficiency | Homo sapiens |
| Y84X | the truncated mutant is nonfunctional | Homo sapiens |
Localization
| Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|
| cytosol | isoform HMGCS1 | Homo sapiens | 5829 | - |
| mitochondrion | isoform HMGCS2 | Homo sapiens | 5739 | - |
Organism
| Organism | UniProt | Comment | Textmining |
|---|---|---|---|
| Homo sapiens | P54868 | isoform HMGCS2 | - |
| Homo sapiens | Q01581 | isoform HMGCS1 | - |
Purification (Commentary)
| Purification (Comment) | Organism |
|---|---|
| Ni-Sepharose resin column chromatography and Superdex 200 gel filtration | Homo sapiens |
Substrates and Products (Substrate)
| Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|
| acetyl-CoA + acetoacetyl-CoA + H2O | - |
Homo sapiens | (S)-3-hydroxy-3-methylglutaryl-CoA + CoA | - |
? |  |
| additional information | the enzyme catalyzes a three-step ping-pong reaction involving an acylation, a condensation, and a hydrolysis | Homo sapiens | ? | - |
? | |
Synonyms
| Synonyms | Comment | Organism |
|---|---|---|
| 3-hydroxy-3-methylglutaryl coenzyme A synthase | - |
Homo sapiens |
| HMG-CoA synthase | - |
Homo sapiens |
| HMGCS | - |
Homo sapiens |
| HMGCS1 | isoform | Homo sapiens |
| HMGCS2 | isoform | Homo sapiens |
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Release 2023.1 (January 2023)
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