4.1.3.4: hydroxymethylglutaryl-CoA lyase
This is an abbreviated version!
For detailed information about hydroxymethylglutaryl-CoA lyase, go to the full flat file.
Word Map on EC 4.1.3.4
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4.1.3.4
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3-hydroxy-3-methylglutaric
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aciduria
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ketogenesis
-
hypoglycemia
-
acidosis
-
inborn
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3-methylglutaric
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3-methylglutaconic
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ketogenic
-
3-hydroxyisovaleric
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hyperammonemia
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hypoketotic
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mevalonii
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lethargy
-
3-methylcrotonyl-coa
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medicine
-
acetoacetyl-coa
-
hl-deficient
-
hmgcs2
- 4.1.3.4
-
3-hydroxy-3-methylglutaric
- aciduria
-
ketogenesis
- hypoglycemia
- acidosis
-
inborn
-
3-methylglutaric
-
3-methylglutaconic
-
ketogenic
-
3-hydroxyisovaleric
-
hyperammonemia
-
hypoketotic
- mevalonii
- lethargy
- 3-methylcrotonyl-coa
- medicine
- acetoacetyl-coa
-
hl-deficient
- hmgcs2
Reaction
Synonyms
3-hydroxy-3-methylglutarate-CoA lyase, 3-Hydroxy-3-methylglutaryl CoA cleaving enzyme, 3-hydroxy-3-methylglutaryl CoA lyase, 3-Hydroxy-3-methylglutaryl coenzyme A lyase, 3-Hydroxy-3-methylglutaryl-CoA lyase, 3-hydroxy-3-methylglutaryl-CoA lyase-like protein, 3-hydroxy-3-methylglutaryl-coenzyme A lyase, 3-hydroxy-methylglutaryl coenzyme A lyase, beta-hydroxy-beta-methylglutaryl-CoA lyase, HCL1, HL, HMG CoA cleavage enzyme, HMG CoA lyase, HMG-CoA lyase, HMGCL, HMGCLL1, HMGL, Hydroxymethylglutaryl coenzyme A lyase, Hydroxymethylglutaryl coenzyme A-cleaving enzyme, LiuE protein
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Reference on EC 4.1.3.4 - hydroxymethylglutaryl-CoA lyase
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REF. 
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Aragon, J.J.; Lowenstein, J.M.
A survey of enzymes which generate or use acetoacetyl thioesters in rat liver
J. Biol. Chem.
258
4725-4733
1983
Rattus norvegicus
Kramer, P.R.; Miziorko, H.M.
3-Hydroxy-3-methylglutaryl-CoA lyase: catalysis of acetyl coenzyme A enolization
Biochemistry
22
2353-2357
1983
Anas sp.
Kramer, P.R.; Miziorko, H.M.
Purification and characterization of avian liver 3-hydroxy-3-methylglutaryl coenzyme A lyase
J. Biol. Chem.
255
11023-11028
1980
Anas sp.
Zammit, V.A.
Localization of 3-hydroxy-3-methylglutaryl-CoA lyase and 3-oxo acid CoA-transferase in liver of three species of ruminants
Biochem. Soc. Trans.
8
543-544
1980
Capra hircus, Ovis aries
Clinkenbeard, K.D.; Reed, W.D.; Mooney, R.A.; Lane, M.D.
Intracellular localization of the 3-hydroxy-3-methylglutaryl coenzyme a cycle enzymes in liver
J. Biol. Chem.
250
3108-3116
1975
Gallus gallus, Rattus norvegicus
Messner, B.; Eggerer, H.; Cornforth, J.W.; Mallaby, R.
Substrate stereochemistry of the hydroxymethylglutaryl-CoA lyase and methylglutaconyl-CoA hydratase reactions
Eur. J. Biochem.
53
255-264
1975
Bos taurus
-
Narasimhan, C.; Roberts, J.R.; Miziorko, H.M.
Pseudomonas mevalonii 3-hydroxy-3-methylglutaryl-CoA lyase: testing the function of the active site cysteine by site-directed mutation
Biochemistry
34
9930-9935
1995
Pseudomonas mevalonii
Bachhawat, B.K.; Robinson, W.G.; Coon, M.J.
The enzymatic cleavage of beta-hydroxy-beta-methylglutaryl coenzyme A to acetoacetate and acetyl coenzyme A
J. Biol. Chem.
216
727-736
1955
Columba livia, Sus scrofa
Wysocki, S.J.; Haehnel, R.
3-Hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: a review
J. Inherit. Metab. Dis.
9
225-233
1986
Homo sapiens
Ashmarina, L.I.; Robert, M.F.; Elsliger, M.A.; Mitchell, G.A.
Characterization of the hydroxymethylglutaryl-CoA lyase precursor, a protein targeted to peroxisomes and mitochondria
Biochem. J.
315
71-75
1996
Homo sapiens
Hruz, P.W.; Miziorko, H.M.
Avian 3-hydroxy-3-methylglutaryl-CoA lyase: sensitivity of enzyme activity to thiol/disulfide exchange and identification of proximal reactive cysteines
Protein Sci.
1
1144-1153
1992
Gallus gallus, Tetrahymena pyriformis
Weber, T.; Bach, T.J.
Partial purification and characterization of membrane-associated 3-hydroxy-3-methylglutaryl coenzyme A lyase from radish seedlings
Z. Naturforsch. C
48
444-450
1993
Raphanus sativus
-
Mitchell, G.A.; Robert, M.F.; Hruz, P.W.; Wang, S.; Fontaine, G.; Behnke, C.E.; Mende-Mueller, L.M.; Schappert, K.; Lee, C.; Gibson, K.M.; Miziorko, H.M.
3-Hydroxy-3-methylglutaryl coenzyme A lyase (HL)
J. Biol. Chem.
268
4376-4381
1993
Gallus gallus, Homo sapiens
Roberts, J.R.; Narasimhan, C.; Hruz, P.W.; Mitchell, G.A.; Miziorko, H.M.
3-Hydroxy-3-methylglutaryl-CoA lyase: expression and isolation of the recombinant human enzyme and investigation of a mechanism for regulation of enzyme activity
J. Biol. Chem.
269
17841-17846
1994
Homo sapiens
Wanders, R.J.A.; Schutgens, R.B.H.; Zoeters, P.H.M.
3-Hydroxy-3-methylglutaryl-CoA lyase in human skin fibroblasts: study of its properties and deficient activity in 3-hydroxy-3-methylglutaric aciduria patients using a simple spectrophotometric method
Clin. Chim. Acta
171
95-102
1988
Homo sapiens
Narasimhan, C.; Miziorko, H.M.
Pseudomonas mevalonii 3-hydroxy-3-methylglutaryl-CoA lyase: characterization of the isolated recombinant protein and investigation of the enzyme's cation requirements
Biochemistry
31
11224-11230
1992
Pseudomonas mevalonii
Tuinstra, R.L.; Burgner, J.W.2nd.; Miziorko, H.M.
Investigation of the oligomeric status of the peroxisomal isoform of human 3-hydroxy-3-methylglutaryl-CoA lyase
Arch. Biochem. Biophys.
408
286-294
2002
Homo sapiens
Tuinstra, R.L.; Wang, C.Z.; Mitchell, G.A.; Miziorko, H.M.
Evaluation of 3-hydroxy-3-methylglutaryl-coenzyme A lyase arginine-41 as a catalytic residue: Use of acetyldithio-coenzyme A to monitor product enolization
Biochemistry
43
5287-5295
2004
Homo sapiens
Casals, N.; Gomez-Puertas, P.; Pie, J.; Mir, C.; Roca, R.; Puisac, B.; Aledo, R.; Clotet, J.; Menao, S.; Serra, D.; Asins, G.; Till, J.; Elias-Jones, A.C.; Cresto, J.C.; Chamoles, N.A.; Abdenur, J.E.; Mayatepek, E.; Besley, G.; Valencia, A.; Hegardt, F.G.
Structural (betaalpha)8 TIM barrel model of 3-hydroxy-3-methylglutaryl-coenzyme A lyase
J. Biol. Chem.
278
29016-29023
2003
Homo sapiens
Ashmarina, L.I.; Pshezhetsky, A.V.; Branda, S.S.; Isaya, G.; Mitchell, G.A.
3-Hydroxy-3-methylglutaryl coenzyme A lyase: targeting and processing in peroxisomes and mitochondria
J. Lipid Res.
40
70-75
1999
Homo sapiens
Miziorko, H.M.; Narasimhan, C.
Pseudomonas mevalonii 3-hydroxy-3-methylglutaryl-CoA lyase
Methods Enzymol.
324
139-149
2000
Pseudomonas mevalonii
Puisac, B.; Lopez-Vinas, E.; Moreno, S.; Mir, C.; Perez-Cerda, C.; Menao, S.; Lluch, D.; Pie, A.; Gomez-Puertas, P.; Casals, N.; Ugarte, M.; Hegardt, F.; Pie, J.
Skipping of exon 2 and exons 2 plus 3 of HMG-CoA lyase (HL) gene produces the loss of beta sheets 1 and 2 in the recently proposed (beta-alpha)8 TIM barrel model of HL
Biophys. Chem.
115
241-245
2005
Homo sapiens
Fu, Z.; Runquist, J.A.; Forouhar, F.; Hussain, M.; Hunt, J.F.; Miziorko, H.M.; Kim, J.J.
Crystal structure of human HMG-CoA lyase: Insights into catalysis and the molecular basis for hydroxymethylglutaric aciduria
J. Biol. Chem.
281
7526-7532
2005
Homo sapiens
Forouhar, F.; Hussain, M.; Farid, R.; Benach, J.; Abashidze, M.; Edstrom, W.C.; Vorobiev, S.M.; Xiao, R.; Acton, T.B.; Fu, Z.; Kim, J.J.; Miziorko, H.M.; Montelione, G.T.; Hunt, J.F.
Crystal structures of two bacterial Hmg-CoA lyases suggest a common catalytic mechanism among a family of TIM-barrel metalloenzymes cleaving carbon-carbon bonds
J. Biol. Chem.
281
7533-7545
2005
Bacillus subtilis (O34873), Bacillus subtilis, Brucella melitensis (Q8YEF2), Brucella melitensis
Cardoso, M.L.; Rodrigues, M.R.; Leao, E.; Martins, E.; Diogo, L.; Rodrigues, E.; Garcia, P.; Rolland, M.O.; Vilarinho, L.
The E37X is a common HMGCL mutation in Portuguese patients with 3-hydroxy-3-methylglutaric CoA lyase deficiency
Mol. Genet. Metab.
82
334-338
2004
Homo sapiens
Al-Sayed, M.; Imtiaz, F.; Alsmadi, O.A.; Rashed, M.S.; Meyer, B.F.
Mutations underlying 3-hydroxy-3-methylglutaryl CoA lyase deficiency in the Saudi population
BMC Med. Genet.
7
86
2006
Homo sapiens
Mir, C.; Lopez-Vinas, E.; Aledo, R.; Puisac, B.; Rizzo, C.; Dionisi-Vici, C.; Deodato, F.; Pie, J.; Gomez-Puertas, P.; Hegardt, F.G.; Casals, N.
A single-residue mutation, G203E, causes 3-hydroxy-3-methylglutaric aciduria by occluding the substrate channel in the 3D structural model of HMG-CoA lyase
J. Inherit. Metab. Dis.
29
64-70
2006
Homo sapiens (P35914)
Carrasco, P.; Menao, S.; Lopez-Vinas, E.; Santpere, G.; Clotet, J.; Sierra, A.Y.; Gratacos, E.; Puisac, B.; Gomez-Puertas, P.; Hegardt, F.G.; Pie, J.; Casals, N.
C-terminal end and amino acid Lys48 in HMG-CoA lyase are involved in substrate binding and enzyme activity
Mol. Genet. Metab.
91
120-127
2007
Homo sapiens (P35914), Homo sapiens
Pie, J.; Lopez-Vinas, E.; Puisac, B.; Menao, S.; Pie, A.; Casale, C.; Ramos, F.J.; Hegardt, F.G.; Gomez-Puertas, P.; Casals, N.
Molecular genetics of HMG-CoA lyase deficiency
Mol. Genet. Metab.
92
198-209
2007
Homo sapiens (P35914), Homo sapiens
Lin, W.D.; Wang, C.H.; Lai, C.C.; Tsai, Y.; Wu, J.Y.; Chen, C.P.; Tsai, F.J.
Molecular analysis of Taiwanese patients with 3-hydroxy-3-methylglutaryl CoA lyase deficiency
Clin. Chim. Acta
401
33-36
2009
Homo sapiens
Leipnitz, G.; Seminotti, B.; Haubrich, J.; Dalcin, M.B.; Dalcin, K.B.; Solano, A.; de Bortoli, G.; Rosa, R.B.; Amaral, A.U.; Dutra-Filho, C.S.; Latini, A.; Wajner, M.
Evidence that 3-hydroxy-3-methylglutaric acid promotes lipid and protein oxidative damage and reduces the nonenzymatic antioxidant defenses in rat cerebral cortex
J. Neurosci. Res.
86
683-693
2008
Rattus norvegicus (P97519)
Leung, A.A.; Chan, A.K.; Ezekowitz, J.A.; Leung, A.K.
A case of dilated cardiomyopathy associated with 3-hydroxy-3-methylglutaryl-coenzyme A (HMG CoA) lyase deficiency
Case Report Med.
2009
183125
2009
Homo sapiens
Leipnitz, G.; Seminotti, B.; Fernandes, C.G.; Amaral, A.U.; Beskow, A.P.; da Silva, L.d.e..B.; Zanatta, A.; Ribeiro, C.A.; Vargas, C.R.; Wajner, M.
Striatum is more vulnerable to oxidative damage induced by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency as compared to liver
Int. J. Dev. Neurosci.
27
351-356
2009
Rattus norvegicus
Reimao, S.; Morgado, C.; Almeida, I.T.; Silva, M.; Real, H.C.; Campos, J.
3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: Initial presentation in a young adult
J. Inherit. Metab. Dis.
32
S49-52
2009
Homo sapiens
Chavez-Aviles, M.; Diaz-Perez, A.L.; Campos-Garcia, J.
The bifunctional role of LiuE from Pseudomonas aeruginosa, displays additionally HIHG-CoA lyase enzymatic activity
Mol. Biol. Rep.
37
1787-1791
2010
Pseudomonas aeruginosa, Pseudomonas aeruginosa PAO1SM
Montgomery, C.; Miziorko, H.M.
Influence of multiple cysteines on human 3-hydroxy-3-methylglutaryl-CoA lyase activity and formation of inter-subunit adducts
Arch. Biochem. Biophys.
511
48-55
2011
Homo sapiens (P35914), Homo sapiens
Fu, Z.; Runquist, J.A.; Montgomery, C.; Miziorko, H.M.; Kim, J.J.
Functional insights into human HMG-CoA lyase from structures of Acyl-CoA-containing ternary complexes
J. Biol. Chem.
285
26341-26349
2010
Homo sapiens
Puisac, B.; Arnedo, M.; Casale, C.H.; Ribate, M.P.; Castiella, T.; Ramos, F.J.; Ribes, A.; Perez-Cerda, C.; Casals, N.; Hegardt, F.G.; Pie, J.
Differential HMG-CoA lyase expression in human tissues provides clues about 3-hydroxy-3-methylglutaric aciduria
J. Inherit. Metab. Dis.
33
405-410
2010
Homo sapiens
Nakagawa, S.; Kojima, Y.; Sekino, K.; Yamato, S.
Effect of polyphenols on 3-hydroxy-3-methylglutaryl-coenzyme A lyase activity in human hepatoma HepG2 cell extracts
Biol. Pharm. Bull.
36
1902-1906
2013
Homo sapiens
Isaac, D.T.; Coady, A.; Van Prooyen, N.; Sil, A.
The 3-hydroxy-methylglutaryl coenzyme A lyase HCL1 is required for macrophage colonization by human fungal pathogen Histoplasma capsulatum
Infect. Immun.
81
411-420
2013
Histoplasma capsulatum
Montgomery, C.; Pei, Z.; Watkins, P.A.; Miziorko, H.M.
Identification and characterization of an extramitochondrial human 3-hydroxy-3-methylglutaryl-CoA lyase
J. Biol. Chem.
287
33227-33236
2012
Homo sapiens
Dong, Y.; Zhang, J.; Xu, R.; Lv, X.; Wang, L.; Sun, A.; Wei, D.
Insertion mutation in HMG-CoA lyase increases the production yield of MPA through Agrobacterium tumefaciens-mediated transformation
J. Microbiol. Biotechnol.
26
1924-1932
2016
Penicillium brevicompactum, Penicillium brevicompactum ATCC 16024
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