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Information on EC 4.1.3.4 - hydroxymethylglutaryl-CoA lyase and Organism(s) Rattus norvegicus and UniProt Accession P97519

for references in articles please use BRENDA:EC4.1.3.4
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EC Tree
     4 Lyases
         4.1 Carbon-carbon lyases
             4.1.3 Oxo-acid-lyases
                4.1.3.4 hydroxymethylglutaryl-CoA lyase
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Select one or more organisms in this record: ?
This record set is specific for:
Rattus norvegicus
UNIPROT: P97519 not found.
Word Map
The taxonomic range for the selected organisms is: Rattus norvegicus
The enzyme appears in selected viruses and cellular organisms
Synonyms
hmg-coa lyase, 3-hydroxy-3-methylglutaryl-coa lyase, hmgcl, 3-hydroxy-3-methylglutaryl-coenzyme a lyase, hmgcll1, 3-hydroxy-3-methylglutaryl coenzyme a lyase, 3-hydroxy-3-methylglutaryl coa lyase, hydroxymethylglutaryl-coa lyase, hmg coa lyase, liue protein, more
SYNONYM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
3-hydroxy-3-methylglutarate-CoA lyase
-
-
-
0
3-Hydroxy-3-methylglutaryl CoA cleaving enzyme
-
-
-
0
3-Hydroxy-3-methylglutaryl coenzyme A lyase
-
-
-
0
3-Hydroxy-3-methylglutaryl-CoA lyase
HMG CoA cleavage enzyme
-
-
-
0
HMG-CoA lyase
-
-
-
0
HMGCL
286826
-
Hydroxymethylglutaryl coenzyme A lyase
-
-
-
0
Hydroxymethylglutaryl coenzyme A-cleaving enzyme
-
-
-
0
SYSTEMATIC NAME
IUBMB Comments
(S)-3-hydroxy-3-methylglutaryl-CoA acetoacetate-lyase (acetyl-CoA-forming)
-
CAS REGISTRY NUMBER
COMMENTARY hide
9030-83-5
-
SUBSTRATE
PRODUCT                       
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
(S)-3-Hydroxy-3-methylglutaryl-CoA
Acetyl-CoA + acetoacetate
show the reaction diagram
-
-
-
?
(S)-3-Hydroxy-3-methylglutaryl-CoA
?
show the reaction diagram
(S)-3-Hydroxy-3-methylglutaryl-CoA
Acetyl-CoA + acetoacetate
show the reaction diagram
-
-
-
-
?
NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate) hide
LITERATURE
(Substrate)
COMMENTARY
(Product) hide
LITERATURE
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
(S)-3-Hydroxy-3-methylglutaryl-CoA
Acetyl-CoA + acetoacetate
show the reaction diagram
-
-
-
?
(S)-3-Hydroxy-3-methylglutaryl-CoA
?
show the reaction diagram
(S)-3-Hydroxy-3-methylglutaryl-CoA
Acetyl-CoA + acetoacetate
show the reaction diagram
-
-
-
-
?
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
7.4
-
enzyme assay at
ORGANISM
COMMENTARY hide
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
Wistar rat
UniProt
Manually annotated by BRENDA team
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
SOURCE
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY hide
GeneOntology No.
LITERATURE
SOURCE
-
13% of activity
Manually annotated by BRENDA team
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION?
HMGCL_RAT
325
0
34192
Swiss-Prot
Mitochondrion (Reliability: 1)
APPLICATION
ORGANISM
UNIPROT
COMMENTARY hide
LITERATURE
medicine
3-hydroxy-3-methylglutaryl CoA lyase deficiency is a rare autosomal recessive mitochondrial disease characterized by a deficiency in the enzyme 3-hydroxy-3-methylglutaryl CoA lyase
medicine
-
3-hydroxy-3-methylglutaric aciduria is an autosomal recessive branched chain organic aciduria caused by the deficiency of the enzyme 3-hydroxy-3-methylglutaryl-CoA lyase
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
Manually annotated by BRENDA team
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
Manually annotated by BRENDA team
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)
Manually annotated by BRENDA team
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
Manually annotated by BRENDA team
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