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Information on EC 3.1.2.4 - 3-hydroxyisobutyryl-CoA hydrolase and Organism(s) Homo sapiens and UniProt Accession Q6NVY1
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3.1.2.4 3-hydroxyisobutyryl-CoA hydrolaseIUBMB Comments
Also hydrolyses 3-hydroxypropanoyl-CoA.
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The taxonomic range for the selected organisms is: Homo sapiens
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria
The expected taxonomic range for this enzyme is: Eukaryota, Bacteria
Synonyms
hibch, 3-hydroxyisobutyryl-coa hydrolase, smb20752, ngr_b20860, hib-coa hydrolase, hibyl-coa hydrolase, beta-hydroxyisobutyryl-coa hydrolase, beta-hydroxyisobutyryl-coenzyme a hydrolase, 
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topSYNONYM 
ORGANISM
UNIPROT
COMMENTARY 
LITERATURE
3-hydroxy-isobutyryl CoA hydrolase
-
-
-
-
hydrolase, 3-hydroxyisobutyryl coenzyme A
-
-
-
-
REACTION TYPE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
hydrolysis of thioester
hydrolysis of thioester
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-
-
-
PATHWAY SOURCE
PATHWAYS
BRENDA
-
-, -, -, -
SYSTEMATIC NAME
IUBMB Comments
3-hydroxy-2-methylpropanoyl-CoA hydrolase
Also hydrolyses 3-hydroxypropanoyl-CoA.
CAS REGISTRY NUMBER
COMMENTARY
9025-88-1
-
SUBSTRATE
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
3-hydroxy-2-methylpropanoyl-CoA + H2O
?
the enzyme is responsible for the specific hydrolysis of 3-hydroxy-2-methylpropanoyl-CoA, a saline catabolite
-
-
?
3-hydroxy-2-methylpropanoyl-CoA + H2O
CoA + 3-hydroxy-2-methylpropanoate
-
-
-
?
(S)-3-hydroxy-2-methylpropanoyl-CoA + H2O
CoA + (S)-3-hydroxy-2-methylpropanoate
-
-
-
?
3-hydroxy-2-methylbutyryl-CoA + H2O
CoA + 3-hydroxy-2-methylbutyrate
-
low activity
-
?
DL-3-hydroxybutyryl-CoA + H2O
CoA + DL-3-hydroxybutyrate
-
low activity
-
?
L-3-hydroxybutyryl-CoA + H2O
CoA + L-3-hydroxybutyrate
-
low activity
-
?
3-hydroxypropanoyl-CoA + H2O
CoA + 3-hydroxypropanoate
hydrolysis of beta-hydroxypropionyl-CoA, an intermediate in a minor pathway of propionate metabolism
-
-
?
3-hydroxy-2-methylpropanoyl-CoA + H2O
CoA + 3-hydroxy-2-methylpropanoate
-
-
-
?
3-hydroxy-2-methylpropanoyl-CoA + H2O
CoA + 3-hydroxy-2-methylpropanoate
-
-
-
ir
3-hydroxy-2-methylpropanoyl-CoA + H2O
CoA + 3-hydroxy-2-methylpropanoate
-
key enzyme in valine metabolism regulating the toxic concentration of methacrylyl-coenzyme A, human liver disease decreases the enzyme activity in vivo
-
ir
additional information
?
-
mutations in the gene encoding 3-hydroxyisobutyryl-CoA hydrolase results in progressive infantile neurodegeneration
-
-
?
additional information
?
-
-
mutations in the gene encoding 3-hydroxyisobutyryl-CoA hydrolase results in progressive infantile neurodegeneration
-
-
?
additional information
?
-
-
substrate specificity, overview, acetoacetyl-CoA, DL-methylmalonyl-CoA, isobutyryl-CoA, malonyl-CoA, acetyl-CoA, propionyl-CoA, and n-valeryl-CoA are poor substrates
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?
NATURAL SUBSTRATE
NATURAL PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
REVERSIBILITY
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
3-hydroxy-2-methylpropanoyl-CoA + H2O
?
the enzyme is responsible for the specific hydrolysis of 3-hydroxy-2-methylpropanoyl-CoA, a saline catabolite
-
-
?
3-hydroxypropanoyl-CoA + H2O
CoA + 3-hydroxypropanoate
hydrolysis of beta-hydroxypropionyl-CoA, an intermediate in a minor pathway of propionate metabolism
-
-
?
3-hydroxy-2-methylpropanoyl-CoA + H2O
CoA + 3-hydroxy-2-methylpropanoate
-
-
-
?
3-hydroxy-2-methylpropanoyl-CoA + H2O
CoA + 3-hydroxy-2-methylpropanoate
-
key enzyme in valine metabolism regulating the toxic concentration of methacrylyl-coenzyme A, human liver disease decreases the enzyme activity in vivo
-
ir
additional information
?
-
mutations in the gene encoding 3-hydroxyisobutyryl-CoA hydrolase results in progressive infantile neurodegeneration
-
-
?
additional information
?
-
-
mutations in the gene encoding 3-hydroxyisobutyryl-CoA hydrolase results in progressive infantile neurodegeneration
-
-
?
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
KM VALUE [mM]
SUBSTRATE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
Ki VALUE [mM]
INHIBITOR
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
IMAGE
SPECIFIC ACTIVITY [µmol/min/mg]
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
430
-
purified recombinant enzyme, substrate (S)-3-hydroxy-2-methylpropanoyl-CoA
pH OPTIMUM
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
pH RANGE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
ORGANISM
COMMENTARY
LITERATURE
UNIPROT
SEQUENCE DB
SOURCE
SOURCE TISSUE
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
SOURCE
-
36% reduced activity in livers with chronic hepatitis and cirrhosis compared to healthy livers
LOCALIZATION
ORGANISM
UNIPROT
COMMENTARY
GeneOntology No.
LITERATURE
SOURCE
UNIPROT
ENTRY NAME
ORGANISM
NO. OF AA
NO. OF TRANSM. HELICES
MOLECULAR WEIGHT[Da]
SOURCE
SEQUENCE
LOCALIZATION PREDICTION
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable).
The reliability class of the localization prediction ranges from 1 (very reliable) to 5 (not reliable). HIBCH_HUMAN
386
0
43482
Swiss-Prot
Mitochondrion (Reliability: 1)
PDB
SCOP
CATH
UNIPROT
ORGANISM
MOLECULAR WEIGHT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
38000
-
1 * 39398, amino acid sequence calculation, 1 * 38000, recombinant enzyme, SDS-PAGE
39398
-
1 * 39398, amino acid sequence calculation, 1 * 38000, recombinant enzyme, SDS-PAGE
SUBUNIT
ORGANISM
UNIPROT
COMMENTARY
LITERATURE
monomer
-
1 * 39398, amino acid sequence calculation, 1 * 38000, recombinant enzyme, SDS-PAGE
PURIFICATION (Commentary)
ORGANISM
UNIPROT
LITERATURE
native enzyme from mitochondrial membranes of HL-60 cells by two-step heparin affinity chromatography method using divalent cations, e.g. Mg2+, as eluents
-
CLONED (Commentary)
ORGANISM
UNIPROT
LITERATURE
DNA and amino acid sequence determination and analysis, expression in Escherichia coli BL21(DE3) as His-tagged enzyme
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REF.
AUTHORS
TITLE
JOURNAL
VOL.
PAGES
YEAR
ORGANISM (UNIPROT)
PUBMED ID
SOURCE
Hawes, J.W.; Jaskiewicz, J.; Shimomura, Y.; Huang, B.; Bunting, J.; Harper, E.T.; Harris, R.A.
Primary structure and tissue-specific expression of human beta-hydroxyisobutyryl-coenzyme A hydrolase
J. Biol. Chem.
271
26430-26434
1996
Homo sapiens (Q6NVY1), Homo sapiens, Rattus norvegicus
Ishigure, K.; Shimomura, Y.; Murakami, T.; Kaneko, T.; Takeda, S.; Inoue, S.; Nomoto, S.; Koshikawa, K.; Nonami, T.; Nakao, A.
Human liver disease decreases methacrylyl-CoA hydratase and beta-hydroxyisobutyryl-CoA hydrolase activities in valine catabolism
Clin. Chim. Acta
312
115-121
2001
Homo sapiens
Shimomura, Y.; Murakami, T.; Nakai, N.; Huang, B.; Hawes, J.W.; Harris, R.A.
3-Hydroxyisobutyryl-CoA hydrolase
Methods Enzymol.
324
229-240
2000
Homo sapiens, Rattus norvegicus
Iida, T.; Kamo, M.; Uozumi, N.; Inui, T.; Imai, K.
Further application of a two-step heparin affinity chromatography method using divalent cations as eluents: purification and identification of membrane-bound heparin binding proteins from the mitochondrial fraction of HL-60 cells
J. Chromatogr. B
823
209-212
2005
Homo sapiens
Loupatty, F.J.; Clayton, P.T.; Ruiter, J.P.; Ofman, R.; Ijlst, L.; Brown, G.K.; Thorburn, D.R.; Harris, R.A.; Duran, M.; Desousa, C.; Krywawych, S.; Heales, S.J.; Wanders, R.J.
Mutations in the gene encoding 3-hydroxyisobutyryl-CoA hydrolase results in progressive infantile neurodegeneration
Am. J. Hum. Genet.
80
195-199
2007
Homo sapiens (Q6NVY1), Homo sapiens
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