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KETONE BODIES METABOLIC PATHWAY (PW:0000069)

View Ontology Report

Description

The ketone bodies metabolic pathway is used to convert acetyl-CoA formed in the liver into "ketone bodies": acetone, and more importantly acetoacetate and 3-hydroxybutyrate, which are transported in the blood to extrahepatic tissues where they are converted to acetyl-CoA and oxidized via the citrate cycle pathway for energy. The brain, which usually uses glucose for energy, can utilize ketone bodies under starvation conditions, when glucose is not available. When acetyl-CoA is not being metaboli

Pathway Diagram:

Ariadne Genomics Inc. Adc acetyl- CoA Bdh1 Acat1 Hmgcs2 Oxct1 Oxct2a acetone aceto- acetate 3-hydroxy-3-methyl- glutaryl-CoA ---> acetyl- CoA Aacs 3-hydroxy- butyrate aceto- acetate ---> acetone aceto- acetate ---> 3-hydroxy- butyrate acetyl- CoA ---> 3-hydroxy-3-methyl- glutaryl-CoA aceto- acetate ---> aceto- acetyl-CoA aceto- acetyl-CoA aceto- acetyl-CoA ---> aceto- acetate fatty acid beta degradation pathway ---> acetyl- CoA 3-hydroxy-3-methyl- glutaryl-CoA cholesterol biosynthetic pathway 3-hydroxy-3-methyl- glutaryl-CoA ---> cholesterol biosynthetic pathway 3-hydroxy- butyrate ---> aceto- acetate Bdh2 acetyl- CoA ---> aceto- acetyl-CoA aceto- acetyl-CoA ---> acetyl- CoA aceto- acetyl-CoA ---> 3-hydroxy-3-methyl- glutaryl-CoA Hmgcs1 pyruvate metabolic pathway ---> acetyl- CoA fatty acid biosynthetic pathway fatty acid beta degradation pathway pyruvate metabolic pathway Acat2 3-hydroxy-3-methyl- glutaryl-CoA ---> aceto- acetate acetyl- CoA ---> fatty acid biosynthetic pathway Hmgcl citrate cycle pathway acetyl- CoA ---> citrate cycle pathway
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Genes in Pathway:

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ketone bodies metabolic pathway term browser
Symbol Object Name Evidence Notes Source PubMed Reference(s) RGD Reference(s) Position
G Aacs acetoacetyl-CoA synthetase ISO RGD PMID:15877199 RGD:2326093 NCBI chr12:36,512,393...36,555,694
Ensembl chr12:36,512,393...36,555,694
JBrowse link
G Acat1 acetyl-CoA acetyltransferase 1 IEA
ISO
KEGG
SMPDB
PMID:15877199 SMP:00071, rno:00072, RGD:2326093 NCBI chr 8:58,166,990...58,195,884
Ensembl chr 8:58,166,990...58,195,884
JBrowse link
G Acat2 acetyl-CoA acetyltransferase 2 ISO
IEA
KEGG PMID:15877199 rno:00072, RGD:2326093 NCBI chr 1:47,972,399...47,992,654
Ensembl chr 1:47,972,399...47,992,653
JBrowse link
G Bdh1 3-hydroxybutyrate dehydrogenase 1 ISO
IEA
SMPDB
KEGG
PMID:15877199 SMP:00071, rno:00072, RGD:2326093 NCBI chr11:72,577,547...72,619,435
Ensembl chr11:72,578,533...72,614,176
JBrowse link
G Bdh2 3-hydroxybutyrate dehydrogenase 2 IEA KEGG rno:00072 NCBI chr 2:240,461,505...240,482,149
Ensembl chr 2:240,461,505...240,482,145
JBrowse link
G Hmgcl 3-hydroxy-3-methylglutaryl-CoA lyase IEA
ISO
KEGG
SMPDB
PMID:15877199 SMP:00071, rno:00072, RGD:2326093 NCBI chr 5:154,294,841...154,308,582
Ensembl chr 5:154,294,806...154,308,640
JBrowse link
G Hmgcs1 3-hydroxy-3-methylglutaryl-CoA synthase 1 IEA KEGG rno:00072 NCBI chr 2:52,427,351...52,445,082
Ensembl chr 2:52,431,601...52,445,055
JBrowse link
G Hmgcs2 3-hydroxy-3-methylglutaryl-CoA synthase 2 IEA
ISO
KEGG
SMPDB
SMP:00071, rno:00072 NCBI chr 2:200,452,623...200,480,785
Ensembl chr 2:200,452,624...200,479,423
JBrowse link
G Oxct1 3-oxoacid CoA transferase 1 ISO
IEA
SMPDB
KEGG
PMID:15877199 SMP:00071, rno:00072, RGD:2326093 NCBI chr 2:53,859,738...54,007,733
Ensembl chr 2:53,859,737...54,007,756
JBrowse link
G Oxct2a 3-oxoacid CoA transferase 2A IEA KEGG rno:00072 NCBI chr 5:141,016,542...141,018,832 JBrowse link
G Oxct2b 3-oxoacid CoA transferase 2B ISO RGD PMID:11090426 RGD:2326216 NCBI chr 5:140,813,957...140,815,711 JBrowse link
ketone bodies biosynthetic pathway term browser
Symbol Object Name Evidence Notes Source PubMed Reference(s) RGD Reference(s) Position
G Hmgcs1 3-hydroxy-3-methylglutaryl-CoA synthase 1 TAS RGD PMID:9893938 RGD:2326134 NCBI chr 2:52,427,351...52,445,082
Ensembl chr 2:52,431,601...52,445,055
JBrowse link
G Hmgcs2 3-hydroxy-3-methylglutaryl-CoA synthase 2 TAS RGD PMID:9893938 RGD:2326134 NCBI chr 2:200,452,623...200,480,785
Ensembl chr 2:200,452,624...200,479,423
JBrowse link

Pathway Gene Annotations

Disease Annotations Associated with Genes in the ketone bodies metabolic pathway
Pathway Annotations Associated with Genes in the ketone bodies metabolic pathway
Pathway TermsGene Symbols
2-aminoadipic 2-oxoadipic aciduria pathwayAcat1
3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
3-hydroxyacyl-CoA dehydrogenase deficiency pathwayAcat1
3-hydroxyisobutyric aciduria pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
3-methylcrotonyl CoA carboxylase 1 deficiency pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
3-methylglutaconic aciduria type 1 pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
3-methylglutaconic aciduria type 3 pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
alendronate pharmacodynamics pathwayAcat2 , Hmgcs1
bile acid signaling pathwayAcat2
butanoate metabolic pathwayAacs , Acat1 , Acat2 , Bdh1 , Bdh2 , Hmgcl , Hmgcs1 , Hmgcs2 , Oxct1 , Oxct2a
carnitine palmitoyltransferase I deficiency pathwayAcat1
cholesterol biosynthetic pathwayAcat2 , Hmgcs1
cholesterol ester storage disease pathwayAcat2 , Hmgcs1
congenital hemidysplasia with ichthyosiform erythroderma and limb defects pathwayAcat2 , Hmgcs1
desmosterolosis pathwayAcat2 , Hmgcs1
eicosanoid signaling pathway via peroxisome proliferator-activated receptor gammaHmgcs2
ethylmalonic encephalopathy pathwayAcat1
fatty acid beta degradation pathwayAcat1
fatty acid metabolic pathwayAcat1 , Acat2
forkhead class A signaling pathwayBdh1 , Hmgcs1
glutaric aciduria type I pathwayAcat1
glyoxylate and dicarboxylate metabolic pathwayAcat1 , Acat2
hypercholesterolemia pathwayAcat2 , Hmgcs1
hyperlysinemia pathwayAcat1
ibandronate pharmacodynamics pathwayAcat2 , Hmgcs1
isobutyryl-CoA dehydrogenase deficiency pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
isovaleric acidemia pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
ketone bodies biosynthetic pathwayHmgcs1 , Hmgcs2
ketone bodies metabolic pathwayAacs , Acat1 , Acat2 , Bdh1 , Bdh2 , Hmgcl , Hmgcs1 , Hmgcs2 , Oxct1 , Oxct2a , Oxct2b
Leigh disease pathwayAcat1
lysine degradation pathwayAcat1 , Acat2
malonic aciduria pathwayAcat1
maple syrup urine disease pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
medium chain acyl-CoA dehydrogenase deficiency pathwayAcat1
methylmalonate semialdehyde dehydrogenase deficiency pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
methylmalonic acidemia pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
methylmalonic aciduria, cobalamin-related pathwayAcat1
mevalonic aciduria pathwayAcat2 , Hmgcs1
nitrogenous bisphosphonate pharmacodynamics pathwayAcat2 , Hmgcs1
pamidronate pharmacodynamics pathwayAcat2 , Hmgcs1
primary hyperoxaluria type 2 pathwayAcat1
propanoate metabolic pathwayAcat1 , Acat2
propionic acidemia pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
pyruvate decarboxylase deficiency pathwayAcat1
pyruvate dehydrogenase E1 deficiency pathwayAcat1
pyruvate kinase deficiency of red cells pathwayAcat1
pyruvate metabolic pathwayAcat1 , Acat2
risedronate pharmacodynamics pathwayAcat2 , Hmgcs1
saccharopinuria pathwayAcat1
short-chain acyl-CoA dehydrogenase deficiency pathwayAcat1
Smith-Lemli-Opitz Syndrome pathwayAcat2 , Hmgcs1
statin pharmacodynamics pathwayAcat2 , Hmgcs1
succinyl-CoA:3-oxoacid transferase deficiency pathwayAcat1 , Bdh1 , Hmgcl , Hmgcs2 , Oxct1
terpenoid biosynthetic pathwayAcat1 , Acat2 , Hmgcs1 , Hmgcs2
trifunctional protein deficiency pathwayAcat1
tryptophan metabolic pathwayAcat1 , Acat2
valine, leucine and isoleucine degradation pathwayAcat1 , Acat2 , Hmgcl , Hmgcs1 , Hmgcs2 , Oxct1 , Oxct2a
very long-chain acyl-CoA dehydrogenase deficiency pathwayAcat1
Wolman disease pathwayAcat2 , Hmgcs1
X-linked dominant chondrodysplasia punctata 2 pathwayAcat2 , Hmgcs1
zoledronate pharmacodynamics pathwayAcat2 , Hmgcs1

References Associated with the ketone bodies metabolic pathway:

Ontology Path Diagram:

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paths to the root

Import into Pathway Studio:


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RGD is funded by grant HL64541 from the National Heart, Lung, and Blood Institute on behalf of the NIH.