KETONE BODIES METABOLIC PATHWAY (PW:0000069)

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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 GBrowse Evidence Chr Start Stop Reference Source
G Aacs acetoacetyl-CoA synthetase GBrowse link ISS 12 32,206,863 32,250,583 RGD:2326093 RGD
G Acat1 acetyl-CoA acetyltransferase 1 GBrowse link IEA 8 57,044,478 57,072,970 RGD:6907045 KEGG
G Acat1 acetyl-CoA acetyltransferase 1 GBrowse link ISS 8 57,044,478 57,072,970 RGD:2326093 RGD
G Acat2 acetyl-CoA acetyltransferase 2 GBrowse link IEA 1 41,922,020 41,940,074 RGD:6907045 KEGG
G Acat2 acetyl-CoA acetyltransferase 2 GBrowse link ISS 1 41,922,020 41,940,074 RGD:2326093 RGD
G Bdh1 3-hydroxybutyrate dehydrogenase, type 1 GBrowse link IEA 11 71,130,561 71,165,695 RGD:6907045 KEGG
G Bdh1 3-hydroxybutyrate dehydrogenase, type 1 GBrowse link ISS 11 71,130,561 71,165,695 RGD:2326093 RGD
G Bdh2 3-hydroxybutyrate dehydrogenase, type 2 GBrowse link IEA 2 232,765,049 232,785,694 RGD:6907045 KEGG
G Hmgcl 3-hydroxymethyl-3-methylglutaryl-CoA lyase GBrowse link IEA 5 154,730,232 154,743,974 RGD:6907045 KEGG
G Hmgcl 3-hydroxymethyl-3-methylglutaryl-CoA lyase GBrowse link ISS 5 154,730,232 154,743,974 RGD:2326093 RGD
G Hmgcs1 3-hydroxy-3-methylglutaryl-CoA synthase 1 (soluble) GBrowse link IEA 2 51,737,090 51,754,583 RGD:6907045 KEGG
G Hmgcs2 3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial) GBrowse link IEA 2 193,128,736 193,143,106 RGD:6907045 KEGG
G Oxct1 3-oxoacid CoA transferase 1 GBrowse link IEA 2 53,189,358 53,217,848 RGD:6907045 KEGG
G Oxct1 3-oxoacid CoA transferase 1 GBrowse link ISS 2 53,189,358 53,217,848 RGD:2326093 RGD
G Oxct2a 3-oxoacid CoA transferase 2A GBrowse link IEA 5 142,636,815 142,638,554 RGD:6907045 KEGG
G Oxct2b 3-oxoacid CoA transferase 2B GBrowse link ISS 5 142,432,259 142,434,013 RGD:2326216 RGD
ketone bodies biosynthetic pathway term browser
Symbol Object Name GBrowse Evidence Chr Start Stop Reference Source
G Hmgcs1 3-hydroxy-3-methylglutaryl-CoA synthase 1 (soluble) GBrowse link TAS 2 51,737,090 51,754,583 RGD:2326134 RGD
G Hmgcs2 3-hydroxy-3-methylglutaryl-CoA synthase 2 (mitochondrial) GBrowse link TAS 2 193,128,736 193,143,106 RGD:2326134 RGD

Pathway Gene Annotations

Disease Annotations Associated with Genes in the ketone bodies metabolic pathway
Disease TermsGene Symbols
Intellectual DisabilityAcat2
Amino Acid Metabolism, Inborn ErrorsHmgcl
Beta ketothiolase deficiencyAcat1
ObesityHmgcs1 , Aacs , Oxct1
HepatomegalyHmgcs2
Diabetes Mellitus, Type 2Oxct1
Metabolic Syndrome XAcat2
Carbohydrate Metabolism, Inborn ErrorsAcat1 , Hmgcl , Oxct1
Nephrotic SyndromeAcat1
ArteriosclerosisAcat1 , Acat2
Kidney Failure, ChronicAcat1
Gallbladder DiseasesAcat2
HypercholesterolemiaAcat2
Succinyl-CoA:3-oxoacid CoA transferase deficiencyOxct1
3-Hydroxy-3-Methylglutaryl-CoA Lyase DeficiencyHmgcl
Cholestasis, ExtrahepaticHmgcs2
BurnsOxct1
Coronary Artery DiseaseAcat2
Diabetes Mellitus, ExperimentalHmgcs2 , Hmgcs1 , Bdh1 , Hmgcl , Aacs , Oxct1
HyperthyroidismBdh1
Spinal Cord CompressionHmgcs1
Smith-Lemli-Opitz SyndromeAcat1
3-Hydroxy-3-Methylglutaryl-CoA Synthase 2 DeficiencyHmgcs2
Pathway Annotations Associated with Genes in the ketone bodies metabolic pathway
Pathway TermsGene Symbols
pyruvate metabolic pathwayAcat1 , Acat2
tryptophan metabolic pathwayAcat1 , Acat2
fatty acid metabolic pathwayAcat1 , Acat2
glyoxylate and dicarboxylate metabolic pathwayAcat1 , Acat2
propanoate metabolic pathwayAcat1 , Acat2
butanoate metabolic pathwayAcat1 , Hmgcs2 , Hmgcs1 , Bdh1 , Hmgcl , Aacs , Bdh2 , Acat2 , Oxct1 , Oxct2a
ketone bodies metabolic pathwayAcat1 , Hmgcs2 , Hmgcs1 , Bdh1 , Hmgcl , Aacs , Bdh2 , Acat2 , Oxct1 , Oxct2a , Oxct2b
valine, leucine and isoleucine degradation pathwayAcat1 , Hmgcs2 , Hmgcs1 , Hmgcl , Acat2 , Oxct1 , Oxct2a
lysine degradation pathwayAcat1 , Acat2
terpenoid biosynthetic pathwayAcat1 , Hmgcs1 , Acat2 , Hmgcs2
cholesterol biosynthetic pathwayAcat2 , Hmgcs1
ketone bodies biosynthetic pathwayHmgcs1 , Hmgcs2
forkhead class A signaling pathwayBdh1 , Hmgcs1
eicosanoid signaling pathway via peroxisome proliferator-activated receptor gammaHmgcs2

References Associated with the ketone bodies metabolic pathway:

Ontology Path Diagram:

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