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 Chr Start Stop Reference
G Aacs acetoacetyl-CoA synthetase GBrowse link 12 38,381,585 38,425,026 RGD:2326093
G Acat1 acetyl-CoA acetyltransferase 1 GBrowse link 8 56,749,861 56,778,336 RGD:2326093
RGD:6907045
RGD:10402751
G Acat2 acetyl-CoA acetyltransferase 2 GBrowse link 1 51,757,961 51,776,935 RGD:2326093
RGD:6907045
G Bdh1 3-hydroxybutyrate dehydrogenase, type 1 GBrowse link 11 75,651,886 75,693,764 RGD:2326093
RGD:6907045
RGD:10402751
G Bdh2 3-hydroxybutyrate dehydrogenase, type 2 GBrowse link 2 258,981,008 259,001,652 RGD:6907045
G Hmgcl 3-hydroxymethyl-3-methylglutaryl-CoA lyase GBrowse link 5 158,059,692 158,073,433 RGD:2326093
RGD:6907045
RGD:10402751
G Hmgcs1 3-hydroxy-3-methylglutaryl-CoA synthase 1 GBrowse link 2 70,791,035 70,809,191 RGD:6907045
G Hmgcs2 3-hydroxy-3-methylglutaryl-CoA synthase 2 GBrowse link 2 219,928,546 219,956,233 RGD:6907045
RGD:10402751
G Oxct1 3-oxoacid CoA transferase 1 GBrowse link 2 72,891,713 73,037,617 RGD:2326093
RGD:6907045
RGD:10402751
G Oxct2a 3-oxoacid CoA transferase 2A RGD:6907045
G Oxct2b 3-oxoacid CoA transferase 2B GBrowse link 5 144,806,548 144,808,302 RGD:2326216
ketone bodies biosynthetic pathway term browser
Symbol Object Name GBrowse Chr Start Stop Reference
G Hmgcs1 3-hydroxy-3-methylglutaryl-CoA synthase 1 GBrowse link 2 70,791,035 70,809,191 RGD:2326134
G Hmgcs2 3-hydroxy-3-methylglutaryl-CoA synthase 2 GBrowse link 2 219,928,546 219,956,233 RGD:2326134

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 disease pathwayAcat1
3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
3-hydroxyacyl-CoA dehydrogenase deficiency pathwayAcat1
alendronate pharmacodynamics pathwayAcat2 , Hmgcs1
butanoate metabolic pathwayAacs , Acat1 , Acat2 , Bdh1 , Bdh2 , Hmgcl , Hmgcs1 , Hmgcs2 , Oxct1 , Oxct2a
cholesterol biosynthetic pathwayAcat2 , Hmgcs1
cholesterol ester storage disease pathwayAcat2 , Hmgcs1
congenital hemidysplasia with ichthyosiform erythroderma and limb defects disease pathwayAcat2 , Hmgcs1
desmosterolosis disease pathwayAcat2 , Hmgcs1
eicosanoid signaling pathway via peroxisome proliferator-activated receptor gammaHmgcs2
ethylmalonic encephalopathy disease pathwayAcat1
fatty acid beta degradation pathwayAcat1
fatty acid metabolic pathwayAcat1 , Acat2
forkhead class A signaling pathwayBdh1 , Hmgcs1
glutaric aciduria type I disease pathwayAcat1
glyoxylate and dicarboxylate metabolic pathwayAcat1 , Acat2
hypercholesterolemia disease pathwayAcat2 , Hmgcs1
hyperlysinemias disease pathwayAcat1
ibandronate pharmacodynamics pathwayAcat2 , Hmgcs1
isobutyryl-CoA dehydrogenase deficiency pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
isovaleric acidemia disease pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
ketone bodies biosynthetic pathwayHmgcs1 , Hmgcs2
ketone bodies metabolic pathwayAacs , Acat1 , Acat2 , Bdh1 , Bdh2 , Hmgcl , Hmgcs1 , Hmgcs2 , Oxct1 , Oxct2a , Oxct2b
Leigh syndrome pathwayAcat1
lysine degradation pathwayAcat1 , Acat2
malonic aciduria disease 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 disease pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
methylmalonic aciduria, cobalamin-related, disease pathwayAcat1
mevalonate kinase deficiency pathwayAcat2 , Hmgcs1
nitrogenous bisphosphonate pharmacodynamics pathwayAcat2 , Hmgcs1
pamidronate pharmacodynamics pathwayAcat2 , Hmgcs1
primary hyperoxaluria type 2 disease pathwayAcat1
propanoate metabolic pathwayAcat1 , Acat2
propionic acidemia disease pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
pyruvate dehydrogenase complex deficiency pathwayAcat1
pyruvate dehydrogenase E1 deficiency pathwayAcat1
pyruvate kinase deficiency pathwayAcat1
pyruvate metabolic pathwayAcat1 , Acat2
risedronate pharmacodynamics pathwayAcat2 , Hmgcs1
saccharopinuria disease pathwayAcat1
short-chain acyl-CoA dehydrogenase deficiency pathwayAcat1
Smith-Lemli-Opitz Syndrome pathwayAcat2 , Hmgcs1
statin pharmacodynamics pathwayAcat2 , Hmgcs1
terpenoid biosynthetic pathwayAcat1 , Acat2 , Hmgcs1 , Hmgcs2
trifunctional protein deficiency pathwayAcat1
tryptophan metabolic pathwayAcat1 , Acat2
type I 3-Methylglutaconic aciduria disease pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
type III 3-Methylglutaconic aciduria disease pathwayAcat1 , Hmgcl , Hmgcs2 , Oxct1
valine, leucine and isoleucine degradation pathwayAcat1 , Acat2 , Hmgcl , Hmgcs1 , Hmgcs2 , Oxct1 , Oxct2a
very long-chain acyl-CoA dehydrogenase deficiency pathwayAcat1
Wolman disease pathwayAcat2 , Hmgcs1
zoledronate pharmacodynamics pathwayAcat2 , Hmgcs1

References Associated with the ketone bodies metabolic pathway:

Ontology Path Diagram:

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