FATTY ACID BETA DEGRADATION PATHWAY (PW:0000738)

View Ontology Report

Description

The degradation of fatty acids represents an essential source of energy; a major route is the mitochondrial beta-oxidation pathway. Before beta-oxidation can proceed, fatty acids need to be 'primed' or activated. The formation of fatty acyl-CoA thioesters is catalyzed by acyl-CoA synthetases in a two-step reaction. There are several acyl-CoA synthetases named after their chain length specificity. Once activated, acyl-CoAs need to reach the mitochondrial matrix where the enzymes of the beta-oxida

Pathway Diagram:

Ariadne Genomics Inc. NADH Hadha 3-ketoacyl-CoA Acaa2 3-hydroxyacyl-CoA Hadh Echs1 FADH2 CoQ e2 e1 Etfdh Etfb Cpt2 Slc25a20 Acs Cpt1 Acad CoQ ---> oxidative phosphorylation pathway carnitine-in carnitine-out acylcarnitine translocated fatty acyl-CoA Hadhb NAD ---> NADH NAD translocated fatty acyl-CoA ---> trans-2-enoyl-CoA fatty acids ---> fatty acyl-CoA 3-ketoacyl-CoA ---> acyl-CoA - 2C 3-hydroxyacyl-CoA <--> trans-2-enoyl-CoA 3-hydroxyacyl-CoA <--> 3-ketoacyl-CoA trans-2-enoyl-CoA fatty acids fatty acyl-CoA ---> acylcarnitine carnitine-out ---> acylcarnitine carnitine-in ---> carnitine-out acylcarnitine ---> carnitine-in acylcarnitine ---> translocated fatty acyl-CoA FADH2 ---> e1 FADH2 ---> e2 e1 ---> CoQ e2 ---> CoQ acetyl-CoA ---> citrate cycle pathway acetyl-CoA ---> ketone bodies metabolic pathway acyl-CoA - 2C ---> translocated fatty acyl-CoA 3-ketoacyl-CoA ---> acetyl-CoA FAD FAD ---> FADH2 fatty acyl-CoA acetyl-CoA acylcarnitine ---> translocated fatty acyl-CoA citrate cycle pathway ketone bodies metabolic pathway acyl-CoA - 2C Etfa oxidative phosphorylation pathway
GO TO:

Genes in Pathway:

show annotations for term's descendants       view all columns           Sort by:
 
fatty acid beta degradation pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Abcd1 ATP binding cassette subfamily D member 1 JBrowse link X 157,073,860 157,095,652 RGD:10402751
G Abcd2 ATP binding cassette subfamily D member 2 JBrowse link 7 132,294,564 132,343,169 RGD:10402751
G Acaa2 acetyl-CoA acyltransferase 2 JBrowse link 18 70,733,872 70,762,395 RGD:2317624
RGD:10402751
G Acad9 acyl-CoA dehydrogenase family, member 9 JBrowse link 2 122,782,051 122,806,166 RGD:2317589
G Acadl acyl-CoA dehydrogenase, long chain JBrowse link 9 73,833,368 73,871,857 RGD:2317589
RGD:10402751
RGD:2317678
G Acadm acyl-CoA dehydrogenase medium chain JBrowse link 2 260,124,418 260,148,589 RGD:2317589
RGD:10402751
RGD:2317678
G Acads acyl-CoA dehydrogenase short chain JBrowse link 12 47,254,503 47,263,747 RGD:2317589
RGD:10402751
RGD:2317678
G Acadvl acyl-CoA dehydrogenase, very long chain JBrowse link 10 56,619,321 56,624,468 RGD:2317589
RGD:2317683
G Acat1 acetyl-CoA acetyltransferase 1 JBrowse link 8 58,166,990 58,195,884 RGD:10402751
G Acsl1 acyl-CoA synthetase long-chain family member 1 JBrowse link 16 48,937,456 49,003,898 RGD:2317576
RGD:10402751
G Acsl3 acyl-CoA synthetase long-chain family member 3 JBrowse link 9 84,569,601 84,593,565 RGD:2317576
G Acsl4 acyl-CoA synthetase long-chain family member 4 JBrowse link X 113,596,247 113,660,024 RGD:2317576
G Acsl5 acyl-CoA synthetase long-chain family member 5 JBrowse link 1 276,240,703 276,290,012 RGD:2317576
G Acsl6 acyl-CoA synthetase long-chain family member 6 JBrowse link 10 39,654,771 39,717,592 RGD:2317576
G Acsm1 acyl-CoA synthetase medium-chain family member 1 JBrowse link 1 189,359,374 189,395,277 RGD:10402751
G Acsm2 acyl-CoA synthetase medium-chain family member 2 JBrowse link 1 189,289,957 189,329,007 RGD:2317576
G Acsm3 acyl-CoA synthetase medium-chain family member 3 JBrowse link 1 189,514,504 189,541,233 RGD:2317576
G Acsm4 acyl-CoA synthetase medium-chain family member 4 JBrowse link 1 189,432,604 189,458,799 RGD:2317576
G Acss1 acyl-CoA synthetase short-chain family member 1 JBrowse link 3 146,420,346 146,470,293 RGD:2317576
G Acss2 acyl-CoA synthetase short-chain family member 2 JBrowse link 3 151,032,925 151,075,856 RGD:2317576
G Acss3 acyl-CoA synthetase short-chain family member 3 JBrowse link 7 49,047,022 49,250,953 RGD:10402751
G Cpt1a carnitine palmitoyltransferase 1A JBrowse link 1 218,568,157 218,629,679 RGD:2317584
RGD:10402751
G Cpt1b carnitine palmitoyltransferase 1B JBrowse link 7 130,395,211 130,404,731 RGD:2317584
G Cpt2 carnitine palmitoyltransferase 2 JBrowse link 5 127,505,646 127,523,016 RGD:2317584
RGD:10402751
G Crat carnitine O-acetyltransferase JBrowse link 3 8,967,984 8,981,959 RGD:10402751
G Crot carnitine O-octanoyltransferase JBrowse link 4 22,079,837 22,116,265 RGD:10402751
G Echs1 enoyl-CoA hydratase, short chain 1 JBrowse link 1 212,570,213 212,579,040 RGD:2317616
RGD:10402751
G Etfa electron transfer flavoprotein alpha subunit JBrowse link 8 60,028,786 60,086,352 RGD:2317589
G Etfb electron transfer flavoprotein beta subunit JBrowse link 1 98,472,776 98,486,940 RGD:2317589
G Etfdh electron transfer flavoprotein dehydrogenase JBrowse link 2 178,367,547 178,389,641 RGD:2317589
G Hadh hydroxyacyl-CoA dehydrogenase JBrowse link 2 236,353,445 236,395,067 RGD:2302227
RGD:10402751
G Hadha hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha JBrowse link 6 27,589,840 27,628,921 RGD:2317625
RGD:10402751
G Hadhb hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta JBrowse link 6 27,555,408 27,589,539 RGD:2317625
RGD:10402751
G Hsd17b10 hydroxysteroid (17-beta) dehydrogenase 10 JBrowse link X 21,696,796 21,699,241 RGD:10402751
G Pex11g peroxisomal biogenesis factor 11 gamma JBrowse link 12 2,000,426 2,007,516 RGD:10402751
G Pex13 peroxisomal biogenesis factor 13 JBrowse link 14 108,394,299 108,411,994 RGD:10402751
G Pex14 peroxisomal biogenesis factor 14 JBrowse link 5 165,782,895 165,918,445 RGD:10402751
G Slc25a20 solute carrier family 25 member 20 JBrowse link 8 117,455,308 117,476,762 RGD:2317584
RGD:10402751

Additional Elements in Pathway:

(includes Gene Groups, Small Molecules, Other Pathways..etc.)
Object TypePathway ObjectPathway Object Description
Gene GroupACSacyl-CoA synthetase members
Gene GroupAcadacyl-Coenzyme A dehydrogenase members
Gene GroupCPT 1carnitine palmitoyltransferase system 1 members

Pathway Gene Annotations

Disease Annotations Associated with Genes in the fatty acid beta degradation pathway
Disease TermsGene Symbols
3-Hydroxyacyl-CoA Dehydrogenase DeficiencyHadh
AcidosisHadhb
Acute Kidney InjuryHadhb
Acyl-CoA Dehydrogenase Family, Member 9, Deficiency ofAcad9
Addison DiseaseAbcd1
Adenocarcinoma of LungHsd17b10
AdrenoleukodystrophyAbcd1 , Pex13
Alzheimer DiseaseAcadvl , Hadha , Hadhb , Hsd17b10
Amphetamine-Related DisordersAcsl6
ArteriosclerosisAcat1
Arthritis, ExperimentalAcsl1 , Acsl4 , Acsl5
Autism Spectrum DisorderCpt1b
Beta Ketothiolase DeficiencyAcat1
Brain Diseases, MetabolicCpt2
Breast NeoplasmsCpt1a , Hadhb
Carbohydrate Metabolism, Inborn ErrorsAcat1
CarcinomaAcsl1
Carcinoma, HepatocellularAcsl4 , Etfa
CardiomegalyCpt1b
Cardiomyopathy, DilatedAcadvl
Cardiomyopathy, HypertrophicAcadvl
Cardiomyopathy, Hypertrophic, FamilialAcadvl
Carnitine Palmitoyl Transferase 1A DeficiencyCpt1a
Carnitine Palmitoyl Transferase 2 DeficiencyCpt2
Carnitine Palmitoyltransferase II Deficiency, InfantileCpt2
Carnitine Palmitoyltransferase II Deficiency, Late-OnsetCpt2
Carnitine Palmitoyltransferase II Deficiency, Lethal NeonatalCpt2
Carnitine-Acylcarnitine Translocase DeficiencySlc25a20
Chemical and Drug Induced Liver InjuryHadha
Colorectal NeoplasmsAbcd2
Congenital HyperinsulinismHadh
Depressive DisorderAcsl4
Diabetes MellitusCpt1a , Hadh
Diabetes Mellitus, ExperimentalAcadvl , Acsl1 , Acsl6 , Cpt1a , Cpt1b , Hadh , Hadha
Diabetes Mellitus, Type 2Cpt1a
ENCEPHALOPATHY, ACUTE, INFECTION-INDUCED, SUSCEPTIBILITY TO, 4Cpt2
Enterocolitis, NecrotizingAcadm
ErythemaAcsl4
Fatty LiverHadha , Hadhb
Fetal Growth RetardationHadha
Genetic Diseases, InbornAcat1 , Etfb , Etfdh
Glomerulonephritis, IGAAcsm3
Glutaric Acidemia IAcadvl
Heart DiseasesHadhb
Heat Stress DisordersAcadvl
Hepatitis C, ChronicCpt1a
Hydrocephalus, Nonsyndromic, Autosomal Recessive 2Acadm
Hydroxyacyl-CoA Dehydrogenase, Type 2, DeficiencyHsd17b10
Hyperinsulinemic Hypoglycemia, Familial, 4Hadh
HyperinsulinismHadh
HypertensionAcsm3
Hypertension, EssentialAcsm3
Hypertrophy, Left VentricularCrot
HypoxiaAcadvl
InflammationAcsl6
Inflammatory Bowel Disease 11Acsm3
Insulin ResistanceAcsl1
Intellectual DisabilityAcsl4
Kidney Failure, ChronicAcat1 , Cpt1a
Leigh DiseaseEchs1
LeukemiaAcsl6
Leukemia, Myeloid, AcuteAcsl6
Lipid Metabolism DisordersHadhb
Lipid Metabolism, Inborn ErrorsCpt1a , Cpt2
Liver Cirrhosis, ExperimentalAcat1 , Acsl1 , Crot
Liver FailureAcadm
Liver NeoplasmsCpt1b
Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase DeficiencyHadha , Hadhb
Lung NeoplasmsAcsm1
Malformations of Cortical Development, Group IIAcsm2
Mammary Neoplasms, AnimalAcsl1
Mammary Neoplasms, ExperimentalAcsl1
Medium Chain Acyl CoA Dehydrogenase DeficiencyAcadm
Mental Retardation, X-Linked 63Acsl4
Metabolic DiseasesEtfdh
Metabolic Syndrome XAcadvl
Mitochondrial DiseasesHadhb
MITOCHONDRIAL SHORT-CHAIN ENOYL-COA HYDRATASE 1 DEFICIENCYEchs1
Multiple Acyl Coenzyme A Dehydrogenase DeficiencyEtfa , Etfb , Etfdh
Muscular DiseasesAcadvl , Cpt2
Myocardial InfarctionHadh
Myocardial IschemiaCpt1b , Hadha
myopathyAcadvl
NarcolepsyCpt1b
Nephrotic SyndromeAcat1
Non-alcoholic Fatty Liver DiseaseAcadm , Acadvl
ObesityAcadm , Acsl1 , Cpt1a , Echs1 , Etfdh , Hadh
Ocular HypertensionAcsm3
OsteoarthritisAcaa2 , Etfa , Hadha
Ovarian CystsPex14
OverweightAcsm3
Pericardial EffusionHadhb
Peroxisome Biogenesis Disorder 11A (Zellweger)Pex13
Peroxisome Biogenesis Disorder 11BPex13
Peroxisome Biogenesis Disorder 2BPex13
Peroxisome Biogenesis Disorder, Complementation Group KPex14
Peroxisome Biogenesis DisordersPex13 , Pex14
Pleomorphic Xanthoastrocytoma Crot
Precancerous ConditionsHadh
Prostatic NeoplasmsAcsl4 , Acsm3
Respiratory Distress Syndrome, AdultAcaa2
RhabdomyolysisHadhb
SeizuresAcat1
Short Chain Acyl CoA Dehydrogenase DeficiencyAcads
StarvationAcsl1
Stomach NeoplasmsEchs1
Trifunctional Protein Deficiency with Myopathy and NeuropathyHadha , Hadhb
VLCAD DeficiencyAcadl , Acadvl
Weight GainAcadm
Zellweger SyndromePex13 , Pex14
Pathway Annotations Associated with Genes in the fatty acid beta degradation pathway
Pathway TermsGene Symbols
2-aminoadipic 2-oxoadipic aciduria disease pathwayAcat1 , Echs1 , Hadh
3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
3-hydroxyacyl-CoA dehydrogenase deficiency pathwayAcaa2 , Acadl , Acads , Acat1 , Acss3 , Echs1 , Hadh , Hsd17b10
3-hydroxyisobutyric aciduria pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
3-methylcrotonyl CoA carboxylase 1 deficiency pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
adrenoleukodystrophy pathwayAbcd1 , Abcd2 , Acsl1 , Cpt2 , Crat , Crot , Pex11g , Pex13 , Pex14 , Slc25a20
Alzheimer disease pathwayHsd17b10
beta-alanine metabolic pathwayAcadm , Echs1 , Hadha
butanoate metabolic pathwayAcads , Acat1 , Acsm1 , Acsm3 , Acsm4 , Echs1 , Hadh , Hadha
carnitine palmitoyl transferase deficiency type I pathwayAcaa2 , Acadl , Acadm , Acads , Acadvl , Acat1 , Acsl1 , Cpt1a , Cpt2 , Echs1 , Hadha , Hadhb
carnitine-acylcarnitine translocase deficiencyAbcd1 , Abcd2 , Acsl1 , Cpt2 , Crat , Crot , Pex11g , Pex13 , Pex14 , Slc25a20
disulfiram pharmacodynamics pathwayAcss1 , Acss2
eicosanoid signaling pathway via peroxisome proliferator-activated receptor gammaAcadl , Acadm , Acsl1 , Acsl3 , Acsl4 , Acsl5 , Acsl6 , Cpt1a , Cpt1b , Cpt2
ethylmalonic encephalopathy disease pathwayAcaa2 , Acadl , Acadm , Acads , Acadvl , Acat1 , Acsl1 , Cpt1a , Cpt2 , Echs1 , Hadha , Hadhb
fatty acid beta degradation pathwayAbcd1 , Abcd2 , Acaa2 , Acad9 , Acadl , Acadm , Acads , Acadvl , Acat1 , Acsl1 , Acsl3 , Acsl4 , Acsl5 , Acsl6 , Acsm1 , Acsm2 , Acsm3 , Acsm4 , Acss1 , Acss2 , Acss3 , Cpt1a , Cpt1b , Cpt2 , Crat , Crot , Echs1 , Etfa , Etfb , Etfdh , Hadh , Hadha , Hadhb , Hsd17b10 , Pex11g , Pex13 , Pex14 , Slc25a20
fatty acid elongation pathwayAcaa2 , Echs1 , Hadh , Hadha , Hadhb , Hsd17b10
fatty acid metabolic pathwayAcaa2 , Acadl , Acadm , Acads , Acadvl , Acat1 , Acsl1 , Acsl3 , Acsl4 , Acsl5 , Acsl6 , Cpt1a , Cpt1b , Cpt2 , Echs1 , Hadh , Hadha , Hadhb
forkhead class A signaling pathwayAcadm , Acadvl , Cpt1a , Cpt1b , Hadh
gluconeogenesis pathwayAcss1 , Acss2
glutaric aciduria type I disease pathwayAcaa2 , Acadl , Acadm , Acads , Acadvl , Acat1 , Acsl1 , Cpt1a , Cpt2 , Echs1 , Hadh , Hadha , Hadhb
glycolysis pathwayAcss1 , Acss2
glycolysis/gluconeogenesis pathwayAcss1 , Acss2
glyoxylate and dicarboxylate metabolic pathwayAcat1
hyperlysinemias disease pathwayAcat1 , Echs1 , Hadh
isobutyryl-CoA dehydrogenase deficiency pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
isovaleric acidemia disease pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
ketone bodies metabolic pathwayAcat1
Leigh syndrome pathwayAcat1 , Acss2
lysine degradation pathwayAcat1 , Echs1 , Hadh , Hadha
malonic aciduria disease pathwayAcadm , Acat1 , Acss1 , Acss3 , Echs1
malonic aciduria pathwayAcadm , Acat1 , Acss1 , Acss3 , Echs1
maple syrup urine disease pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
medium-chain acyl-CoA dehydrogenase deficiency pathwayAcaa2 , Acadl , Acadm , Acads , Acadvl , Acat1 , Acsl1 , Cpt1a , Cpt2 , Echs1 , Hadha , Hadhb
methylmalonate semialdehyde dehydrogenase deficiency pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
methylmalonic acidemia disease pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
methylmalonic aciduria, cobalamin-related, disease pathwayAcadm , Acat1 , Acss1 , Acss3 , Echs1
phytanic acid degradation pathwayAbcd1 , Abcd2
primary hyperoxaluria type 2 disease pathwayAcat1 , Acss2
propanoate metabolic pathwayAcadm , Acat1 , Acss1 , Acss2 , Acss3 , Echs1 , Hadha
propionic acidemia disease pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
pyruvate dehydrogenase complex deficiency pathwayAcat1 , Acss2
pyruvate dehydrogenase E1 deficiency pathwayAcat1 , Acss2
pyruvate kinase deficiency pathwayAcat1 , Acss2
pyruvate metabolic pathwayAcat1 , Acss1 , Acss2
Refsum disease pathwayAbcd1 , Abcd2
saccharopinuria disease pathwayAcat1 , Echs1 , Hadh
short-chain acyl-CoA dehydrogenase deficiency pathwayAcaa2 , Acadl , Acadm , Acads , Acadvl , Acat1 , Acsl1 , Cpt1a , Cpt2 , Echs1 , Hadha , Hadhb
succinyl-CoA:3-oxoacid transferase deficiency pathwayAcat1
terpenoid biosynthetic pathwayAcat1
trifunctional protein deficiency pathwayAcaa2 , Acadl , Acadm , Acads , Acadvl , Acat1 , Acsl1 , Cpt1a , Cpt2 , Echs1 , Hadha , Hadhb
tryptophan metabolic pathwayAcat1 , Echs1 , Hadh , Hadha
type I 3-Methylglutaconic aciduria disease pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
type III 3-Methylglutaconic aciduria disease pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
unsaturated fatty acid biosynthetic pathwayHadha
valine, leucine and isoleucine degradation pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hadh , Hadha , Hadhb , Hsd17b10
valproic acid pharmacokinetics pathwayAcsm1 , Hadha , Hadhb , Hsd17b10
very long-chain acyl-CoA dehydrogenase deficiency pathwayAcaa2 , Acadl , Acadm , Acads , Acadvl , Acat1 , Acsl1 , Cpt1a , Cpt2 , Echs1 , Hadha , Hadhb

References Associated with the fatty acid beta degradation pathway:

Ontology Path Diagram:

paths to the root

Import into Pathway Studio: