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 subunit alpha JBrowse link 8 60,028,786 60,086,352 RGD:2317589
G Etfb electron transfer flavoprotein subunit beta 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
acute myeloid leukemiaAcsl6
Acyl-CoA Dehydrogenase Family, Member 9, Deficiency ofAcad9
Addison's diseaseAbcd1
adrenoleukodystrophyAbcd1 , Pex13
adult respiratory distress syndromeAcaa2
Alzheimer's diseaseAcadvl , Hadha , Hadhb , Hsd17b10
amphetamine abuseAcsl6
Animal Disease ModelsAcsl4
Animal Mammary NeoplasmsAcsl1
arteriosclerosisAcat1
asthmaAcsl3
autism spectrum disorderCpt1b
azoospermiaHsd17b10
beta-ketothiolase deficiencyAcat1
breast cancerAcsl3
Breast NeoplasmsCpt1a , Hadhb
carbohydrate metabolic disorderAcat1
carcinomaAcsl1
CardiomegalyCpt1b
cardiomyopathyAcsl1
carnitine palmitoyltransferase I deficiencyCpt1a
carnitine palmitoyltransferase II deficiencyCpt2
Carnitine Palmitoyltransferase II Deficiency, InfantileCpt2
Carnitine Palmitoyltransferase II Deficiency, Lethal NeonatalCpt2
Carnitine-Acylcarnitine Translocase DeficiencySlc25a20
Chemical and Drug Induced Liver InjuryAcsl1 , Echs1 , Hadha
Colorectal NeoplasmsAbcd2
Congenital HyperinsulinismHadh
diabetes mellitusCpt1a , Hadh
Diabetes Mellitus, Experimental Acadvl , Acsl1 , Acsl3 , Acsl6 , Cpt1a , Cpt1b , Hadh , Hadha
dilated cardiomyopathyAcadvl
disease of metabolismEtfdh
encephalitisAbcd1
ENCEPHALOPATHY, ACUTE, INFECTION-INDUCED, SUSCEPTIBILITY TO, 4Cpt2
Encephalopathy, Neonatal Severe, due to Mecp2 MutationsAbcd1
end stage renal failureAcat1 , Cpt1a
ErythemaAcsl4
essential hypertensionAcsm3
Experimental Allergic AsthmaAcsl3
Experimental ArthritisAcsl1 , Acsl4 , Acsl5
Experimental Liver CirrhosisAcat1 , Acsl1 , Crot
Experimental MelanomaAcss1 , Acss2
familial hyperinsulinemic hypoglycemia 4Hadh
familial hypertrophic cardiomyopathyAcadvl
fatty liver diseaseHadha , Hadhb
Fetal Growth RetardationHadha
Gait Disorders, NeurologicAbcd1
genetic diseaseAbcd1 , Acat1 , Acsl4 , Cpt2 , Echs1 , Etfb , Etfdh , Hadha , Hsd17b10
Genu ValgumCpt2
Glutaric Acidemia IAcadvl
heart diseaseHadhb
Heat Stress DisordersAcadvl
Hepatitis C, ChronicCpt1a
hepatocellular carcinomaAcsl4 , Etfa
Hereditary Neoplastic SyndromesAcsl6
hereditary spastic paraplegia 17Acad9
hyperinsulinismHadh
hypertensionAcsm3
HypothermiaAcss1
hypothyroidismAbcd2
HypoxiaAcadvl
IgA glomerulonephritisAcsm3
InflammationAcsl3 , Acsl4 , Acsl6 , Acss2
Insulin ResistanceAcsl1
intellectual disabilityAcsl4
Late-Onset Carnitine Palmitoyltransferase II DeficiencyCpt2
Left Ventricular HypertrophyCrot
Leigh diseaseEchs1
leukemiaAcsl6
lipid metabolism disorderCpt1a , Cpt2 , Hadhb
liver benign neoplasmCpt1b
Liver FailureAcadm
long QT syndromeAcss2
Long-Chain 3-hydroxyacyl-CoA Dehydrogenase DeficiencyHadha , Hadhb
lung cancerAcsl3
Lung NeoplasmsAcsm1
Malformations of Cortical Development, Group IIAcsm2
Mammary Neoplasms, ExperimentalAcsl1
medium chain acyl-CoA dehydrogenase deficiencyAcadm
mental depressionAcsl4
Mental Retardation, X-Linked 63Acsl4
Metabolic Brain DiseasesCpt2
Metabolic SyndromeAcadvl
mitochondrial complex I deficiencyAcad9
mitochondrial metabolism diseaseHadhb
MITOCHONDRIAL SHORT-CHAIN ENOYL-COA HYDRATASE 1 DEFICIENCYEchs1
morbid obesityAcss2
multiple acyl-CoA dehydrogenase deficiencyEtfa , Etfb , Etfdh
muscular diseaseAcadvl , Cpt2
myocardial infarctionHadh
Myocardial IschemiaCpt1b , Hadha
myopathyAcadvl
narcolepsyCpt1b
nephrotic syndromeAcat1
neurodegeneration with brain iron accumulationCrat
NEURODEGENERATION WITH BRAIN IRON ACCUMULATION 8Crat
non-syndromic X-linked intellectual disabilityAcsl4
nonalcoholic fatty liver diseaseAcadm , Acadvl
Nonsyndromic Hydrocephalus, Autosomal Recessive 2Acadm
obesityAcadm , Acsl1 , Cpt1a , Echs1 , Etfdh , Hadh
ocular hypertensionAcsm3
orofacial cleftAcss2
osteoarthritisAcaa2 , Etfa , Hadha
osteosarcomaHsd17b10
ovarian cystPex14
OverweightAcsm3
pancytopeniaCpt2
paraplegiaAbcd1
Pearson syndromeAcadvl
perinatal necrotizing enterocolitisAcadm
Peroxisome Biogenesis Disorder 11A (Zellweger)Pex13
Peroxisome Biogenesis Disorder 11BPex13
PEROXISOME BIOGENESIS DISORDER 13A (ZELLWEGER)Pex14
Peroxisome Biogenesis Disorder 2BPex13
Peroxisome Biogenesis Disorder, Complementation Group KPex14
Peroxisome Biogenesis DisordersPex13 , Pex14
pheochromocytomaHsd17b10
pleomorphic xanthoastrocytomaCrot
pre-malignant neoplasmHadh
prostate cancerAcsl3
Prostatic NeoplasmsAcsl4 , Acsm3
RhabdomyolysisCpt2
short chain acyl-CoA dehydrogenase deficiencyAcads
spinal muscular atrophyEtfdh
StarvationAcsl1
Stomach NeoplasmsEchs1
Sudden Death, CardiacAcsl3
syndromic X-linked intellectual disability type 10Hsd17b10
Trifunctional Protein Deficiency with Myopathy and NeuropathyHadha , Hadhb
type 2 diabetes mellitusCpt1a
ulcerative colitisAcsm3
very long chain acyl-CoA dehydrogenase deficiencyAcadl , Acadvl
visual epilepsyAcat1
Weight GainAcadm
Weissenbacher-Zweymuller syndromeAcadvl
Zellweger syndromePex13 , Pex14
Pathway Annotations Associated with Genes in the fatty acid beta degradation pathway
Pathway TermsGene Symbols
2-aminoadipic 2-oxoadipic aciduria 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
3-methylglutaconic aciduria type 1 pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
3-methylglutaconic aciduria type 3 pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
adrenoleukodystrophy pathwayAbcd1 , Abcd2 , Acsl1 , Cpt2 , Crat , Crot , Pex11g , Pex13 , Pex14 , Slc25a20
Alzheimer's disease pathwayHsd17b10
beta-alanine metabolic pathwayAcadm , Echs1 , Hadha
butanoate metabolic pathwayAcads , Acat1 , Acsm1 , Acsm3 , Acsm4 , Echs1 , Hadh , Hadha
carnitine palmitoyltransferase I deficiency 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 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 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
hyperlysinemia pathwayAcat1 , Echs1 , Hadh
isobutyryl-CoA dehydrogenase deficiency pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
isovaleric acidemia pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
ketone bodies metabolic pathwayAcat1
Leigh disease pathwayAcat1 , Acss2
lysine degradation pathwayAcat1 , Echs1 , Hadh , Hadha
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 pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
methylmalonic aciduria, cobalamin-related pathwayAcadm , Acat1 , Acss1 , Acss3 , Echs1
phytanic acid degradation pathwayAbcd1 , Abcd2
primary hyperoxaluria type 2 pathwayAcat1 , Acss2
propanoate metabolic pathwayAcadm , Acat1 , Acss1 , Acss2 , Acss3 , Echs1 , Hadha
propionic acidemia pathwayAcaa2 , Acadm , Acads , Acat1 , Echs1 , Hsd17b10
pyruvate decarboxylase deficiency pathwayAcat1 , Acss2
pyruvate dehydrogenase E1 deficiency pathwayAcat1 , Acss2
pyruvate kinase deficiency of red cells pathwayAcat1 , Acss2
pyruvate metabolic pathwayAcat1 , Acss1 , Acss2
Refsum disease pathwayAbcd1 , Abcd2
saccharopinuria 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
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: