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CITRIC ACID CYCLE PATHWAY (PW:0000026)

View Ontology Report

Description

The citric acid cycle, also known as the Krebs or tricarboxylic acid cycle (TCA), is considered a hub of cellular fuel metabolism. It consists of a series of eight reactions that oxidize the acetyl group of acetyl-CoA - the only precursor of the cycle - to two molecules of CO2 while conserving the liberated energy in the reduced NADH and FADH2 compounds. The stored electrons are passed to oxygen via the electron transport chain and the generated electrochemical gradien

Pathway Diagram:

Ariadne Genomics Inc. Aco2 Idh3 complex alpha-keto complex Scs complex Sdh complex Fh Mdh2 Cs succinyl-CoA alpha-ketoglutarate ---> succinyl-CoA isocitrate ---> alpha-ketoglutarate amino acid metabolic pathway gluconeogenesis pathway fatty acid biosynthetic pathway fatty acid beta degradation pathway ketone bodies metabolic pathway pyruvate metabolic pathway alpha-ketoglutarate <--> amino acid metabolic pathway FADH2 alpha-ketoglutarate succinate fumarate isocitrate malate <--> fumarate isocitrate <--> citrate glycolysis pathway isocitrate ---> NADH1 citrate oxaloacetate fumarate <--> succinate amino acid metabolic pathway <--> pyruvate amino acid metabolic pathway ---> succinyl-CoA succinyl-CoA <--> succinate oxaloacetate <--> amino acid metabolic pathway amino acid metabolic pathway ---> acetyl-CoA malate ---> NADH3 amino acid metabolic pathway ---> fumarate acetyl-CoA ---> citrate oxaloacetate ---> citrate succinate ---> FADH2 pyruvate ---> acetyl-CoA pyruvate malate alpha-ketoglutarate ---> NADH2 malate <--> oxaloacetate pyruvate ---> oxaloacetate fatty acid beta degradation pathway ---> acetyl-CoA acetyl-CoA acetyl-CoA <--> ketone bodies metabolic pathway citrate ---> fatty acid biosynthetic pathway glycolysis pathway ---> pyruvate NADH1 NADH2 NADH3 pyruvate ---> gluconeogenesis pathway
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Genes in Pathway:


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citric acid cycle pathway term browser
Symbol Object Name Evidence Notes Source PubMed Reference(s) RGD Reference(s) Position
G Acly ATP citrate lyase IEA KEGG rno:00020 NCBI chr10:85,412,045...85,464,253
Ensembl chr10:85,412,049...85,463,320
JBrowse link
G Aco1 aconitase 1 IEA KEGG rno:00020 NCBI chr 5:55,259,841...55,315,872
Ensembl chr 5:55,259,827...55,316,391
JBrowse link
G Aco2 aconitase 2 IEA
ISO
IDA
KEGG
SMPDB
RGD
PMID:9712727 SMP:00057 rno:00020, RGD:2306877 NCBI chr 7:113,385,677...113,428,794
Ensembl chr 7:113,385,646...113,428,261
JBrowse link
G Cs citrate synthase IEA
ISO
IDA
KEGG
SMPDB
RGD
PMID:9712727 SMP:00057 rno:00020, RGD:2306877 NCBI chr 7:758,074...791,421
Ensembl chr 7:758,345...791,421
JBrowse link
G Dlat dihydrolipoamide S-acetyltransferase IEA
ISO
KEGG
SMPDB
SMP:00057 rno:00020 NCBI chr 8:50,979,151...51,004,435
Ensembl chr 8:50,978,051...51,004,479
Ensembl chr 1:50,978,051...51,004,479
JBrowse link
G Dld dihydrolipoamide dehydrogenase ISO
IEA
SMPDB
KEGG
RGD
PMID:10672230 SMP:00057 rno:00020, RGD:2306878 NCBI chr 6:47,904,153...47,924,814
Ensembl chr 6:47,903,914...47,924,795
JBrowse link
G Dlst dihydrolipoamide S-succinyltransferase ISO
IEA
SMPDB
KEGG
RGD
PMID:10672230 SMP:00057 rno:00020, RGD:2306878 NCBI chr 6:104,758,511...104,783,296
Ensembl chr 6:104,758,631...104,783,296
JBrowse link
G Fh fumarate hydratase ISO SMPDB SMP:00057 NCBI chr13:87,524,331...87,550,215
Ensembl chr13:87,524,337...87,550,266
JBrowse link
G Idh1 isocitrate dehydrogenase (NADP(+)) 1 IEA KEGG rno:00020 NCBI chr 9:66,534,146...66,563,703
Ensembl chr 9:66,534,146...66,563,708
JBrowse link
G Idh2 isocitrate dehydrogenase (NADP(+)) 2 IEA KEGG rno:00020 NCBI chr 1:134,038,644...134,057,969
Ensembl chr 1:134,029,772...134,058,025
JBrowse link
G Idh3a isocitrate dehydrogenase (NAD(+)) 3 catalytic subunit alpha IEA
ISO
KEGG
SMPDB
SMP:00057 rno:00020 NCBI chr 8:54,971,694...54,991,085
Ensembl chr 8:54,971,740...54,991,084
JBrowse link
G Idh3b isocitrate dehydrogenase (NAD(+)) 3 non-catalytic subunit beta IEA
ISO
KEGG
SMPDB
SMP:00057 rno:00020 NCBI chr 3:117,481,845...117,486,909
Ensembl chr 3:117,481,845...117,486,982
JBrowse link
G Idh3g isocitrate dehydrogenase (NAD(+)) 3 non-catalytic subunit gamma IEA
ISO
KEGG
SMPDB
SMP:00057 rno:00020 NCBI chr  X:151,515,244...151,524,175
Ensembl chr  X:151,515,247...151,524,171
JBrowse link
G Mdh1 malate dehydrogenase 1 IEA KEGG rno:00020 NCBI chr14:95,630,625...95,645,920
Ensembl chr14:95,630,306...95,645,925
JBrowse link
G Mdh2 malate dehydrogenase 2 IEA
ISO
KEGG
SMPDB
SMP:00057 rno:00020 NCBI chr12:20,894,269...20,907,225
Ensembl chr12:20,894,262...20,907,271
JBrowse link
G Mpc1 mitochondrial pyruvate carrier 1 ISO SMPDB SMP:00057 NCBI chr 1:52,437,745...52,449,399
Ensembl chr 1:52,437,741...52,449,400
JBrowse link
G Ogdh oxoglutarate dehydrogenase IDA
ISO
IEA
SMPDB
KEGG
RGD
PMID:9712727 PMID:10672230 SMP:00057 rno:00020, RGD:2306877, RGD:2306878 NCBI chr14:81,150,021...81,217,479
Ensembl chr14:81,150,091...81,217,479
JBrowse link
G Ogdhl oxoglutarate dehydrogenase L IEA KEGG rno:00020 NCBI chr16:7,578,343...7,604,385
Ensembl chr16:7,578,367...7,604,386
JBrowse link
G Pc pyruvate carboxylase IEA
ISO
KEGG
SMPDB
SMP:00057 rno:00020 NCBI chr 1:201,799,374...201,898,412
Ensembl chr 1:201,804,267...201,898,380
JBrowse link
G Pck1 phosphoenolpyruvate carboxykinase 1 IEA KEGG rno:00020 NCBI chr 3:161,930,256...161,936,205
Ensembl chr 3:161,930,256...161,936,191
JBrowse link
G Pck2 phosphoenolpyruvate carboxykinase 2 (mitochondrial) IEA KEGG rno:00020 NCBI chr15:29,027,891...29,036,729
Ensembl chr15:29,027,894...29,037,283
JBrowse link
G Pdha1 pyruvate dehydrogenase E1 subunit alpha 1 IEA
ISO
KEGG
SMPDB
SMP:00057 rno:00020 NCBI chr  X:34,700,481...34,714,309
Ensembl chr  X:34,700,409...34,714,311
JBrowse link
G Pdha2 pyruvate dehydrogenase E1 subunit alpha 2 IEA KEGG rno:00020 NCBI chr 2:229,872,300...229,873,848 JBrowse link
G Pdhb pyruvate dehydrogenase E1 subunit beta IEA
ISO
KEGG
SMPDB
SMP:00057 rno:00020 NCBI chr15:16,752,561...16,758,503
Ensembl chr15:16,750,980...16,758,500
JBrowse link
G Sdha succinate dehydrogenase complex flavoprotein subunit A ISO
IEA
SMPDB
KEGG
RGD
PMID:16143825 SMP:00057 rno:00020, RGD:2306881 NCBI chr 1:28,935,965...28,960,936
Ensembl chr 1:28,940,164...28,961,535
JBrowse link
G Sdhb succinate dehydrogenase complex iron sulfur subunit B ISO
IEA
SMPDB
KEGG
RGD
PMID:16143825 SMP:00057 rno:00020, RGD:2306881 NCBI chr 5:153,264,906...153,285,570
Ensembl chr 5:153,264,899...153,314,293
JBrowse link
G Sdhc succinate dehydrogenase complex subunit C ISO
IEA
SMPDB
KEGG
RGD
PMID:16143825 SMP:00057 rno:00020, RGD:2306881 NCBI chr13:83,544,652...83,565,560
Ensembl chr13:83,544,652...83,566,253
JBrowse link
G Sdhd succinate dehydrogenase complex subunit D ISO
IEA
SMPDB
KEGG
RGD
PMID:16143825 SMP:00057 rno:00020, RGD:2306881 NCBI chr 8:50,944,702...50,954,298
Ensembl chr 8:50,944,704...50,954,238
JBrowse link
G Sucla2 succinate-CoA ligase ADP-forming subunit beta IDA
IEA
KEGG
RGD
PMID:17403370 rno:00020, RGD:2306915 NCBI chr15:48,752,356...48,805,495
Ensembl chr15:48,752,312...48,805,138
JBrowse link
G Suclg1 succinate-CoA ligase GDP/ADP-forming subunit alpha IDA
ISO
IEA
SMPDB
KEGG
RGD
PMID:17403370 SMP:00057 rno:00020, RGD:2306915 NCBI chr 4:105,308,236...105,337,595
Ensembl chr 4:105,308,039...105,337,600
JBrowse link
G Suclg2 succinate-CoA ligase GDP-forming subunit beta IDA
ISO
IEA
SMPDB
KEGG
RGD
PMID:17403370 SMP:00057 rno:00020, RGD:2306915 NCBI chr 4:128,067,031...128,337,146
Ensembl chr 4:128,067,033...128,337,170
JBrowse link
altered citric acid cycle pathway term browser
Symbol Object Name Evidence Notes Source PubMed Reference(s) RGD Reference(s) Position
G Fh fumarate hydratase ISO RGD PMID:17211469 RGD:6907135 NCBI chr13:87,524,331...87,550,215
Ensembl chr13:87,524,337...87,550,266
JBrowse link
G Sdhb succinate dehydrogenase complex iron sulfur subunit B ISO RGD PMID:21771581 RGD:6907134 NCBI chr 5:153,264,906...153,285,570
Ensembl chr 5:153,264,899...153,314,293
JBrowse link
G Sdhd succinate dehydrogenase complex subunit D ISO RGD PMID:21771581 RGD:6907134 NCBI chr 8:50,944,702...50,954,298
Ensembl chr 8:50,944,704...50,954,238
JBrowse link

Additional Elements in Pathway:

(includes Gene Groups, Small Molecules, Other Pathways..etc.)
Object TypePathway ObjectPathway Object Description
Complexalpha keto complexalpha keto complex
ComplexIdh3 complexisocitrate dehydrogenase complex
ComplexSdh complexsuccinate dehydrogenase complex
ComplexScs ComplexSuccinyl-CoA synthetase complex catalyzes the reversible interconversion of succinyl-CoA and succinate and releases GTP or ATP.
ProteinPDB, October 2012 molecule of the month, TCA cycleThe structures of proteins/complexes of the eight reactions of the cycle, at the Protein Data Bank (the link is also provided at the end of the description)

Pathway Gene Annotations

Disease Annotations Associated with Genes in the citric acid cycle pathway
Disease TermsGene Symbols
2-hydroxyglutaric aciduriaIdh2
acute intermittent porphyriaAco2
Acute Liver FailureIdh1 , Mdh1
acute lymphoblastic leukemiaIdh1
acute myeloid leukemiaIdh1 , Idh2
acute myocardial infarctionIdh2 , Sdhb
acute promyelocytic leukemiaIdh1 , Idh2
adenoid cystic carcinomaIdh1
adenylosuccinase lyase deficiencyAco2
adrenoleukodystrophyIdh3g
adult respiratory distress syndromeAco2 , Mdh1
Aicardi-Goutieres Syndrome 3Pc
Alzheimer's diseaseDlst , Pck1
Animal Disease ModelsIdh2 , Suclg2
Animal Mammary NeoplasmsMdh2
ataxia telangiectasiaDlat , Sdhd
atrial fibrillationCs
autistic disorderIdh3g , Pdha1
autoimmune diseaseIdh1
autoimmune interstitial lung, joint, and kidney diseaseSdhc
autosomal hemophilia AIdh3g
azoospermiaPdha2
B-lymphoblastic leukemia/lymphomaFh
B-lymphoblastic leukemia/lymphoma with hypodiploidySdha
Bannayan-Riley-Ruvalcaba syndromeSdhb
Bardet-Biedl syndromeMdh1
Barth syndromeIdh3g
BH4-deficient hyperphenylalaninemia ADlat , Sdhd
bilateral breast cancerSdhb
bipolar disorderCs , Pc
Bloom syndromeIdh2 , Idh3a
bone remodeling diseaseIdh2
brain diseaseMdh2
Brain Hypoxia-IschemiaCs
brain ischemiaMdh2
Brain NeoplasmsIdh1 , Sdha
Brain-Lung-Thyroid SyndromePck2
breast cancerFh , Sdhb , Sdhc
Breast Cancer, FamilialSdha
Breast NeoplasmsIdh1
Burkitt lymphomaPc
carcinomaMdh2
CardiomegalyCs , Idh2
cardiomyopathyCs
Carney TriadSdha , Sdhb , Sdhc
Carney-Stratakis syndromeDlat , Sdhb , Sdhc , Sdhd
Carotid Body TumorSdhb
celiac diseasePck1
central nervous system diseaseIdh2
cerebral creatine deficiency syndrome 1Idh3g
Charcot-Marie-Tooth diseaseSdhc
Charcot-Marie-Tooth disease dominant intermediate DSdhc
Charcot-Marie-Tooth disease intermediate typeSdhc
Charcot-Marie-Tooth disease type 1Sdhc
Charcot-Marie-Tooth disease type 1BSdhc
Charcot-Marie-Tooth disease type 2ISdhc
Charcot-Marie-Tooth disease type 4ESdhc
Chemical and Drug Induced Liver InjuryDlat , Idh2 , Mdh1 , Ogdh , Pc
cholangiocarcinomaIdh1 , Idh2
CholangiofibrosisIdh1
chondromaIdh1 , Idh2
chromosome 11 partial duplication syndromeDlat , Sdhd
chromosome 13q14 deletion syndromeSucla2
chromosome 1p36 deletion syndromeSdhb
Chromosome 7q11.23 Deletion Syndrome, Distal, 1.2-MBMdh2
Chromosome Xq28 Duplication SyndromeIdh3g
Chronic Allograft NephropathyAco1 , Cs
Chronic Cerebral HypoperfusionAco1
Chronic Intermittent HypoxiaPck1
Cockayne syndrome BOgdhl
Coffin-Lowry syndromePdha1
colorectal cancerIdh2 , Idh3a
combined oxidative phosphorylation deficiency 8Sdhd
common variable immunodeficiency 4Aco2
congenital disorder of glycosylation IlDlat
congenital disorder of glycosylation IyIdh3g
Congenital Infantile Lactic AcidosisPc
Congenital Infantile Lactic Acidosis due to LAD DeficiencyDld
congestive heart failureAcly , Cs , Mdh2 , Ogdhl , Pck1
COVID-19Aco2 , Fh , Idh1 , Pck2 , Sdhd
Cowden syndromeSdhb
Craniofacial AbnormalitiesIdh2
D-2-hydroxyglutaric aciduriaIdh2
D-2-hydroxyglutaric aciduria 2Idh2
depressive disorderOgdhl
developmental and epileptic encephalopathy 1Mdh2
developmental and epileptic encephalopathy 2Pdha1
developmental and epileptic encephalopathy 51Mdh2
developmental and epileptic encephalopathy 88Mdh1
Developmental DisabilitiesAco2 , Pc , Sucla2
diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtypeIdh1 , Sdha
dilated cardiomyopathyCs , Sdha
dilated cardiomyopathy 1GGSdha
dilated cardiomyopathy 1IIDlat , Sdhd
Disease ProgressionIdh3b , Pdha1 , Suclg1
dopamine transporter deficiency syndromeSdha
duodenal ulcerAco1
dyskeratosis congenitaIdh3g
Emery-Dreifuss muscular dystrophyIdh3g
endometriosisSuclg2
EndotoxemiaCs , Pck1 , Pck2
epilepsyPdha1 , Pdhb
esophagus squamous cell carcinomaIdh2
Experimental Diabetes MellitusCs , Pc , Pck1
Experimental Liver CirrhosisMdh1 , Pc , Pck1 , Pck2
Experimental Mammary NeoplasmsMdh2
factor VIII deficiencyIdh3g
favismIdh3g
Fetal Growth RetardationPck1
Fraser syndrome 3Mpc1
fumarase deficiencyFh
fundus dystrophyAco2 , Idh3a , Idh3b
gastrointestinal stromal tumorFh , Sdha , Sdhb , Sdhc
genetic diseaseAco2 , Dlat , Dld , Fh , Idh2 , Mdh2 , Ogdhl , Pc , Pck1 , Pdha1 , Pdhb , Sucla2 , Suclg1
glioblastomaIdh1
Glomus Jugulare TumorSdhd
glycogen storage disease IXAPdha1
Growth DisordersIdh2
hemangiomaIdh1 , Idh2
hepatoblastomaFh
hepatocellular carcinomaAcly , Aco2 , Fh , Idh1 , Idh2 , Pck1
hepatocellular clear cell carcinomaIdh1 , Idh2
Hereditary Leiomyomatosis and Renal Cell CancerFh
Hereditary Neoplastic SyndromesFh , Sdha , Sdhb , Sdhc , Sdhd
Hereditary Paraganglioma-Pheochromocytoma SyndromesSdha , Sdhb , Sdhc , Sdhd
hereditary renal cell carcinomaSdha
high grade gliomaIdh1
Huntington's disease-like 1Idh3b
hydronephrosisIdh2
hyperglycemiaPck1
hypertensionMdh1
hyperthyroidismCs
hypertrophic cardiomyopathyCs , Idh2
hypothyroidismCs
HypoxiaCs , Pck2
Immunoblastic LymphadenopathyIdh2
immunodeficiency 33Idh3g
infantile cerebellar-retinal degenerationAco2
Infantile PolymyoclonusSdha
intellectual disabilityDlat , Dlst , Pc , Sdhd , Sucla2
interstitial lung disease 2Sdha
Intestinal Carcinoid TumorsSdhd
intrahepatic cholangiocarcinomaIdh1 , Idh2
Islet Cell Tumor SyndromeSdhb , Sdhc , Sdhd
kidney failureIdh2
Kidney NeoplasmsSdhb
lactic acidosisPdha1
Leigh diseaseDld , Sdha , Sdhc
Leigh Necrotizing Encephalopathy due to Pyruvate Carboxylase DeficiencyPc
Leigh Syndrome, X-LinkedPdha1
leiomyoma cutisFh
limb ischemiaCs
lissencephaly 5Dld
Liver InjuryMdh1
Liver Reperfusion InjuryCs , Idh2
lung adenocarcinomaIdh1 , Idh2 , Suclg2
lung cancerIdh2
lung non-small cell carcinomaFh , Idh2 , Sdha , Sdhc
lung squamous cell carcinomaIdh1 , Idh2
lymphomaIdh1
lysinuric protein intolerancePck2
Maffucci syndromeIdh1
maple syrup urine diseaseDld
megacolonOgdhl
Megalencephaly-Polymicrogyria-Polydactyly-Hydrocephalus Syndrome 2Fh
melanomaSdhd
metabolic dysfunction-associated steatohepatitisIdh2 , Pck1
metabolic dysfunction-associated steatotic liver diseaseAco1 , Idh2
Metabolic SyndromeIdh2 , Pck1
methylmalonic acidemiaSuclg2
microcephalyFh , Sdhd
mitochondrial complex II deficiencySdha , Sdhb , Sdhd
Mitochondrial Complex II Deficiency Nuclear Type 1Sdha , Sdhb , Sdhd
Mitochondrial Complex II Deficiency Nuclear Type 3Sdhd
Mitochondrial Complex II Deficiency Nuclear Type 4Sdhb
mitochondrial DNA depletion syndromeSucla2 , Suclg1
mitochondrial DNA depletion syndrome 5Sucla2
mitochondrial DNA depletion syndrome 9Suclg1 , Suclg2
mitochondrial metabolism diseaseAco2 , Pdha1
mitochondrial pyruvate carrier deficiencyMpc1
multiple endocrine neoplasia type 2ASdha
muscular diseaseSdha
myelodysplastic syndromeIdh1 , Idh2
myocardial infarctionCs , Mdh2
Myocardial IschemiaDlat , Pdha1
myopathySdha
Nance-Horan syndromePdha1
Neoplasm InvasivenessAco1
Neoplasm Recurrence, LocalIdh1 , Idh2
neuroblastomaSdhc
Neurodegeneration with Ataxia and Late-Onset Optic AtrophySdha
Neurodevelopmental DisordersFh , Idh1 , Pdha1
Nijmegen breakage syndromeSdhb
obesityAcly , Cs , Idh1 , Pck1 , Sucla2 , Suclg2
oligodendrogliomaIdh1 , Idh2
Ollier diseaseIdh1 , Idh2
Opsoclonus-Myoclonus SyndromeSdha
optic atrophyAco2 , Idh3b
optic atrophy 9Aco2
osteoarthritisAco1 , Idh2 , Idh3a , Pdha1 , Sdha
osteochondrodysplasiaIdh1 , Idh2
osteoporosisIdh2
ovarian cancerFh , Sdhb , Sdhc
ovarian carcinomaMdh2
oxoglutarate dehydrogenase deficiencyDlst , Ogdh
pantothenate kinase-associated neurodegenerationIdh3b
paragangliomaSdha , Sdhb , Sdhc , Sdhd
Paragangliomas 1Sdha , Sdhd
Paragangliomas 3Sdhb , Sdhc , Sdhd
Paragangliomas 4Sdha , Sdhb , Sdhd
Paragangliomas 5Sdha
Paragangliomas 7Dlst
Paragangliomas with Sensorineural Hearing LossSdhd
paraplegiaIdh3g
parathyroid carcinomaFh , Sdhc
Parkinson's diseaseSdha
paroxysmal extreme pain disorderIdh1
peripheral T-cell lymphomaIdh2
Peritoneal AdhesionsSdha
periventricular nodular heterotopiaIdh3g
pheochromocytomaSdha , Sdhb , Sdhc , Sdhd
Phosphoenolpyruvate Carboxykinase DeficiencyPck1
Phosphoenolpyruvate Carboxykinase Deficiency, CytosolicPck1
Phosphoenolpyruvate Carboxykinase Deficiency, MitochondrialPck2
pilocytic astrocytomaSdha
pleomorphic xanthoastrocytomaDld , Mdh2 , Ogdh
primary pulmonary hypertensionIdh1
prostate adenocarcinomaIdh1
prostate cancerDlat
Prostatic NeoplasmsIdh1 , Pdha1
Pulmonary AtresiaSdha
pyruvate carboxylase deficiency diseasePc
pyruvate decarboxylase deficiencyDld , Pdha1 , Pdhb
Pyruvate Dehydrogenase E1 Alpha DeficiencyPdha1
Pyruvate Dehydrogenase E1-Beta DeficiencyPdhb
Pyruvate Dehydrogenase E2 DeficiencyDlat , Sdhd
Pyruvate Dehydrogenase Phosphatase DeficiencyPdhb
Pyruvate Metabolism, Inborn ErrorsPdha1
quadriplegiaIdh3g
Recurrent Infections, with Encephalopathy, Hepatic Dysfunction, and Cardiovascular MalformationsPc
renal cell carcinomaFh , Pdhb , Sdhb , Sdhd
Renal Cell Carcinoma 1Sdhb
retinitis pigmentosaIdh3a , Idh3b
retinitis pigmentosa 27Pck2
retinitis pigmentosa 46Idh3b
retinitis pigmentosa 90Idh3a
retinoblastomaSucla2
rhabdomyosarcomaSdha , Sdhc
Rubinstein-Taybi syndromeAco2
schizophreniaOgdhl
SepsisCs
severe congenital encephalopathy due to MECP2 mutationIdh3g
skin melanomaIdh1
Sleep DeprivationDlat
Specific Granule DeficiencyPck2
spermatogenic failure 1Pdha2
spermatogenic failure 70Pdha2
spinocerebellar ataxia 45Fh
SplenomegalyIdh3g
status epilepticusIdh2
stomach cancerIdh2
Stomach NeoplasmsIdh3b , Pdha1 , Suclg1
Subacute Necrotizing Encephalopathy of Leigh, InfantileDld , Sdha
substance-related disorderMpc1
sudden infant death syndromePdha1
syndromic X-linked intellectual disability Lubs typeIdh3g , Pdha1
teratomaIdh2
type 2 diabetes mellitusPc , Pck1 , Sdhb
uterine fibroidFh
Vascular MalformationsIdh2
Ventricular Dysfunction, LeftCs , Idh2
von Hippel-Lindau diseaseSdhb
Yoon-Bellen neurodevelopmental syndromeOgdhl
Pathway Annotations Associated with Genes in the citric acid cycle pathway
Pathway TermsGene Symbols
2-aminoadipic 2-oxoadipic aciduria pathwayDld , Dlst
3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathwayDld
3-hydroxyisobutyric aciduria pathwayDld
3-methylcrotonyl CoA carboxylase 1 deficiency pathwayDld
3-methylglutaconic aciduria type 1 pathwayDld
3-methylglutaconic aciduria type 3 pathwayDld
alanine metabolic pathwayPc
altered citric acid cycle pathwayFh , Sdhb , Sdhd
Alzheimer's disease pathwaySdha , Sdhb , Sdhc , Sdhd
butanoate metabolic pathwayPdha1 , Pdha2 , Pdhb
citric acid cycle pathwayAcly , Aco1 , Aco2 , Cs , Dlat , Dld , Dlst , Fh , Idh1 , Idh2 , Idh3a , Idh3b , Idh3g , Mdh1 , Mdh2 , Mpc1 , Ogdh , Ogdhl , Pc , Pck1 , Pck2 , Pdha1 , Pdha2 , Pdhb , Sdha , Sdhb , Sdhc , Sdhd , Sucla2 , Suclg1 , Suclg2
dihydropyrimidine dehydrogenase deficiency pathwayDld
dimethylglycine dehydrogenase deficiency pathwayDld
doxorubicin response pathwayAco1
eicosanoid signaling pathway via peroxisome proliferator-activated receptor gammaPck1 , Pck2
electron transport chain pathwaySdha , Sdhb , Sdhc , Sdhd
fatty acid biosynthetic pathwayAcly
folate cycle metabolic pathwayDld
forkhead class A signaling pathwayPck1
fructose-1,6-bisphosphatase deficiency pathwayMdh2 , Mpc1 , Pc , Pck1
fumaric aciduria pathwayAco2 , Cs , Dlat , Dld , Dlst , Fh , Idh3a , Idh3b , Idh3g , Mdh2 , Mpc1 , Ogdh , Pc , Pdha1 , Pdhb , Sdha , Sdhb , Sdhc , Sdhd , Suclg1 , Suclg2
gluconeogenesis pathwayDlat , Dld , Mdh2 , Mpc1 , Pc , Pck1 , Pck2 , Pdha1 , Pdha2 , Pdhb
glutaric aciduria type I pathwayDld , Dlst
glutathione metabolic pathwayIdh1 , Idh2
glycine, serine and threonine metabolic pathwayDld
glycogen storage disease type Ia pathwayMdh2 , Mpc1 , Pc , Pck1
glycogen storage disease type Ib pathwayMdh2 , Mpc1 , Pc , Pck1
glycolysis pathwayDlat , Dld , Pck1 , Pck2 , Pdha1 , Pdha2 , Pdhb
glycolysis/gluconeogenesis pathwayDlat , Dld , Pck1 , Pck2 , Pdha1 , Pdha2 , Pdhb
glyoxylate and dicarboxylate metabolic pathwayAco1 , Aco2 , Cs , Mdh1 , Mdh2
Huntington's disease pathwaySdha , Sdhb , Sdhc , Sdhd
hyperlysinemia pathwayDld , Dlst
insulin signaling pathwayPck1 , Pck2
iron homeostasis pathwayAco1
isobutyryl-CoA dehydrogenase deficiency pathwayDld
isovaleric acidemia pathwayDld
lactic acidosis pathwayPc
Leigh disease pathwayDlat , Dld , Mdh1 , Pc , Pck1 , Pdha1 , Pdhb
lysine degradation pathwayDld , Dlst , Ogdh , Ogdhl
malonic aciduria pathwayDld
maple syrup urine disease pathwayDld
methylmalonate semialdehyde dehydrogenase deficiency pathwayDld
methylmalonic acidemia pathwayDld
methylmalonic aciduria, cobalamin-related pathwayDld
mitochondrial complex II deficiency pathwayAco2 , Cs , Dlat , Dld , Dlst , Fh , Idh3a , Idh3b , Idh3g , Mdh2 , Mpc1 , Ogdh , Pc , Pdha1 , Pdhb , Sdha , Sdhb , Sdhc , Sdhd , Suclg1 , Suclg2
nicotinamide adenine dinucleotide metabolic pathwayMdh1 , Mdh2
nonketotic hyperglycinemia pathwayDld
oxidative phosphorylation pathwaySdha , Sdhb , Sdhc , Sdhd
Parkinson's disease pathwaySdha , Sdhb , Sdhc , Sdhd
phosphoenolpyruvate carboxykinase deficiency pathwayMdh2 , Mpc1 , Pc , Pck1
primary hyperoxaluria type 1 pathwayPc
primary hyperoxaluria type 2 pathwayDlat , Dld , Mdh1 , Pc , Pck1 , Pdha1 , Pdhb
propanoate metabolic pathwayDld , Sucla2 , Suclg1 , Suclg2
propionic acidemia pathwayDld
pyruvate carboxylase deficiency pathwayPc
pyruvate decarboxylase deficiency pathwayDlat , Dld , Mdh1 , Pc , Pck1 , Pdha1 , Pdhb
pyruvate dehydrogenase E1 deficiency pathwayDlat , Dld , Mdh1 , Pc , Pck1 , Pdha1 , Pdhb
pyruvate dehydrogenase E2 deficiency pathwayAco2 , Cs , Dlat , Dld , Dlst , Fh , Idh3a , Idh3b , Idh3g , Mdh2 , Mpc1 , Ogdh , Pc , Pdha1 , Pdhb , Sdha , Sdhb , Sdhc , Sdhd , Suclg1 , Suclg2
pyruvate dehydrogenase E3 deficiency pathwayAco2 , Cs , Dlat , Dld , Dlst , Fh , Idh3a , Idh3b , Idh3g , Mdh2 , Mpc1 , Ogdh , Pc , Pdha1 , Pdhb , Sdha , Sdhb , Sdhc , Sdhd , Suclg1 , Suclg2
pyruvate kinase deficiency of red cells pathwayDlat , Dld , Mdh1 , Pc , Pck1 , Pdha1 , Pdhb
pyruvate metabolic pathwayDlat , Dld , Mdh1 , Mdh2 , Pc , Pck1 , Pck2 , Pdha1 , Pdha2 , Pdhb
renal cell carcinoma pathwayFh , Sdhb , Sdhd
saccharopinuria pathwayDld , Dlst
sarcosinemia pathwayDld
triosephosphate isomerase deficiency pathwayMdh2 , Mpc1 , Pc , Pck1
tryptophan metabolic pathwayOgdh , Ogdhl
valine, leucine and isoleucine biosynthetic pathwayPdha1 , Pdha2 , Pdhb
valine, leucine and isoleucine degradation pathwayDld
Phenotype Annotations Associated with Genes in the citric acid cycle pathway

References Associated with the citric acid cycle pathway:

Ontology Path Diagram:

paths to the root
paths to the root

Import into Pathway Studio: