GLUCONEOGENESIS PATHWAY (PW:0000641)

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Description

Glucose is a major source of energy in the cell. If the liver has been depleted of its stores of glycogen or glucose is not available from dietary sources, glucose synthesis occurs via the gluconeogenesis pathway. Gluconeogenic precursors include the glycolytic pyruvate or lactate, citric acid cycle intermediates and the carbon skeletons of amino acids. Whatever the precursor, it first has to be converted to oxaloacetate (a four-carbon compound and a citric acid cycle intermediate). From pyruvat

Pathway Diagram:

Ariadne Genomics Inc. enolases Pgk1 aldolases Pfkfb members (kinase) Pfkfb members (phosphatase) 2-phosphoglycerate 3-phosphoglycerate Tpi1 Gapdh Pgam2 G6pc Gpi Fbp1 Fbp2 Pck1 Pc G6pc3 phosphoenolpyruvate dihydroxyacetone-phosphate glyceraldehyde-3-phosphate 1,3-biphosphoglycerate Pck2 oxaloacetate oxaloacetate ---> phosphoenolpyruvate glycolysis pathway glyceraldehyde-3-phosphate <--> 1,3-biphosphoglycerate fructose-1,6-diphosphate ---> fructose-6-phosphate 2-phosphoglycerate <--> phosphoenolpyruvate glyceraldehyde-3-phosphate <--> dihydroxyacetone-phosphate glucose-6-phosphate ---> glucose glucose glucose ---> glycolysis pathway fructose-1,6-diphosphate <--> dihydroxyacetone-phosphate fructose-1,6-diphosphate <--> glyceraldehyde-3-phosphate glucose-6-phosphate <--> fructose-6-phosphate 3-phosphoglycerate <--> 2-phosphoglycerate fructose-6-phosphate pyruvate ---> oxaloacetate 3-phosphoglycerate <--> 1,3-biphosphoglycerate glucose-6-phosphate fructose-1,6-diphosphate pyruvate Pfkfb members (phosphatase) ---| F2,6P Pfkfb members (kinase) --+> F2,6P F2,6P F2,6P ---| Fbp2 F2,6P ---| Fbp1
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Genes in Pathway:

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gluconeogenesis pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Acss1 acyl-CoA synthetase short-chain family member 1 JBrowse link 3 146,420,346 146,470,293 RGD:6907045
G Acss2 acyl-CoA synthetase short-chain family member 2 JBrowse link 3 151,032,925 151,075,856 RGD:6907045
G Adh4 alcohol dehydrogenase 4 (class II), pi polypeptide JBrowse link 2 243,702,035 243,720,063 RGD:6907045
G Adh5 alcohol dehydrogenase 5 (class III), chi polypeptide JBrowse link 2 243,728,500 243,740,907 RGD:6907045
G Adh6 alcohol dehydrogenase 6 (class V) JBrowse link 2 243,656,568 243,687,857 RGD:6907045
G Adh7 alcohol dehydrogenase 7 (class IV), mu or sigma polypeptide JBrowse link 2 243,500,540 243,516,865 RGD:6907045
G Adpgk ADP-dependent glucokinase JBrowse link 8 64,088,871 64,116,832 RGD:6907045
G Akr1a1 aldo-keto reductase family 1 member A1 JBrowse link 5 135,482,068 135,498,693 RGD:6907045
G Aldh1a1 aldehyde dehydrogenase 1 family, member A1 JBrowse link 1 238,222,689 238,264,381 RGD:6907045
G Aldh1a3 aldehyde dehydrogenase 1 family, member A3 JBrowse link 1 127,302,920 127,337,828 RGD:6907045
G Aldh1a7 aldehyde dehydrogenase family 1, subfamily A7 JBrowse link 1 240,584,233 240,601,843 RGD:6907045
G Aldh1b1 aldehyde dehydrogenase 1 family, member B1 JBrowse link 5 61,382,351 61,387,359 RGD:6907045
G Aldh2 aldehyde dehydrogenase 2 family (mitochondrial) JBrowse link 12 40,466,418 40,498,813 RGD:6907045
G Aldh3a1 aldehyde dehydrogenase 3 family, member A1 JBrowse link 10 47,490,168 47,499,855 RGD:6907045
G Aldh3a2 aldehyde dehydrogenase 3 family, member A2 JBrowse link 10 47,525,486 47,546,535 RGD:6907045
G Aldh3b1 aldehyde dehydrogenase 3 family, member B1 JBrowse link 1 219,145,457 219,181,536 RGD:6907045
G Aldh7a1 aldehyde dehydrogenase 7 family, member A1 JBrowse link 18 51,619,007 51,651,267 RGD:6907045
G Aldh9a1 aldehyde dehydrogenase 9 family, member A1 JBrowse link 13 85,580,828 85,597,497 RGD:6907045
G Aldoa aldolase, fructose-bisphosphate A JBrowse link 1 198,228,387 198,233,988 RGD:2302796
RGD:6907045
RGD:10402751
G Aldob aldolase, fructose-bisphosphate B JBrowse link 5 64,805,772 64,818,813 RGD:2302796
RGD:6907045
G Aldoc aldolase, fructose-bisphosphate C JBrowse link 10 65,586,504 65,590,093 RGD:6907045
G Bpgm bisphosphoglycerate mutase JBrowse link 4 61,912,210 61,940,697 RGD:6907045
RGD:10402751
G Dlat dihydrolipoamide S-acetyltransferase JBrowse link 8 55,062,549 55,087,832 RGD:6907045
G Dld dihydrolipoamide dehydrogenase JBrowse link 6 50,597,677 50,618,694 RGD:6907045
G Eno1 enolase 1 JBrowse link 5 167,288,223 167,299,610 RGD:2302802
RGD:6907045
RGD:10402751
G Eno2 enolase 2 JBrowse link 4 157,285,192 157,294,090 RGD:2302795
RGD:6907045
G Eno3 enolase 3 JBrowse link 10 57,278,271 57,283,661 RGD:6907045
G Fbp1 fructose-bisphosphatase 1 JBrowse link 17 396,175 417,480 RGD:2302851
RGD:2302970
RGD:6907045
RGD:10402751
G Fbp2 fructose-bisphosphatase 2 JBrowse link 17 372,554 389,918 RGD:2302851
RGD:2302970
RGD:6907045
G G6pc glucose-6-phosphatase, catalytic subunit JBrowse link 10 89,286,009 89,296,213 RGD:2302850
RGD:2302851
RGD:2302970
RGD:6907045
RGD:10402751
G G6pc3 glucose 6 phosphatase catalytic subunit 3 JBrowse link 10 90,134,193 90,138,425 RGD:2302850
RGD:2302851
RGD:2302970
G Galm galactose mutarotase JBrowse link 6 2,808,988 2,860,742 RGD:6907045
RGD:10402751
G Gapdh glyceraldehyde-3-phosphate dehydrogenase JBrowse link 4 157,676,336 157,680,322 RGD:2302795
RGD:6907045
RGD:10402751
G Gapdhs glyceraldehyde-3-phosphate dehydrogenase, spermatogenic JBrowse link 1 89,180,063 89,195,347 RGD:6907045
G Gck glucokinase JBrowse link 14 86,149,146 86,191,589 RGD:6907045
G Got1 glutamic-oxaloacetic transaminase 1 JBrowse link 1 263,246,248 263,269,762 RGD:2302802
G Got2 glutamic-oxaloacetic transaminase 2 JBrowse link 19 9,587,637 9,613,323 RGD:2302802
G Gpi glucose-6-phosphate isomerase JBrowse link 1 90,063,411 90,091,287 RGD:6907045
RGD:10402751
G Hk1 hexokinase 1 JBrowse link 20 31,911,460 31,979,780 RGD:6907045
G Hk2 hexokinase 2 JBrowse link 4 113,559,396 113,609,897 RGD:6907045
RGD:10402751
G Hk3 hexokinase 3 JBrowse link 17 10,134,726 10,152,976 RGD:6907045
G Ldha lactate dehydrogenase A JBrowse link 1 102,900,288 102,909,713 RGD:6907045
G Ldhal6b lactate dehydrogenase A-like 6B JBrowse link 1 46,307,853 46,309,276 RGD:6907045
G Ldhb lactate dehydrogenase B JBrowse link 4 176,701,980 176,719,999 RGD:6907045
G Ldhc lactate dehydrogenase C JBrowse link 1 102,914,875 102,931,843 RGD:6907045
G Mdh2 malate dehydrogenase 2 JBrowse link 12 23,941,451 23,954,406 RGD:10402751
G Mpc1 mitochondrial pyruvate carrier 1 JBrowse link 1 53,026,608 53,038,229 RGD:10402751
G Pank1 pantothenate kinase 1 JBrowse link 1 253,115,400 253,189,643 RGD:10402751
G Pc pyruvate carboxylase JBrowse link 1 219,759,157 219,859,854 RGD:2302809
RGD:2302851
RGD:2302971
RGD:2302972
RGD:10402751
G Pck1 phosphoenolpyruvate carboxykinase 1 JBrowse link 3 171,213,936 171,219,885 RGD:2302802
RGD:2302809
RGD:2302850
RGD:2302851
RGD:2302970
RGD:2302971
RGD:6907045
RGD:10402751
G Pck2 phosphoenolpyruvate carboxykinase 2 (mitochondrial) JBrowse link 15 34,216,735 34,224,357 RGD:2302802
RGD:2302809
RGD:2302851
RGD:2302970
RGD:2302971
RGD:6907045
G Pdha1 pyruvate dehydrogenase (lipoamide) alpha 1 JBrowse link X 37,329,779 37,343,410 RGD:6907045
G Pdha2 pyruvate dehydrogenase (lipoamide) alpha 2 JBrowse link 2 246,736,449 246,737,997 RGD:6907045
G Pdhb pyruvate dehydrogenase (lipoamide) beta JBrowse link 15 18,540,826 18,546,855 RGD:6907045
G Pfkfb1 6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 1 JBrowse link X 23,092,143 23,145,923 RGD:2302793
G Pfkl phosphofructokinase, liver type JBrowse link 20 11,393,860 11,415,889 RGD:6907045
G Pfkm phosphofructokinase, muscle JBrowse link 7 139,702,066 139,722,132 RGD:6907045
G Pfkp phosphofructokinase, platelet JBrowse link 17 68,510,765 68,574,387 RGD:6907045
G Pgam1 phosphoglycerate mutase 1 JBrowse link 1 261,158,204 261,165,814 RGD:6907045
RGD:10402751
G Pgam2 phosphoglycerate mutase 2 JBrowse link 14 86,045,005 86,047,116 RGD:2302794
RGD:2302795
RGD:6907045
RGD:10402751
G Pgk1 phosphoglycerate kinase 1 JBrowse link X 77,263,399 77,279,373 RGD:2302795
RGD:2302860
RGD:6907045
G Pgk2 phosphoglycerate kinase 2 JBrowse link 9 24,095,774 24,097,340 RGD:6907045
G Pgm1 phosphoglucomutase 1 JBrowse link 5 118,743,632 118,803,055 RGD:6907045
RGD:10402751
G Pklr pyruvate kinase, liver and RBC JBrowse link 2 188,449,158 188,458,034 RGD:6907045
G RGD1560402 similar to Phosphoglycerate kinase 1 JBrowse link 2 42,726,747 42,728,100 RGD:6907045
G RGD1560797 similar to glyceraldehyde-3-phosphate dehydrogenase JBrowse link 15 85,087,422 85,112,505 RGD:6907045
G RGD1560826 similar to glyceraldehyde-3-phosphate dehydrogenase JBrowse link 2 226,825,623 226,826,642 RGD:6907045
G RGD1562690 similar to L-lactate dehydrogenase A chain (LDH-A) (LDH muscle subunit) (LDH-M) JBrowse link 8 33,075,109 33,076,725 RGD:6907045
G RGD1562758 similar to glyceraldehyde-3-phosphate dehydrogenase JBrowse link 18 63,824,239 63,826,908 RGD:6907045
G RGD1563601 similar to Tpi1 protein JBrowse link 17 27,764,761 27,765,800 RGD:6907045
G RGD1564958 similar to glyceraldehyde-3-phosphate dehydrogenase (phosphorylating) (EC 1.2.1.12) JBrowse link 16 16,948,541 16,950,239 RGD:6907045
G Slc25a11 solute carrier family 25 member 11 JBrowse link 10 57,265,903 57,268,018 RGD:10402751
G Slc2a2 solute carrier family 2 member 2 JBrowse link 2 114,413,427 114,445,418 RGD:10402751
G Slc37a4 solute carrier family 37 member 4 JBrowse link 8 48,716,914 48,723,024 RGD:10402751
G Tpi1 triosephosphate isomerase 1 JBrowse link 4 157,328,375 157,331,905 RGD:6907045
RGD:10402751

Additional Elements in Pathway:

(includes Gene Groups, Small Molecules, Other Pathways..etc.)
Object TypePathway ObjectPathway Object Description
Functional ClassPfkfb members (phosphatase)Members of the bifunctional enzyme that catalyzes the synthesis and degradation of F2,6P
Functional ClassPfkfb members (kinase)Members of the bifunctional enzyme that catalyzes the synthesis and degradation of F2,6P
Functional ClassaldolasesThe aldolase isozymes
Functional ClassenolasesThe enolase isozymes

Pathway Gene Annotations

Disease Annotations Associated with Genes in the gluconeogenesis pathway
Disease TermsGene Symbols
Abnormalities, MultipleDlat , Pdha1
Acidosis, LacticPdha1
Acute Coronary SyndromeEno1 , Gapdh , Ldhb
AdenocarcinomaEno1 , Pgam1
Adenocarcinoma of LungAldoa , Eno1
Adenosine Triphosphate, Elevated, of ErythrocytesPklr
Alcohol Sensitivity, AcuteAldh2
Alcohol-Induced DisordersAldh2
AlcoholismAdh5 , Aldh2
Alzheimer DiseaseAldh2 , Eno1 , Gapdh , Gapdhs , Pck1 , Tpi1
AnemiaHk1
Anemia, HemolyticGpi , Pgk1 , Tpi1
Anemia, Hemolytic, CongenitalGpi
Anemia, Hemolytic, Congenital NonspherocyticGpi , Hk1 , Pklr , Tpi1
Anemia, Sickle CellHk1
AnoxiaAldob , Gapdh , Pck2
Aortic Valve StenosisEno3
ArteriosclerosisAldh3a1
Arthritis, ExperimentalGpi
Arthritis, RheumatoidEno1
AsthmaAdh5 , Aldh2
AtrophyEno2
Autism Spectrum DisorderAldoa
Autistic DisorderAldh1a3
Autoimmune Diseases of the Nervous SystemAldoc
Bisphosphoglycerate Mutase DeficiencyBpgm
Bone DiseasesAldh7a1
Borna DiseaseAldoc
Brain DiseasesEno2
Brain InfarctionAldh2
Brain IschemiaMdh2
Breast NeoplasmsAldoa , Eno1 , Eno2 , Gpi , Ldhal6b , Ldhb , Slc2a2
Burkitt LymphomaPc
BurnsPklr
Carbohydrate Metabolism, Inborn ErrorsTpi1
Carcinoid TumorEno2
CarcinomaEno1 , Mdh2 , Pgam1
Carcinoma, EndometrioidEno2
Carcinoma, HepatocellularAldoc , Eno1 , Fbp1 , Gapdh , Pck1 , Pgk1 , Slc2a2
Carcinoma, Intraductal, NoninfiltratingLdhb
Carcinoma, NeuroendocrineEno2
Carcinoma, Non-Small-Cell LungEno1 , Eno2 , Pgam1
Carcinoma, Pancreatic DuctalSlc2a2
Carcinoma, Renal CellAldh1a1 , Ldhb , Pdhb , Pgk1
Carcinoma, Squamous CellEno1 , Gapdh , Pgam1 , Tpi1
CardiomyopathiesLdha
CataractAldh3a1
Cell Transformation, NeoplasticEno2
CholestasisAdh5 , Aldh1b1
Chronic Intermittent HypoxiaHk1 , Hk2
ColobomaAldh7a1
Colonic NeoplasmsAldh1b1 , Fbp1
Colorectal NeoplasmsAldh2 , Hk1
Congenital Disorders of GlycosylationPgm1
Congenital HyperinsulinismGck
Congenital Thrombotic Disease, due to Protein C DeficiencyPc
Corneal InjuriesAldh3a1
Coronary Artery DiseaseGck
Creutzfeldt-Jakob SyndromeAldh1a1
CystadenomaAldoa
Dermatitis, OccupationalAldh2
Diabetes ComplicationsAldh2
Diabetes MellitusAldob , Gck
Diabetes Mellitus, ExperimentalAldob , Eno2 , G6pc , Gck , Hk1 , Pc , Pck1 , Pfkm , Pklr , Slc2a2 , Slc37a4
Diabetes Mellitus, Permanent NeonatalGck
Diabetes Mellitus, Type 1Aldh2
Diabetes Mellitus, Type 2Aldh2 , Aldob , G6pc , Gck , Hk1 , Hk2 , Pc , Pck1 , Slc2a2
Diabetes, GestationalG6pc , Gck
Diabetic NeuropathiesAldh2
Diabetic RetinopathyGapdh
Dimauro DiseasePgam2
Disease ProgressionAldh7a1 , Pdha1
Drug-Induced Liver InjuryAldh2 , Aldob , Eno1 , Got1
Dursun SyndromeG6pc3
Endometrial NeoplasmsPgm1
EndotoxemiaAldoa , Pck1 , Pck2
Enterocolitis, NecrotizingAkr1a1 , Aldh2 , Eno1
Epilepsy, GeneralizedPgk1
Esophageal NeoplasmsAdh7 , Aldh2 , Eno1 , Gapdh
Esophageal Squamous Cell CarcinomaAldh2 , Tpi1
Fanconi SyndromeLdha , Slc2a2
Fatty LiverGck , Slc2a2
Fatty Liver, AlcoholicAldh2
Fetal Growth RetardationG6pc , Pck1 , Slc2a2
FlushingAldh2
Fructose IntoleranceAldob
Fructose-1,6-Diphosphatase DeficiencyFbp1
GastrinomaSlc2a2
Gaucher DiseasePklr
Genetic Diseases, InbornDlat , Pdha1
Genetic Predisposition to DiseaseAldh2
GlucagonomaSlc2a2
Glycogen Storage DiseaseSlc2a2
Glycogen Storage Disease IBG6pc , Slc37a4
Glycogen Storage Disease ICSlc37a4
Glycogen Storage Disease Type IG6pc , Slc37a4
Glycogen Storage Disease Type VIIPfkm
Glycogen Storage Disease XIIAldoa
Glycogen Storage Disease XIIIEno3
Glycogen Storage Disease XIVPgm1
Heart DiseasesAldh2
Heart FailureMdh2 , Pck1
Heart InjuriesAldh2
Hemolytic Anemia, Nonspherocytic, due to Glucose Phosphate Isomerase DeficiencyGpi
Hemolytic Anemia, Nonspherocytic, due to Hexokinase DeficiencyHk1
Hepatitis, AutoimmuneAldob
HepatomegalySlc37a4
Hepatorenal Form of Glycogen Storage DiseaseG6pc
HyperglycemiaGck , Ldha , Pck1 , Pklr
Hyperinsulinemic Hypoglycemia, Familial, 3Gck
HyperinsulinismGck , Pfkfb1 , Pklr
HypertensionAldh2 , Eno1 , Eno2 , Gck
Hypertension, PulmonaryAdh5 , Hk1
HypoglycemiaGck
Infertility, MaleAldh2 , Eno1 , Ldhc
Insulin ResistanceGck , Pklr
InsulinomaSlc2a2
Intellectual DisabilityGpi
Kidney NeoplasmsEno2
Kidney Reperfusion InjuryAldh2
Lactate Dehydrogenase B DeficiencyLdhb
Lactate Dehydrogenase Deficiency Type ALdha
Lactic Acidosis, Congenital Infantile, Due To LAD DeficiencyDld
Laryngeal NeoplasmsAdh7
Leigh DiseaseDld
Leigh Syndrome, X-LinkedPdha1
Leukemia, Myeloid, AcuteEno2 , Gpi
Liver Cirrhosis, ExperimentalAdh4 , Aldh1a1 , Aldh1b1 , Aldh2 , G6pc , Gck , Got1 , Ldhb , Pc , Pck1 , Pck2 , Pfkp , Pklr , Slc37a4
Liver DiseasesAldh2 , Eno3 , Hk3
Liver InjuryGck
Liver NeoplasmsAldob
Liver Neoplasms, ExperimentalEno2
Long QT SyndromeHk3
Lung DiseasesLdhc
MalariaPklr
Mammary Neoplasms, AnimalEno1 , Mdh2 , Pgam1
Mammary Neoplasms, ExperimentalEno1 , Mdh2 , Pgam1
Maple Syrup Urine DiseaseDld
Maturity-Onset Diabetes of the YoungGck
Maturity-Onset Diabetes of the Young, Type 2Gck
MelanomaPck1
Melanoma, Cutaneous Malignant, Susceptibility To, 1Pck1
Metabolism, Inborn ErrorsLdhb , Tpi1
Microphthalmia, Isolated 8Aldh1a3
Microphthalmia, Syndromic 1Pfkp
Migraine without AuraEno2
Mitochondrial Pyruvate Carrier DeficiencyMpc1
Mouth NeoplasmsAdh7 , Eno1 , Gapdh , Pgam1 , Tpi1
Multiple MyelomaHk1
Multiple TraumaEno2
Myocardial InfarctionAldh2 , Ldha , Mdh2
Myocardial IschemiaAldh2 , Aldoa , Dlat , Hk1 , Hk2 , Pdha1 , Pfkm , Pgm1
Myocardial Reperfusion InjuryAldh2
MyoglobinuriaLdha , Pgam2
NecrosisGapdh
Neoplasm InvasivenessAldoa , Eno1 , Ldhb
Neoplasm MetastasisAldoa , Gpi
Neoplasms, ExperimentalEno2 , Ldha
Neoplasms, Nerve TissueEno2
Neural Tube DefectsSlc2a2
Neuromuscular DiseasesGpi , Tpi1
Neuromuscular ManifestationsTpi1
Neuropathy, Hereditary Motor and Sensory, Russe TypeHk1
Neutropenia, Severe Congenital, Autosomal Recessive 4G6pc3
ObesityGck , Hk1 , Hk2 , Pck1 , Pklr
OsteoarthritisEno1 , Gapdh , Pdha1
OsteoporosisEno1 , Gapdh , Tpi1
Pancreatic NeoplasmsAldh1a1 , Aldh2
Parkinson DiseaseFbp1
Parkinsonian DisordersAldh1a1 , Eno2
PeritonitisAldob
Pharyngeal NeoplasmsAdh7
Phosphoenolpyruvate Carboxykinase 2 DeficiencyPck2
Phosphoenolpyruvate Carboxykinase DeficiencyPck1
Phosphoglycerate Kinase 1 DeficiencyPgk1
Pleomorphic Xanthoastrocytoma Bpgm , Dld , Gck , Mdh2 , Pgam2
Precancerous ConditionsAldh2
Precursor Cell Lymphoblastic Leukemia-LymphomaGpi , Hk1
Pregnancy in DiabeticsGapdh
Prostatic NeoplasmsAldh9a1 , Dlat , Eno2 , Gapdhs , Ldhb , Pdha1 , Pgam2
Pyridoxine-Dependent EpilepsyAldh7a1
Pyruvate Carboxylase Deficiency DiseasePc
Pyruvate Dehydrogenase Complex Deficiency DiseaseDlat , Dld , Pdha1 , Pdhb
Pyruvate Dehydrogenase E1 Alpha DeficiencyPdha1
Pyruvate Dehydrogenase E1-Beta DeficiencyPdhb
Pyruvate Dehydrogenase E2 DeficiencyDlat
Pyruvate Kinase Deficiency of Red CellsPklr
Pyruvate Metabolism, Inborn ErrorsPdha1
Reperfusion InjuryAldoa , Eno2 , Pklr
Respiratory Distress Syndrome, AdultSlc25a11
SchizophreniaAldh1a3 , Aldoa , Aldob
SeizuresAldh7a1 , Eno2 , Pdha1
Severe Congenital NeutropeniaG6pc3
Shock, SepticEno2
Sjogren-Larsson SyndromeAldh3a2
Sleep DeprivationDlat
Smith-Magenis SyndromeAldh3a1 , Aldh3a2 , Aldoc , G6pc , G6pc3
Spinal Cord InjuriesEno2
Spondylocostal Dysostosis 5Aldoa
Stomach NeoplasmsAldh1a3 , Aldh7a1 , Aldob , Eno1 , Fbp1 , Pdha1 , Pgam1
StrokeAldoc
Substance-Related DisordersMpc1
TeratomaPgm1
Thrombophilia, Hereditary, due to Protein C Deficiency, Autosomal RecessivePc
Triosephosphate Isomerase DeficiencyTpi1
Urinary Bladder NeoplasmsEno2 , Hk1
Urologic NeoplasmsEno2
Vascular DiseasesAldh2
Ventricular DysfunctionAldh2
Pathway Annotations Associated with Genes in the gluconeogenesis pathway
Pathway TermsGene Symbols
2-aminoadipic 2-oxoadipic aciduria disease pathwayAldh7a1 , Dld
2-hydroxyglutaricaciduria disease pathwayGot2
3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathwayAldh2 , Dld
3-hydroxyisobutyric aciduria pathwayAldh2 , Dld
3-methylcrotonyl CoA carboxylase 1 deficiency pathwayAldh2 , Dld
alanine metabolic pathwayPc
alanine, aspartate and glutamate metabolic pathwayGot1 , Got2
alkaptonuria disease pathwayAldh3a1 , Got1
altered galactose metabolic pathwayG6pc , Hk2
Alzheimer disease pathwayGapdh , RGD1560797 , RGD1560826 , RGD1562758 , RGD1564958
amino sugar metabolic pathwayGck , Gpi , Hk1 , Hk2 , Hk3 , Pgm1
arginine and proline metabolic pathwayAldh1a1 , Aldh1a7 , Aldh1b1 , Aldh2 , Aldh3a2 , Aldh7a1 , Aldh9a1 , Got1 , Got2
arginine:glycine amidinotransferase deficiency pathwayGot1
argininosuccinic aciduria disease pathwayGot2
ascorbate and aldarate metabolic pathwayAldh1a1 , Aldh1a7 , Aldh1b1 , Aldh2 , Aldh3a2 , Aldh7a1 , Aldh9a1
beta-alanine metabolic pathwayAldh1a1 , Aldh1a3 , Aldh1a7 , Aldh1b1 , Aldh2 , Aldh3a1 , Aldh3a2 , Aldh3b1 , Aldh7a1 , Aldh9a1
butanoate metabolic pathwayPdha1 , Pdha2 , Pdhb
butirosin and neomycin biosynthetic pathwayGck , Hk1 , Hk2 , Hk3
carbamoyl phosphate synthase I deficiency pathwayGot2
carnitine biosynthetic pathwayAldh9a1
carnosinemia disease pathwayAldh2
choline metabolic pathwayAldh7a1
citric acid cycle pathwayDlat , Dld , Mdh2 , Mpc1 , Pc , Pck1 , Pck2 , Pdha1 , Pdha2 , Pdhb
citrullinemia disease pathwayGot2
cyclophosphamide pharmacodynamics pathwayAldh1a1 , Aldh3a1
cyclophosphamide pharmacokinetics pathwayAldh1a1 , Aldh3a1
cysteine and methionine metabolic pathwayGot1 , Got2 , Ldha , Ldhal6b , Ldhb , Ldhc , RGD1562690
cysteine metabolic pathwayGot1
D-glycericacidemia disease pathwayAldh3a1
dihydropyrimidine dehydrogenase deficiency pathwayAldh2 , Dld
dimethylglycine dehydrogenase deficiency pathwayAldh2 , Dld
disulfiram pharmacodynamics pathwayAcss1 , Acss2 , Aldh1b1 , Aldh2 , Aldh3a1 , Got1
dopamine beta hydroxylase deficiency pathwayAldh3a1 , Got1
doxorubicin pharmacokinetics pathwayAkr1a1
eicosanoid signaling pathway via peroxisome proliferator-activated receptor gammaPck1 , Pck2
electron transport chain pathwayGapdh , Slc37a4
facilitative sugar transporter mediated glucose transport pathwaySlc2a2
fanconi syndrome pathwayAldoa , Bpgm , Eno1 , G6pc , Galm , Gapdh , Gpi , Hk2 , Pank1 , Pfkm , Pgam1 , Pgam2 , Pklr , Slc2a2 , Tpi1
fatty acid beta degradation pathwayAcss1 , Acss2
fatty acid metabolic pathwayAdh4 , Adh5 , Adh6 , Adh7 , Aldh1a1 , Aldh1a7 , Aldh1b1 , Aldh2 , Aldh3a2 , Aldh7a1 , Aldh9a1
felbamate pharmacokinetics pathwayAldh3a1
folate cycle metabolic pathwayDld
forkhead class A signaling pathwayAldob , G6pc , Gck , Pck1 , Pklr , Slc2a2
french type sialuria disease pathwayHk1
fructose and mannose metabolic pathwayAldoa , Aldob , Aldoc , Fbp1 , Fbp2 , Hk1 , Hk2 , Hk3 , Pfkfb1 , Pfkl , Pfkm , Pfkp , RGD1563601 , Tpi1
fructose intolerance pathwayAldoa , Aldob , Fbp1 , Hk1 , Pfkfb1 , Pfkl , Tpi1
fructose-1,6-diphosphatase deficiency pathwayAldoa , Bpgm , Eno1 , Fbp1 , G6pc , Galm , Gapdh , Gpi , Hk2 , Mdh2 , Mpc1 , Pank1 , Pc , Pck1 , Pgam1 , Pgam2 , Pgm1 , Slc25a11 , Slc2a2 , Slc37a4 , Tpi1
fructosuria disease pathwayAldoa , Aldob , Fbp1 , Hk1 , Pfkfb1 , Pfkl , Tpi1
fumaric aciduria disease pathwayDlat , Dld , Mdh2 , Mpc1 , Pc , Pdha1 , Pdhb
galactose metabolic pathwayG6pc , Gck , Hk1 , Hk2 , Hk3 , Pfkl , Pfkm , Pfkp , Pgm1
GALE deficiency pathwayGck , Pgm1
GALK deficiency pathwayGck , Pgm1
GALT deficiency pathwayG6pc , Gck , Hk1 , Pgm1
gamma aminobutyric acid transaminase deficiency pathwayAldh2
gliclazide pharmacodynamics pathwaySlc2a2
gluconeogenesis pathwayAcss1 , Acss2 , Adh4 , Adh5 , Adh6 , Adh7 , Adpgk , Akr1a1 , Aldh1a1 , Aldh1a3 , Aldh1a7 , Aldh1b1 , Aldh2 , Aldh3a1 , Aldh3a2 , Aldh3b1 , Aldh7a1 , Aldh9a1 , Aldoa , Aldob , Aldoc , Bpgm , Dlat , Dld , Eno1 , Eno2 , Eno3 , Fbp1 , Fbp2 , G6pc , G6pc3 , Galm , Gapdh , Gapdhs , Gck , Got1 , Got2 , Gpi , Hk1 , Hk2 , Hk3 , Ldha , Ldhal6b , Ldhb , Ldhc , Mdh2 , Mpc1 , Pank1 , Pc , Pck1 , Pck2 , Pdha1 , Pdha2 , Pdhb , Pfkfb1 , Pfkl , Pfkm , Pfkp , Pgam1 , Pgam2 , Pgk1 , Pgk2 , Pgm1 , Pklr , RGD1560402 , RGD1560797 , RGD1560826 , RGD1562690 , RGD1562758 , RGD1563601 , RGD1564958 , Slc25a11 , Slc2a2 , Slc37a4 , Tpi1
Glut1 deficiency syndrome pathwayG6pc
glutamic acid/glutamate metabolic pathwayGot2
glutaric aciduria type I disease pathwayAldh7a1 , Dld
glyburide pharmacodynamics pathwaySlc2a2
glycerolipid metabolic pathwayAkr1a1 , Aldh1a1 , Aldh1a7 , Aldh1b1 , Aldh2 , Aldh3a1 , Aldh3a2 , Aldh7a1 , Aldh9a1
glycine, serine and threonine metabolic pathwayAldh7a1 , Dld
glycogen biosynthetic pathwayGck , Pgm1
glycogen degradation pathwayG6pc , Pgm1
glycogen metabolic pathwayG6pc , Gck
glycogen storage disease pathway, type IAAldoa , Bpgm , Eno1 , Fbp1 , G6pc , Galm , Gapdh , Gpi , Hk2 , Mdh2 , Mpc1 , Pank1 , Pc , Pck1 , Pgam1 , Pgam2 , Pgm1 , Slc25a11 , Slc2a2 , Slc37a4 , Tpi1
glycogen storage disease pathway, type IBAldoa , Bpgm , Eno1 , Fbp1 , G6pc , Galm , Gapdh , Gpi , Hk2 , Mdh2 , Mpc1 , Pank1 , Pc , Pck1 , Pgam1 , Pgam2 , Pgm1 , Slc25a11 , Slc2a2 , Slc37a4 , Tpi1
glycogen storage disease pathway, type IIIGck , Gpi , Hk2 , Pgm1
glycogen storage disease pathway, type IV.Gck , Gpi , Hk2 , Pgm1
glycogen storage disease pathway, type VIGck , Gpi , Hk2 , Pgm1
glycogen storage disease pathway, type VIIAldoa , Bpgm , Eno1 , G6pc , Galm , Gapdh , Gpi , Hk2 , Pank1 , Pfkm , Pgam1 , Pgam2 , Pklr , Slc2a2 , Tpi1
glycolysis pathwayAcss1 , Acss2 , Adh4 , Adh5 , Adh6 , Adh7 , Adpgk , Akr1a1 , Aldh1a1 , Aldh1a3 , Aldh1a7 , Aldh1b1 , Aldh2 , Aldh3a1 , Aldh3a2 , Aldh3b1 , Aldh7a1 , Aldh9a1 , Aldoa , Aldob , Aldoc , Bpgm , Dlat , Dld , Eno1 , Eno2 , Eno3 , Fbp1 , Fbp2 , G6pc , Galm , Gapdh , Gapdhs , Gck , Gpi , Hk1 , Hk2 , Hk3 , Ldha , Ldhal6b , Ldhb , Ldhc , Pank1 , Pck1 , Pck2 , Pdha1 , Pdha2 , Pdhb , Pfkfb1 , Pfkl , Pfkm , Pfkp , Pgam1 , Pgam2 , Pgk1 , Pgk2 , Pgm1 , Pklr , RGD1560402 , RGD1560797 , RGD1560826 , RGD1562690 , RGD1562758 , RGD1563601 , RGD1564958 , Slc2a2 , Tpi1
glycolysis/gluconeogenesis pathwayAcss1 , Acss2 , Adh4 , Adh5 , Adh6 , Adh7 , Adpgk , Akr1a1 , Aldh1a1 , Aldh1a3 , Aldh1a7 , Aldh1b1 , Aldh2 , Aldh3a1 , Aldh3a2 , Aldh3b1 , Aldh7a1 , Aldh9a1 , Aldoa , Aldob , Aldoc , Bpgm , Dlat , Dld , Eno1 , Eno2 , Eno3 , Fbp1 , Fbp2 , G6pc , Galm , Gapdh , Gapdhs , Gck , Gpi , Hk1 , Hk2 , Hk3 , Ldha , Ldhal6b , Ldhb , Ldhc , Pck1 , Pck2 , Pdha1 , Pdha2 , Pdhb , Pfkl , Pfkm , Pfkp , Pgam1 , Pgam2 , Pgk1 , Pgk2 , Pgm1 , Pklr , RGD1560402 , RGD1560797 , RGD1560826 , RGD1562690 , RGD1562758 , RGD1563601 , RGD1564958 , Tpi1
glyoxylate and dicarboxylate metabolic pathwayMdh2
guanidinoacetate methyltransferase deficiency pathwayGot1
gyrate atrophy pathwayGot1
hawkinsinuria disease pathwayAldh3a1 , Got1
hexosamine biosynthetic pathwayGpi
histidine metabolic pathwayAldh1a1 , Aldh1a3 , Aldh1a7 , Aldh1b1 , Aldh2 , Aldh3a1 , Aldh3a2 , Aldh3b1 , Aldh7a1 , Aldh9a1
histidinemia disease pathwayAldh2 , Aldh3a1
homocarnosinosis disease pathwayGot2
hyperargininemia disease pathwayGot2
hyperglycerolemia disease pathwayAldh3a1
hyperlipoproteinemia type I disease pathwayAldh3a1
hyperlysinemias disease pathwayAldh7a1 , Dld
hyperornithinemia-hyperammonemia-homocitrullinemia syndrome pathwayGot1
hyperprolinemia disease pathwayGot1
hyperprolinemia type II disease pathwayGot1
hypoxia inducible factor pathwayAldoa , Eno1 , Gck , Hk1 , Hk2 , Ldha , Pfkl , Pgk1 , Pgm1
ifosfamide pharmacodynamics pathwayAldh1a1 , Aldh3a1
ifosfamide pharmacokinetics pathwayAldh1a1 , Aldh3a1
inositol phosphate metabolic pathwayRGD1563601 , Tpi1
insulin secretion pathwayGck , Slc2a2
insulin signaling pathwayFbp1 , Fbp2 , G6pc , Gck , Hk1 , Hk2 , Hk3 , Pck1 , Pck2 , Pklr
isobutyryl-CoA dehydrogenase deficiency pathwayAldh2 , Dld
isovaleric acidemia disease pathwayAldh2 , Dld
kynurenine metabolic pathwayGot2
lactic acidosis pathwayPc
lactose biosynthetic pathwayG6pc
lactose degradation pathwaySlc2a2
lamivudine pharmacokinetics pathwayPgk1
Leigh syndrome pathwayAcss2 , Aldh2 , Dlat , Dld , Pc , Pck1 , Pdha1 , Pdhb , Pklr
lysine biosynthetic pathwayAldh7a1
lysine degradation pathwayAldh1a1 , Aldh1a7 , Aldh1b1 , Aldh2 , Aldh3a2 , Aldh7a1 , Aldh9a1 , Dld
malonic aciduria disease pathwayAcss1
malonic aciduria pathwayAcss1
maple syrup urine disease pathwayAldh2 , Dld
maturity onset diabetes of the young pathwayGck , Pklr , Slc2a2
meracaptolactate cysteine disulfiduria pathwayGot1
methylmalonate semialdehyde dehydrogenase deficiency pathwayAldh2 , Dld
methylmalonic acidemia disease pathwayAldh2 , Dld
methylmalonic aciduria, cobalamin-related, disease pathwayAcss1
mitochondrial complex II deficiency pathwayDlat , Dld , Mdh2 , Mpc1 , Pc , Pdha1 , Pdhb
neviparine pharmacokinetics pathwayAldh1a1
nicotinamide adenine dinucleotide metabolic pathwayGapdh , Got1 , Got2 , Mdh2 , Slc25a11
nonketotic hyperglycinemia disease pathwayAldh2 , Dld
Nonketotic hyperglycinemia disease pathwayAldh2 , Dld
Notch signaling pathwayEno1
nucleotide sugar metabolic pathwayGck , Gpi , Hk1 , Hk2 , Hk3 , Pgm1
ocular nephropathic cystinosis disease pathwayGot1
ornithine carbamoyltransferase deficiency disease pathwayGot2
pantothenic acid metabolic pathwayPank1
pentose and glucuronate interconversion pathwayAldh1a1 , Aldh1a7 , Aldh1b1 , Aldh2 , Aldh3a2
pentose phosphate pathwayAldoa , Aldob , Aldoc , Fbp1 , Fbp2 , Gpi , Pfkl , Pfkm , Pfkp , Pgm1
phase I biotransformation pathway via cytochrome P450Adh4 , Adh5 , Adh6 , Adh7 , Aldh1a3 , Aldh3a1 , Aldh3b1
phenylalanine metabolic pathwayAldh1a3 , Aldh3a1 , Aldh3b1 , Got1 , Got2
phenylalanine, tyrosine and tryptophan biosynthetic pathwayGot1 , Got2
phenylketonuria disease pathwayGot1
phosphoenolpyruvate carboxykinase deficiency pathwayAldoa , Bpgm , Eno1 , Fbp1 , G6pc , Galm , Gapdh , Gpi , Hk2 , Mdh2 , Mpc1 , Pank1 , Pc , Pck1 , Pgam1 , Pgam2 , Pgm1 , Slc25a11 , Slc2a2 , Slc37a4 , Tpi1
phytanic acid degradation pathwayAldh3a2
potassium channel inhibitors pharmacodynamics pathwaySlc2a2
primary hyperoxaluria type 1 disease pathwayPc
primary hyperoxaluria type 2 disease pathwayAcss2 , Aldh2 , Dlat , Dld , Pc , Pck1 , Pdha1 , Pdhb , Pklr
prolidase deficiency pathwayGot1
propanoate metabolic pathwayAcss1 , Acss2 , Aldh1a1 , Aldh1a7 , Aldh1b1 , Aldh2 , Aldh3a2 , Aldh7a1 , Aldh9a1 , Ldha , Ldhal6b , Ldhb , Ldhc , RGD1562690
propionic acidemia disease pathwayAldh2 , Dld
prostaglandin biosynthetic pathwayAkr1a1
purine metabolic pathwayPgm1 , Pklr
pyruvate carboxylase deficiency disease pathwayPc
pyruvate dehydrogenase complex deficiency pathwayAcss2 , Aldh2 , Dlat , Dld , Pc , Pck1 , Pdha1 , Pdhb , Pklr
pyruvate dehydrogenase E1 deficiency pathwayAcss2 , Aldh2 , Dlat , Dld , Pc , Pck1 , Pdha1 , Pdhb , Pklr
pyruvate dehydrogenase E2 deficiency pathwayDlat , Dld , Mdh2 , Mpc1 , Pc , Pdha1 , Pdhb
pyruvate dehydrogenase E3 deficiency pathwayDlat , Dld , Mdh2 , Mpc1 , Pc , Pdha1 , Pdhb
pyruvate kinase deficiency pathwayAcss2 , Aldh2 , Dlat , Dld , Pc , Pck1 , Pdha1 , Pdhb , Pklr
pyruvate metabolic pathwayAcss1 , Acss2 , Aldh1a1 , Aldh1a7 , Aldh1b1 , Aldh2 , Aldh3a2 , Aldh7a1 , Aldh9a1 , Dlat , Dld , Ldha , Ldhal6b , Ldhb , Ldhc , Mdh2 , Pc , Pck1 , Pck2 , Pdha1 , Pdha2 , Pdhb , Pklr , RGD1562690
Refsum disease pathwayAldh3a2
remethylation pathway of homocysteine metabolism - cobalamin independent, betaine dependentAldh7a1
retinoic acid metabolic pathwayAldh1a1 , Aldh1a3
retinol metabolic pathwayAdh4 , Adh5 , Adh6 , Adh7 , Aldh1a1
ribose 5-phosphate isomerase deficiency pathwayAldoa , Fbp1 , Gpi , Pfkl , Pgm1
RNA degradation pathwayEno1 , Eno2 , Eno3
saccharopinuria disease pathwayAldh7a1 , Dld
sarcosinemia disease pathwayAldh2 , Dld
sialic acid storage disease pathwayHk1
starch and sucrose metabolic pathwayG6pc , Gck , Gpi , Hk1 , Hk2 , Hk3 , Pgm1
succinic semialdehyde dehydrogenase deficiency pathwayGot2
Sucrase-isomaltase deficiency pathwayGck , Gpi , Hk2 , Pgm1
Tay-Sachs disease pathwayHk1
transaldolase deficiency pathwayAldoa , Fbp1 , Gpi , Pfkl , Pgm1
trehalose degradation pathwayGck , Slc2a2
triosephosphate isomerase deficiency pathwayAldoa , Bpgm , Eno1 , Fbp1 , G6pc , Galm , Gapdh , Gpi , Hk2 , Mdh2 , Mpc1 , Pank1 , Pc , Pck1 , Pgam1 , Pgam2 , Pgm1 , Slc25a11 , Slc2a2 , Slc37a4 , Tpi1
tryptophan metabolic pathwayAldh1a1 , Aldh1a7 , Aldh1b1 , Aldh2 , Aldh3a2 , Aldh7a1 , Aldh9a1
type 2 diabetes mellitus pathwayGck , Hk1 , Hk2 , Hk3 , Pklr , Slc2a2
type I 3-Methylglutaconic aciduria disease pathwayAldh2 , Dld
type III 3-Methylglutaconic aciduria disease pathwayAldh2 , Dld
tyrosine metabolic pathwayAdh4 , Adh5 , Adh6 , Adh7 , Aldh1a3 , Aldh3a1 , Aldh3b1 , Got1 , Got2
tyrosinemia type I disease pathwayAldh3a1 , Got1
tyrosinemia type II disease pathwayGot1
tyrosinemia type III disease pathwayGot1
urea cycle pathwayGot2
valine, leucine and isoleucine biosynthetic pathwayPdha1 , Pdha2 , Pdhb
valine, leucine and isoleucine degradation pathwayAldh1a1 , Aldh1a7 , Aldh1b1 , Aldh2 , Aldh3a2 , Aldh7a1 , Aldh9a1 , Dld
vitamin A deficiency pathwayAldh1a1
Phenotype Annotations Associated with Genes in the gluconeogenesis pathway

References Associated with the gluconeogenesis pathway:

Ontology Path Diagram:

paths to the root

Import into Pathway Studio: