PENTOSE PHOSPHATE PATHWAY (PW:0000045)

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Description

The pentose phosphate pathway (PPP), also known as the pentose phosphate shunt, represents an alternate route of glucose oxidation. The major route of glucose oxidation in many tissues is via glycolysis. However, 10-20% of glucose will be oxidized via the PPP and this percentage may be higher in tissues involved in lipid biosynthesis. The pathway consists of an oxidative and a non-oxidative phase. The oxidative phase is a series of three irreversible reactions that produce the reducing equivalen

Pathway Diagram:

Elsevier Inc. glycolysis pathway gluconeogenesis pathway glyceraldehyde-3-phosphate sedoheptulose-7-phosphate nucleotide metabolic pathway fatty acid biosynthetic pathway cholesterol biosynthetic pathway NADPH2 NADPH1 Pgd Rpia Rpe Tkt Taldo1 Tkt(2) antioxidation Pgls G6pd fructose-6-phosphate (2) ---> glycolysis pathway ribose-5-phosphate ---> nucleotide metabolic pathway sedoheptulose-7-phosphate <--> ribose-5-phosphate xylulose 5-phosphate <--> ribulose-5-phosphate glucose-6-phosphate ---> NADPH1 fructose-6-phosphate <--> sedoheptulose-7-phosphate ribulose-5-phosphate <--> ribose-5-phosphate glyceraldehyde-3-phosphate <--> erythrose 4-phosphate xylulose 5-phosphate <--> fructose-6-phosphate (2) 6-phosphogluconolactone ribulose-5-phosphate glyceraldehyde-3-phosphate (2) fructose-6-phosphate fructose-6-phosphate (2) glucose-6-phosphate glucose-6-phosphate ---> 6-phosphogluconolactone xylulose 5-phosphate ribose-5-phosphate glyceraldehyde-3-phosphate <--> xylulose 5-phosphate 6-phosphogluconate 6-phosphogluconolactone ---> 6-phosphogluconate 6-phosphogluconate ---> NADPH2 6-phosphogluconate ---> ribulose-5-phosphate NADPH1 ---> fatty acid biosynthetic pathway NADPH2 ---> cholesterol biosynthetic pathway erythrose 4-phosphate erythrose 4-phosphate <--> glyceraldehyde-3-phosphate (2) glucose-6-phosphate ---> glycolysis pathway glyceraldehyde-3-phosphate (2) ---> glycolysis pathway
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Genes in Pathway:

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pentose phosphate pathway term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Aldoa aldolase, fructose-bisphosphate A JBrowse link 1 198,228,387 198,233,988 RGD:6907045
RGD:10402751
G Aldob aldolase, fructose-bisphosphate B JBrowse link 5 64,805,772 64,818,813 RGD:6907045
G Aldoc aldolase, fructose-bisphosphate C JBrowse link 10 65,586,504 65,590,093 RGD:6907045
G Dera deoxyribose-phosphate aldolase JBrowse link 4 171,748,254 171,826,414 RGD:10402751
G Fbp1 fructose-bisphosphatase 1 JBrowse link 17 396,175 417,480 RGD:6907045
RGD:10402751
G Fbp2 fructose-bisphosphatase 2 JBrowse link 17 372,554 389,918 RGD:6907045
G G6pd glucose-6-phosphate dehydrogenase JBrowse link X 156,274,800 156,293,935 RGD:6907045
RGD:10402751
RGD:1599574
G Gpi glucose-6-phosphate isomerase JBrowse link 1 90,063,411 90,091,287 RGD:6907045
RGD:10402751
G H6pd hexose-6-phosphate dehydrogenase (glucose 1-dehydrogenase) JBrowse link 5 166,994,683 167,030,441 RGD:6907045
G Pfkl phosphofructokinase, liver type JBrowse link 20 11,393,860 11,415,889 RGD:6907045
RGD:10402751
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 Pgd phosphogluconate dehydrogenase JBrowse link 5 165,966,128 165,982,327 RGD:1599574
RGD:10402751
G Pgls 6-phosphogluconolactonase JBrowse link 16 20,020,444 20,025,930 RGD:6907045
RGD:10402751
G Pgm1 phosphoglucomutase 1 JBrowse link 5 118,743,632 118,803,055 RGD:6907045
RGD:10402751
G Prps1 phosphoribosyl pyrophosphate synthetase 1 JBrowse link X 111,798,233 111,820,270 RGD:6907045
G Prps1l1 phosphoribosyl pyrophosphate synthetase 1-like 1 JBrowse link 6 54,417,903 54,419,593 RGD:6907045
RGD:10402751
G Prps2 phosphoribosyl pyrophosphate synthetase 2 JBrowse link X 28,435,275 28,472,882 RGD:6907045
G Rbks ribokinase JBrowse link 6 26,051,568 26,128,762 RGD:6907045
RGD:10402751
G Rgn regucalcin JBrowse link X 1,833,484 1,848,904 RGD:6907045
G Rpe ribulose-5-phosphate-3-epimerase JBrowse link 9 73,687,684 73,713,643 RGD:1599574
RGD:10402751
RGD:6907045
G Rpia ribose 5-phosphate isomerase A JBrowse link 4 98,568,028 98,593,664 RGD:1599574
RGD:10402751
RGD:6907045
G Taldo1 transaldolase 1 JBrowse link 1 214,375,555 214,385,886 RGD:6907045
RGD:10402751
RGD:1599574
G Tkt transketolase JBrowse link 16 6,609,670 6,634,608 RGD:6907045
RGD:10402751
RGD:1599574
G Tktl1 transketolase-like 1 JBrowse link X 156,507,797 156,540,733 RGD:6907045
G Tktl2 transketolase-like 2 JBrowse link 16 24,944,197 24,951,478 RGD:6907045
pentose phosphate pathway - non-oxidative phase term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G Rpe ribulose-5-phosphate-3-epimerase JBrowse link 9 73,687,684 73,713,643 RGD:1641814
G Rpia ribose 5-phosphate isomerase A JBrowse link 4 98,568,028 98,593,664 RGD:1641814
G Taldo1 transaldolase 1 JBrowse link 1 214,375,555 214,385,886 RGD:1641803
RGD:1641814
G Tkt transketolase JBrowse link 16 6,609,670 6,634,608 RGD:1641798
RGD:1580394
RGD:1641814
pentose phosphate pathway - oxidative phase term browser
Symbol Object Name JBrowse Chr Start Stop Reference
G G6pd glucose-6-phosphate dehydrogenase JBrowse link X 156,274,800 156,293,935 RGD:1625539
G Pgd phosphogluconate dehydrogenase JBrowse link 5 165,966,128 165,982,327 RGD:1625539
G Pgls 6-phosphogluconolactonase JBrowse link 16 20,020,444 20,025,930 RGD:2311501

Pathway Gene Annotations

Disease Annotations Associated with Genes in the pentose phosphate pathway
Disease TermsGene Symbols
Acid-Base ImbalanceFbp1
acute kidney failureG6pd
acute myeloid leukemiaGpi
ALAD-Deficiency PorphyriaG6pd
anemiaG6pd
Animal Disease ModelsGpi
Arts syndromePrps1
autism spectrum disorderAldoa
autoimmune disease of the nervous systemAldoc
autoimmune hepatitisAldob
autoimmune polyendocrine syndrome type 1Pfkl
borna diseaseAldoc
Brain InjuriesG6pd
Breast NeoplasmsAldoa , Gpi
carbohydrate metabolic disorderTaldo1
carcinomaTkt
cataractG6pd
cerebrovascular diseaseG6pd
Charcot-Marie-Tooth diseasePrps1
Charcot-Marie-Tooth disease X-linked recessive 5Prps1
Chemical and Drug Induced Liver InjuryAldob , G6pd , Pgm1 , Rgn , Taldo1
cholestasisG6pd
chronic granulomatous diseaseG6pd
Colonic NeoplasmsFbp1
Colorectal NeoplasmsPgm1
congenital disorder of glycosylationPgm1
congenital disorder of glycosylation type IPgm1
congenital hemolytic anemiaG6pd , Gpi
congenital hypothyroidismG6pd
congenital merosin-deficient muscular dystrophy 1AG6pd
congenital nonspherocytic hemolytic anemiaG6pd , Gpi
contact dermatitisG6pd
coronin-1A deficiencyAldoa
cortisone reductase deficiencyH6pd
cortisone reductase deficiency 1H6pd
cystadenomaAldoa
Deafness, High-Frequency Sensorineural, X-LinkedPrps1
Deafness, X-Linked 1Prps1
Dementia, Multi-InfarctG6pd
diabetes mellitusAldob , G6pd
Diabetic NephropathiesG6pd
Encephalopathy, Neonatal Severe, due to Mecp2 MutationsG6pd , Tktl1
Endometrial NeoplasmsPgm1
EndotoxemiaAldoa
Experimental ArthritisGpi
Experimental Diabetes MellitusAldob , G6pd , H6pd , Pfkm , Rgn
Experimental Liver CirrhosisPfkp , Rgn
familial hyperlipidemiaRgn
favismG6pd
FeverG6pd
fructose-1,6-bisphosphatase deficiencyFbp1
fundus dystrophyPrps1
genetic diseaseG6pd , Prps1
glucosephosphate dehydrogenase deficiencyG6pd
glycogen storage disease VIIPfkm
Glycogen Storage Disease XIIAldoa
Glycogen Storage Disease XIVPgm1
Hearing LossPrps1
HemolysisG6pd
hemolytic anemiaG6pd , Gpi
hepatic encephalopathyG6pd
hepatocellular carcinomaAldoc , Fbp1 , G6pd , Pgd , Rgn , Taldo1
hereditary fructose intolerance syndromeAldoa , Aldob
Hyperbilirubinemia, NeonatalG6pd
HypercholesterolemiaG6pd
hyperhomocysteinemiaG6pd
HypertriglyceridemiaG6pd
HypoxiaAldob , Tkt
Insulin ResistanceG6pd
intellectual disabilityGpi
kidney diseaseG6pd , Rgn
liver cirrhosisRgn
Liver InjuryG6pd
liver neoplasmAldob
Liver Neoplasms, ExperimentalRgn
lung adenocarcinomaAldoa , Gpi
malariaG6pd
Mammary Neoplasms, AnimalTkt
Mammary Neoplasms, ExperimentalTkt
Metabolic SyndromeAldoa , G6pd
microcephalyTaldo1
Microphthalmia, Syndromic 1Pfkp
multiple sclerosisH6pd
myocardial infarctionG6pd
Myocardial IschemiaAldoa , Pfkm , Pgm1
neonatal jaundiceG6pd
Neoplasm InvasivenessAldoa , Tkt
Neoplasm MetastasisAldoa , Gpi
neurodegenerative diseaseRgn
Neurologic ManifestationsPrps1
neuromuscular diseaseGpi
nonalcoholic fatty liver diseaseG6pd
Nonspherocytic Hemolytic Anemia due to Glucose Phosphate Isomerase DeficiencyGpi
Nonspherocytic Hemolytic Anemia, due to G6PD DeficiencyG6pd
obesityG6pd
osteoporosisPgls
Parkinson's diseaseFbp1 , Taldo1
peritonitisAldob
phagocyte bactericidal dysfunctionG6pd
phenylketonuriaG6pd
Phosphoribosylpyrophosphate Synthetase SuperactivityPrps1
Plasmodium Falciparum Blood Infection LevelG6pd
pleomorphic xanthoastrocytomaPrps1l1
precursor lymphoblastic lymphoma/leukemiaGpi
Pulmonary Hypertension, Hypoxia-Induced G6pd
renovascular hypertensionG6pd
Reperfusion InjuryAldoa , G6pd
retinitis pigmentosaPrps1
Ribose 5-Phosphate Isomerase DeficiencyRpia
schizophreniaAldoa , Aldob
SHORT STATURE, DEVELOPMENTAL DELAY, AND CONGENITAL HEART DEFECTSTkt
Smith-Magenis syndromeAldoc
Spondylocostal Dysostosis 5Aldoa
Spondylometaepiphyseal Dysplasia, Short Limb-Hand TypeTkt
Stomach NeoplasmsAldob , Fbp1
StrokeAldoc
teratomaPgm1
Testicular InjuryG6pd
Transaldolase DeficiencyTaldo1
transient cerebral ischemiaG6pd
type 1 diabetes mellitusG6pd
type 2 diabetes mellitusAldob , G6pd
Weight GainPrps1
Wernicke encephalopathyTkt
X-linked nonsyndromic deafnessPrps1
Pathway Annotations Associated with Genes in the pentose phosphate pathway
Pathway TermsGene Symbols
adenine phoshoribosyltransferase deficiency pathwayPrps1l1
adenosine monophosphate deaminase deficiency pathwayPrps1l1
adenylosuccinate lyase deficiency pathwayPrps1l1
AICA-ribosuria pathwayPrps1l1
amino sugar metabolic pathwayGpi , Pgm1
ascorbate and aldarate metabolic pathwayRgn
azathioprine pharmacodynamics pathwayPrps1l1
congenital sucrase-isomaltase deficiency pathwayGpi , Pgm1
de novo purine biosynthetic pathwayPrps1 , Prps1l1 , Prps2
Fanconi syndrome pathwayAldoa , Gpi , Pfkm
forkhead class A signaling pathwayAldob
fructose and mannose metabolic pathwayAldoa , Aldob , Aldoc , Fbp1 , Fbp2 , Pfkl , Pfkm , Pfkp
fructose-1,6-bisphosphatase deficiency pathwayAldoa , Fbp1 , Gpi , Pgm1
fructosuria pathwayAldoa , Aldob , Fbp1 , Pfkl
galactokinase deficiency pathwayPgm1
galactose metabolic pathwayPfkl , Pfkm , Pfkp , Pgm1
galactosemia pathwayPgm1
GALE deficiency pathwayPgm1
gluconeogenesis pathwayAldoa , Aldob , Aldoc , Fbp1 , Fbp2 , Gpi , Pfkl , Pfkm , Pfkp , Pgm1
glutathione metabolic pathwayG6pd
glycogen biosynthetic pathwayPgm1
glycogen degradation pathwayPgm1
glycogen storage disease type Ia pathwayAldoa , Fbp1 , Gpi , Pgm1
glycogen storage disease type Ib pathwayAldoa , Fbp1 , Gpi , Pgm1
glycogen storage disease type III pathwayGpi , Pgm1
glycogen storage disease type IV pathwayGpi , Pgm1
glycogen storage disease type VI pathwayGpi , Pgm1
glycogen storage disease type VII pathwayAldoa , Gpi , Pfkm
glycolysis pathwayAldoa , Aldob , Aldoc , Fbp1 , Fbp2 , Gpi , Pfkl , Pfkm , Pfkp , Pgm1
glycolysis/gluconeogenesis pathwayAldoa , Aldob , Aldoc , Fbp1 , Fbp2 , Gpi , Pfkl , Pfkm , Pfkp , Pgm1
hereditary fructose intolerance syndrome pathwayAldoa , Aldob , Fbp1 , Pfkl
hexosamine biosynthetic pathwayGpi
hypoxia inducible factor pathwayAldoa , Pfkl , Pgm1
insulin signaling pathwayFbp1 , Fbp2
Kelley-Seegmiller syndrome pathwayPrps1l1
Lesch-Nyhan syndrome pathwayPrps1l1
mercaptopurine pharmacodynamics pathwayPrps1l1
molybdenum cofactor deficiency pathwayPrps1l1
nucleotide sugar metabolic pathwayGpi , Pgm1
pentose and glucuronate interconversion pathwayRpe
pentose phosphate pathwayAldoa , Aldob , Aldoc , Dera , Fbp1 , Fbp2 , G6pd , Gpi , H6pd , Pfkl , Pfkm , Pfkp , Pgd , Pgls , Pgm1 , Prps1 , Prps1l1 , Prps2 , Rbks , Rgn , Rpe , Rpia , Taldo1 , Tkt , Tktl1 , Tktl2
pentose phosphate pathway - non-oxidative phaseRpe , Rpia , Taldo1 , Tkt
pentose phosphate pathway - oxidative phaseG6pd , Pgd , Pgls
phosphoenolpyruvate carboxykinase deficiency pathwayAldoa , Fbp1 , Gpi , Pgm1
purine metabolic pathwayPgm1 , Prps1 , Prps1l1 , Prps2
purine nucleoside phosphorylase deficiency pathwayPrps1l1
ribose 5-phosphate isomerase deficiency pathwayAldoa , Dera , Fbp1 , G6pd , Gpi , Pfkl , Pgd , Pgls , Pgm1 , Prps1l1 , Rbks , Rpe , Rpia , Taldo1 , Tkt
starch and sucrose metabolic pathwayGpi , Pgm1
tioguanine pharmacodynamics pathwayPrps1l1
transaldolase deficiency pathwayAldoa , Dera , Fbp1 , G6pd , Gpi , Pfkl , Pgd , Pgls , Pgm1 , Prps1l1 , Rbks , Rpe , Rpia , Taldo1 , Tkt
triosephosphate isomerase deficiency pathwayAldoa , Fbp1 , Gpi , Pgm1
xanthinuria type I pathwayPrps1l1
xanthinuria pathwayPrps1l1
xanthinuria type II pathwayPrps1l1

References Associated with the pentose phosphate pathway:

Ontology Path Diagram:

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