Biological Pathway: Reactome:R-HSA-6791465

Pentose phosphate pathway disease related metabolites

find 9 related metabolites which is associated with the biological pathway Pentose phosphate pathway disease

this pathway object is a organism specific pathway, which is related to taxonomy Homo sapiens (human).

Mutant forms of two enzymes of the pentose phosphate pathway have been associated with disease in humans. A mutation in ribose-5-phosphate isomerase (RPIA), which normally mediates the reversible interconversion of D-ribulose-5-phosphate and ribose-5-phosphate, has been associated with a slowly progressive leukoencephalopathy, and mutations in transaldolase 1 (TALDO1), which normally mediates the reversible interconversion of D-fructose 6-phosphate and D-erythrose-4-phosphate to form sedoheptulose-7-phosphate and D-glyceraldehyde-3-phosphate, have been associated with congenital liver disease (Wamelink et al. 2008).

View the spectrum consensus network of the metabolites related with current biological pathway.

Sedoheptulose 7-phosphate

[(2R,3R,4R,5S)-2,3,4,5,7-pentahydroxy-6-oxoheptyl] dihydrogen phosphate

C7H15O10P (290.0403)


KEIO_ID S083

   

Sedoheptulose 7-phosphate

sedoheptulose-7-phosphate

C7H15O10P (290.0403)


   

D-Sedoheptulose 7-phosphate

{[(2R,3S,4R,5S,6S)-3,4,5,6-tetrahydroxy-6-(hydroxymethyl)oxan-2-yl]methoxy}phosphonic acid

C7H15O10P (290.0403)


D-Sedoheptulose 7-phosphate (CAS: 2646-35-7) is an intermediate of the pentose phosphate pathway (PPP) that has two functions: (1) the generation of NADPH for reductive syntheses and oxidative stress responses within cells, and (2) the formation of ribose residues for nucleotide and nucleic acid biosynthesis (PMID: 16055050). It is formed by transketolase and acted upon (degraded) by transaldolase. Sedoheptulose 7-phosphate can be increased in the blood of patients affected with a transaldolase deficiency, a genetic disorder (PMID: 12881455). Sedoheptulose is a ketoheptose, a monosaccharide with seven carbon atoms and a ketone functional group. It is one of the few heptoses found in nature (Wikipedia). D-Sedoheptulose 7-phosphate is an intermediate of the Pentose phosphate pathway (PPP) that has two functions: the generation of NADPH for reductive syntheses and oxidative stress responses within cells, and the formation of ribose residues for nucleotide and nucleic acid biosynthesis. (PMID 16055050)

   

D-glyceraldehyde 3-phosphate(2-)

D-glyceraldehyde 3-phosphate(2-)

C3H5O6P-2 (167.9824)


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5-O-phosphonato-D-ribulose

5-O-phosphonato-D-ribulose

C5H9O8P-2 (228.0035)


   

beta-D-fructofuranose 6-phosphate(2-)

beta-D-fructofuranose 6-phosphate(2-)

C6H11O9P-2 (258.0141)


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D-erythrose 4-phosphate(2-)

D-erythrose 4-phosphate(2-)

C4H7O7P-2 (197.9929)


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D-ribofuranose 5-phosphate(2-)

D-ribofuranose 5-phosphate(2-)

C5H9O8P-2 (228.0035)


   

Sedoheptulose 7-phosphate

[(2R,3R,4R,5S)-2,3,4,5,7-pentahydroxy-6-oxoheptyl] dihydrogen phosphate

C7H15O10P (290.0403)


A ketoheptose phosphate consisting of sedoheptulose having a phosphate group at the 7-position. It is an intermediate metabolite in the pentose phosphate pathway.