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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap
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Glycogen metabolism

Assembly and mobilization of a branched glucose polymer, including its chemically distinct terminal reactions. 38 reactions, from Reactome R-HSA-8982491.

  1. GYS1 catalyzes the polyglucosylation of oligoGlc-GYG18 UDP-Glc + oligoGlc-GYG1:GYS1-a tetramer → 8 UDP + polyGlc-GYG1:GYS1-a tetramer + 8 H+
  2. Autoglucosylation of GYG1 complexed with GYS1-b8 UDP-Glc + GYG1:GYS1-b tetramer → 8 UDP + oligoGlc-GYG1:GYS1-b tetramer + 8 H+
  3. GBE1 catalyzes branch formation in polyGlc-GYG1 complexed with GYS1-apolyGlc-GYG1:GYS1-a tetramer → glycogen-GYG1:GYS1-a tetramer
  4. GYS2 catalyzes the polyglucosylation of oligoGlc-GYG28 UDP-Glc + oligoGlc-GYG2:GYS2-a tetramer → 8 UDP + polyGlc-GYG2:GYS2-a tetramer + 8 H+
  5. Autoglucosylation of GYG2 complexed with GYS2-a8 UDP-Glc + GYG2:GYS2-a tetramer → 8 UDP + oligoGlc-GYG2:GYS2-a tetramer + 8 H+
  6. GBE1 catalyzes branch formation in polyGlc-GYG2 complexed with GYS2-apolyGlc-GYG2:GYS2-a tetramer → glycogen-GYG2:GYS2-a tetramer
  7. Autoglucosylation of GYG2 complexed with GYS2-b8 UDP-Glc + GYG2:GYS2-b tetramer → 8 UDP + oligoGlc-GYG2:GYS2-b tetramer + 8 H+
  8. Autoglucosylation of GYG1 complexed with GYS1-a8 UDP-Glc + GYG1:GYS1-a tetramer → 8 UDP + oligoGlc-GYG1:GYS1-a tetramer + 8 H+
  9. Phosphorylated GYS1 catalyzes the polyglucosylation of oligoGlc-GYG18 UDP-Glc + oligoGlc-GYG1:GYS1-b tetramer → 8 UDP + polyGlc-GYG1:GYS1-b tetramer + 8 H+
  10. GBE1 catalyzes branch formation in polyGlc-GYG1 complexed with GYS1-bpolyGlc-GYG1:GYS1-b tetramer → glycogen-GYG1:GYS1-b tetramer
  11. GYS2 catalyzes the incorporation of phosphoglucose into glycogen-GYG2PPP1R3C:glycogen-GYG2:GYS2-a tetramer + UDP-Glc → PPP1R3C:phosphoglycogen-GYG2:GYS2-a tetramer + UMP + H+
  12. NHLRC1 mediated ubiquitination of EPM2A (laforin) and PPP1RC3 (PTG) associated with glycogen-GYG2EPM2A:PPP1R3C:glycogen-GYG2:GYS2-a tetramer + 2 Ub → K48polyUb-PPP1R3C + glycogen-GYG2:GYS2-a tetramer + 2 K48polyUb-EPM2A
  13. PPP1R3C binds to glycogen:GYG2:GYS2PPP1R3C + glycogen-GYG2:GYS2-a tetramer → PPP1R3C:glycogen-GYG2:GYS2-a tetramer
  14. EPM2A dimer binds PPP1R3C:phosphoglycogen-GYG1 complexPPP1R3C:phosphoglycogen-GYG1:GYS1-a tetramer + EPM2A dimer → EPM2A:PPP1R3C:phosphoglycogen-GYG1:GYS1-a tetramer
  15. NHLRC1 mediated ubiquitination of EPM2A and PPP1RC3 associated with glycogen-GYG1EPM2A:PPP1R3C:glycogen-GYG1:GYS1-a tetramer + 2 Ub → K48polyUb-PPP1R3C + glycogen-GYG1:GYS1-a tetramer + 2 K48polyUb-EPM2A
  16. EPM2A dimer dephosphorylates phosphoglycogen-GYG2EPM2A:PPP1R3C:phosphoglycogen-GYG2:GYS2-a tetramer + H2O → EPM2A:PPP1R3C:glycogen-GYG2:GYS2-a tetramer + Pi
  17. EPM2A dimer dephosphorylates phosphoglycogen-GYG1EPM2A:PPP1R3C:phosphoglycogen-GYG1:GYS1-a tetramer + H2O → EPM2A:PPP1R3C:glycogen-GYG1:GYS1-a tetramer + Pi
  18. EPM2A dimer binds PPP1R3C:phosphoglycogen-GYG2 complexEPM2A dimer + PPP1R3C:phosphoglycogen-GYG2:GYS2-a tetramer → EPM2A:PPP1R3C:phosphoglycogen-GYG2:GYS2-a tetramer
  19. PPP1R3C binds to glycogen:GYG1:GYS1PPP1R3C + glycogen-GYG1:GYS1-a tetramer → PPP1R3C:glycogen-GYG1:GYS1-a tetramer
  20. GYS1 catalyzes the incorporation of phosphoglucose into glycogen-GYG1PPP1R3C:glycogen-GYG1:GYS1-a tetramer + UDP-Glc → PPP1R3C:phosphoglycogen-GYG1:GYS1-a tetramer + UMP + H+
  21. glycogen phosphorylase (PYGB) dimer b + 2 ATP => glycogen phosphorylase (PYGB) dimer a + 2 ADPPYGB dimer b form + 2 ATP → PYGB dimer a form + 2 ADP + 2 H+
  22. PYGM b dimer:AMP complex => PYGM dimer, b form + 2 AMPPYGM b dimer:AMP → PYGM dimer, b form + 2 AMP
  23. poly((1,4)-alpha-glucosyl) glycogenin-2 + n orthophosphate => glycogenin-2 + n D-glucose 1-phosphate [PYGL]poly((1,4)-alpha-glucosyl)GYG2 dimer + Pi → GYG2 dimer + G1P
  24. PYGM dimer, b form + 2 AMP => PYGM b dimer:AMP complexPYGM dimer, b form + 2 AMP → PYGM b dimer:AMP
  25. PYGB b dimer:AMP complex => PGYB dimer, b form + 2 AMPPYGB b dimer:AMP → PYGB dimer b form + 2 AMP
  26. PYGB dimer, b form + 2 AMP => PYGB b dimer:AMP complexPYGB dimer b form + 2 AMP → PYGB b dimer:AMP
  27. poly((1,4)-alpha-glycosyl) glycogenin-1 + n orthophosphate => glycogenin-1 + n D-glucose 1-phosphate [PYGM,PYGB]poly((1,4)-alpha-glucosyl)GYG1 dimer + Pi → GYG1 dimer + G1P
  28. UTP + D-glucose 1-phosphate => pyrophosphate + UDP-glucoseUTP + G1P + H+ → PPi + UDP-Glc
  29. glycogen-glycogenin-1 + n orthophosphate => limit dextrin-glycogenin-1 + n D-glucose 1-phosphate [PYGM,PYGB]glycogen-glycogenin-1 dimer + Pi → limit dextrin-glycogenin-1 dimer + G1P
  30. glycogen phosphorylase (PYGM) dimer b + 2 ATP => glycogen phosphorylase (PYGM) dimer a + 2 ADPPYGM dimer, b form + 2 ATP → PYGM dimer a form + 2 ADP + 2 H+
  31. limit dextrin-glycogenin => ((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl) glycogeninlimit dextrin-glycogenin dimer → ((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl)GYG dimer
  32. glycogen phosphorylase (PYGL) dimer b + 2 ATP => glycogen phosphorylase (PYGL) dimer a + 2 ADPPYGL dimer b form + 2 ATP → PYGL dimer a form + 2 ADP + 2 H+
  33. glycogen-glycogenin-2 + n orthophosphate => limit dextrin-glycogenin-2 + n D-glucose 1-phosphate [PYGL]glycogen-glycogenin-2 dimer + Pi → limit dextrin-glycogenin-2 dimer + G1P
  34. ((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl)glycogenin => poly{(1,4)-alpha-glucosyl} glycogenin + alpha-D-glucose((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl)GYG dimer → poly((1,4)-alpha-glucosyl)GYG dimer + Glc
  35. GAA hydrolyzes lysosomal glycogenglycogen-GYG1 dimer + 2 H2O → poly((1,4)-alpha-glucosyl)GYG1 dimer + 2 Glc
  36. PGM1:Mg2+ isomerises G1P to G6PG1P → G6P
  37. PGM1:Mg2+ isomerises G6P to G1PG6P → G1P
  38. AKR1E2 reduces 1,5-aF to 1,5-aG1,5-aF + H+ + NADPH → 1,5-aG + NADP+