| MONDO:0004946 | 
                      MONDO:0004946
                      Disease Name:  hypoglycemia 
                      
                      
                      All associated enzymes in DAR: 
                      
                        G6PC1 
                      
                      
                        . The disease is also associated to other non-enzyme proteins (not in DAR).
                      
                      
                   
                  
                  G6PC1 | P35575 | 3.1.3.9 | R-HSA-70263 R-HSA-9615017
 
                          R-HSA-9615017Reactome Name: FOXO-mediated transcription of oxidative stress, metabolic and neuronal genes 
                          See in Reactome | R-HSA-70326 R-HSA-71387
 R-HSA-9614085
 R-HSA-74160
 R-HSA-212436
 R-HSA-1430728
 R-HSA-73857
 | Gene expression (Transcription) Metabolism
 | R-HSA-71825
                             (
                               
                                 R-HSA-70263
                               
                              ) 
 
                          R-HSA-9623192
                             (
                               
                                 R-HSA-9615017
                               
                              )R-HSA-71825Reaction Name: G6PC hydrolyzes glucose 6-phosphate to form glucose and orthophosphate (liver) 
                          See in Reactome 
                          R-HSA-9623192Reaction Name: G6PC gene expression is stimulated by FOXO1,FOXO3,FOXO4,FOXO6 
                          See in Reactome | 
                
                
                  | MONDO:0005290 | 
                      MONDO:0005290
                      Disease Name:  rhabdomyolysis 
                      
                      
                      All associated enzymes in DAR: 
                      
                        PGAM2 
                      
                        ACADVL 
                      
                        AHCY 
                      
                      
                      
                   
                  
                  PGAM2 | P15259 | 5.4.2.11 5.4.2.4
 | R-HSA-70263 R-HSA-70171
 | R-HSA-1430728 R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-71445
                             (
                               
                                 R-HSA-70263
                               
                              ) 
 
                          R-HSA-71654
                             (
                               
                                 R-HSA-70171
                               
                              )R-HSA-71445Reaction Name: 2-Phospho-D-glycerate <=> 3-Phospho-D-glycerate 
                          See in Reactome 
                          R-HSA-71654Reaction Name: 3-Phospho-D-glycerate <=> 2-Phospho-D-glycerate 
                          See in Reactome | 
                
                
                  | MONDO:0009249 | ALDOB | P05062 | 4.1.2.13 | R-HSA-70263 R-HSA-70171
 R-HSA-70350
 | R-HSA-5652084 R-HSA-1430728
 R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-70342
                             (
                               
                                 R-HSA-70350
                               
                              ) 
 
                          R-HSA-71495
                             (
                               
                                 R-HSA-70263
                               
                              )R-HSA-70342Reaction Name: ALDOB tetramer cleaves Fru-1-P to GA and DHAP 
                          See in Reactome 
 
                          R-HSA-71496
                             (
                               
                                 R-HSA-70171
                               
                              )R-HSA-71495Reaction Name: dihydroxyacetone phosphate + D-glyceraldehyde 3-phosphate <=> D-fructose 1,6-bisphosphate 
                          See in Reactome 
                          R-HSA-71496Reaction Name: D-fructose 1,6-bisphosphate <=> dihydroxyacetone phosphate + D-glyceraldehyde 3-phosphate 
                          See in Reactome | 
                
                
                  | MONDO:0009251 | FBP1 | P09467 | 3.1.3.11 | R-HSA-70263 | R-HSA-1430728 R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-70479
                             (
                               
                                 R-HSA-70263
                               
                              ) 
                          R-HSA-70479Reaction Name: D-fructose 1,6-bisphosphate + H2O => D-fructose 6-phosphate + orthophosphate 
                          See in Reactome | 
                
                
                  | MONDO:0009287 | G6PC1 | P35575 | 3.1.3.9 | R-HSA-70263 R-HSA-9615017
 
                          R-HSA-9615017Reactome Name: FOXO-mediated transcription of oxidative stress, metabolic and neuronal genes 
                          See in Reactome | R-HSA-70326 R-HSA-71387
 R-HSA-9614085
 R-HSA-74160
 R-HSA-212436
 R-HSA-1430728
 R-HSA-73857
 | Gene expression (Transcription) Metabolism
 | R-HSA-71825
                             (
                               
                                 R-HSA-70263
                               
                              ) 
 
                          R-HSA-9623192
                             (
                               
                                 R-HSA-9615017
                               
                              )R-HSA-71825Reaction Name: G6PC hydrolyzes glucose 6-phosphate to form glucose and orthophosphate (liver) 
                          See in Reactome 
                          R-HSA-9623192Reaction Name: G6PC gene expression is stimulated by FOXO1,FOXO3,FOXO4,FOXO6 
                          See in Reactome | 
                
                
                  | MONDO:0009864 | PCK2 | Q16822 | 4.1.1.32 | R-HSA-70263 | R-HSA-1430728 R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-372819
                             (
                               
                                 R-HSA-70263
                               
                              ) 
                          R-HSA-372819Reaction Name: oxaloacetate + GTP => phosphoenolpyruvate + GDP + CO2 [mitochondrial matrix] 
                          See in Reactome | 
                
                
                  | MONDO:0009865 | PGAM2 | P15259 | 5.4.2.11 5.4.2.4
 | R-HSA-70263 R-HSA-70171
 | R-HSA-1430728 R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-71445
                             (
                               
                                 R-HSA-70263
                               
                              ) 
 
                          R-HSA-71654
                             (
                               
                                 R-HSA-70171
                               
                              )R-HSA-71445Reaction Name: 2-Phospho-D-glycerate <=> 3-Phospho-D-glycerate 
                          See in Reactome 
                          R-HSA-71654Reaction Name: 3-Phospho-D-glycerate <=> 2-Phospho-D-glycerate 
                          See in Reactome | 
                
                
                  | MONDO:0009866 | PCK1 | P35558 | 4.1.1.32 2.7.11.-
 | R-HSA-70263 R-HSA-9615017
 
 
                          R-HSA-381340R-HSA-9615017Reactome Name: FOXO-mediated transcription of oxidative stress, metabolic and neuronal genes 
                          See in Reactome 
 
                          R-HSA-9632974R-HSA-381340Reactome Name: Transcriptional regulation of white adipocyte differentiation 
                          See in Reactome 
                          R-HSA-9632974Reactome Name: NR1H2 & NR1H3 regulate gene expression linked to gluconeogenesis  
                          See in Reactome | R-HSA-70326 R-HSA-9006931
 
 
                          R-HSA-212436R-HSA-9006931Reactome Name: Signaling by Nuclear Receptors 
                          See in Reactome R-HSA-74160
 R-HSA-71387
 R-HSA-9614085
 R-HSA-1430728
 R-HSA-9024446
 
 
                          R-HSA-162582R-HSA-9024446Reactome Name: NR1H2 and NR1H3-mediated signaling 
                          See in Reactome R-HSA-1266738
 R-HSA-73857
 | Gene expression (Transcription) Metabolism
 Signal Transduction
 Developmental Biology
 | R-HSA-560472
                             (
                               
                                 R-HSA-381340
                               
                              ) 
 
                          R-HSA-70241
                             (
                               
                                 R-HSA-70263
                               
                              )R-HSA-560472Reaction Name: Expression of Phosphoenolpyruvate carboxykinase 1 (PEPCK-C) 
                          See in Reactome 
 
                          R-HSA-9605051
                             (
                               
                                 R-HSA-9632974
                               
                              )R-HSA-70241Reaction Name: oxaloacetate + GTP => phosphoenolpyruvate + GDP + CO2 [cytosol] 
                          See in Reactome 
 
                          R-HSA-9623280
                             (
                               
                                 R-HSA-9615017
                               
                              )R-HSA-9605051Reaction Name: Expression of PCK1 regulated by NR1H3 
                          See in Reactome 
                          R-HSA-9623280Reaction Name: PCK1 gene expression is stimulated by FOXO1:PPARGC1A 
                          See in Reactome | 
                
                
                  | MONDO:0010392 | PGK1 | P00558 | 2.7.2.3 | R-HSA-70263 R-HSA-70171
 | R-HSA-1430728 R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-70486
                             (
                               
                                 R-HSA-70263
                               
                              ) 
 
                          R-HSA-71850
                             (
                               
                                 R-HSA-70171
                               
                              )R-HSA-70486Reaction Name: ATP + 3-Phospho-D-glycerate <=> ADP + 1,3-bisphospho-D-glycerate 
                          See in Reactome 
                          R-HSA-71850Reaction Name: 1,3-bisphospho-D-glycerate + ADP <=> 3-phospho-D-glycerate + ATP 
                          See in Reactome | 
                
                
                  | MONDO:0012747 | ALDOA | P04075 | 4.1.2.13 | R-HSA-6798695 R-HSA-70171
 R-HSA-114608
 R-HSA-70263
 | R-HSA-70326 R-HSA-168256
 R-HSA-76002
 
 
                          R-HSA-168249R-HSA-76002Reactome Name: Platelet activation, signaling and aggregation 
                          See in Reactome R-HSA-76005
 
 
                          R-HSA-1430728R-HSA-76005Reactome Name: Response to elevated platelet cytosolic Ca2+ 
                          See in Reactome R-HSA-109582
 R-HSA-71387
 | Immune System Metabolism
 Hemostasis
 | R-HSA-481007
                             (
                               
                                 R-HSA-114608
                               
                              ) 
 
                          R-HSA-6798745
                             (
                               
                                 R-HSA-6798695
                               
                              )R-HSA-481007Reaction Name: Exocytosis of platelet alpha granule contents 
                          See in Reactome 
 
                          R-HSA-6798748
                             (
                               
                                 R-HSA-6798695
                               
                              )R-HSA-6798745Reaction Name: Exocytosis of tertiary granule lumen proteins 
                          See in Reactome 
 
                          R-HSA-6800434
                             (
                               
                                 R-HSA-6798695
                               
                              )R-HSA-6798748Reaction Name: Exocytosis of secretory granule lumen proteins 
                          See in Reactome 
 
                          R-HSA-71495
                             (
                               
                                 R-HSA-70263
                               
                              )R-HSA-6800434Reaction Name: Exocytosis of ficolin-rich granule lumen proteins 
                          See in Reactome 
 
                          R-HSA-71496
                             (
                               
                                 R-HSA-70171
                               
                              )R-HSA-71495Reaction Name: dihydroxyacetone phosphate + D-glyceraldehyde 3-phosphate <=> D-fructose 1,6-bisphosphate 
                          See in Reactome 
                          R-HSA-71496Reaction Name: D-fructose 1,6-bisphosphate <=> dihydroxyacetone phosphate + D-glyceraldehyde 3-phosphate 
                          See in Reactome | 
                
                
                  | MONDO:0012930 | G6PC3 | Q9BUM1 | 3.1.3.9 | R-HSA-70263 | R-HSA-1430728 R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-3262512
                             (
                               
                                 R-HSA-70263
                               
                              ) 
                          R-HSA-3262512Reaction Name: G6PC3 hydrolyzes glucose 6-phosphate to form glucose and orthophosphate (ubiquitous) 
                          See in Reactome | 
                
                
                  | MONDO:0013046 | ENO3 | P13929 | 4.2.1.11 | R-HSA-70263 R-HSA-70171
 | R-HSA-1430728 R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-70494
                             (
                               
                                 R-HSA-70263
                               
                              ) 
 
                          R-HSA-71660
                             (
                               
                                 R-HSA-70171
                               
                              )R-HSA-70494Reaction Name: Phosphoenolpyruvate + H2O <=> 2-Phospho-D-glycerate 
                          See in Reactome 
                          R-HSA-71660Reaction Name: 2-Phospho-D-glycerate <=> Phosphoenolpyruvate + H2O 
                          See in Reactome | 
                
                
                  | MONDO:0013275 | GPI | P06744 | 5.3.1.9 | R-HSA-6798695 R-HSA-5628897
 
 
                          R-HSA-70171R-HSA-5628897Reactome Name: TP53 Regulates Metabolic Genes 
                          See in Reactome R-HSA-70263
 | R-HSA-3700989 
 
                          R-HSA-70326R-HSA-3700989Reactome Name: Transcriptional Regulation by TP53 
                          See in Reactome R-HSA-71387
 R-HSA-212436
 R-HSA-168249
 R-HSA-168256
 R-HSA-1430728
 R-HSA-74160
 R-HSA-73857
 | Immune System Metabolism
 Gene expression (Transcription)
 | R-HSA-6798748
                             (
                               
                                 R-HSA-6798695
                               
                              ) 
 
                          R-HSA-6800434
                             (
                               
                                 R-HSA-6798695
                               
                              )R-HSA-6798748Reaction Name: Exocytosis of secretory granule lumen proteins 
                          See in Reactome 
 
                          R-HSA-70471
                             (
                               
                                 R-HSA-70171
                               
                              )R-HSA-6800434Reaction Name: Exocytosis of ficolin-rich granule lumen proteins 
                          See in Reactome 
 
                          R-HSA-70475
                             (
                               
                                 R-HSA-5628897;
                               
                              
                               
                                 R-HSA-70263
                               
                              )R-HSA-70471Reaction Name: alpha-D-glucose 6-phosphate <=> D-fructose 6-phosphate 
                          See in Reactome 
                          R-HSA-70475Reaction Name: D-fructose 6-phosphate <=> alpha-D-Glucose 6-phosphate 
                          See in Reactome | 
                
                
                  | MONDO:0014221 | TPI1 | P60174 | 5.3.1.1 4.2.3.3
 | R-HSA-70263 R-HSA-70171
 | R-HSA-1430728 R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-70454
                             (
                               
                                 R-HSA-70171
                               
                              ) 
 
                          R-HSA-70481
                             (
                               
                                 R-HSA-70263
                               
                              )R-HSA-70454Reaction Name: dihydroxyacetone phosphate <=> D-glyceraldehyde 3-phosphate 
                          See in Reactome 
                          R-HSA-70481Reaction Name: D-glyceraldehyde 3-phosphate <=> dihydroxyacetone phosphate 
                          See in Reactome | 
                
                
                  | MONDO:0015025 | MDH2 | P40926 | 1.1.1.37 | R-HSA-70263 R-HSA-71403
 | R-HSA-1428517 
 
                          R-HSA-71406R-HSA-1428517Reactome Name: The citric acid (TCA) cycle and respiratory electron transport 
                          See in Reactome 
 
                          R-HSA-1430728R-HSA-71406Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle 
                          See in Reactome R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-70979
                             (
                               
                                 R-HSA-71403
                               
                              ) 
 
                          R-HSA-71783
                             (
                               
                                 R-HSA-70263;
                               
                              
                               
                                 R-HSA-71403
                               
                              )R-HSA-70979Reaction Name: (S)-Malate + NAD+ <=> Oxaloacetate + NADH + H+ 
                          See in Reactome 
                          R-HSA-71783Reaction Name: Oxaloacetate + NADH + H+ <=> (S)-Malate + NAD+ 
                          See in Reactome | 
                
                
                  | MONDO:0017366 | MDH2 | P40926 | 1.1.1.37 | R-HSA-70263 R-HSA-71403
 | R-HSA-1428517 
 
                          R-HSA-71406R-HSA-1428517Reactome Name: The citric acid (TCA) cycle and respiratory electron transport 
                          See in Reactome 
 
                          R-HSA-1430728R-HSA-71406Reactome Name: Pyruvate metabolism and Citric Acid (TCA) cycle 
                          See in Reactome R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-70979
                             (
                               
                                 R-HSA-71403
                               
                              ) 
 
                          R-HSA-71783
                             (
                               
                                 R-HSA-70263;
                               
                              
                               
                                 R-HSA-71403
                               
                              )R-HSA-70979Reaction Name: (S)-Malate + NAD+ <=> Oxaloacetate + NADH + H+ 
                          See in Reactome 
                          R-HSA-71783Reaction Name: Oxaloacetate + NADH + H+ <=> (S)-Malate + NAD+ 
                          See in Reactome | 
                
                
                  | MONDO:0018141 | PC | P11498 | 6.4.1.1 | R-HSA-196780 
 
                          R-HSA-70263R-HSA-196780Reactome Name: Biotin transport and metabolism 
                          See in Reactome | R-HSA-196854 
 
                          R-HSA-196849R-HSA-196854Reactome Name: Metabolism of vitamins and cofactors 
                          See in Reactome 
 
                          R-HSA-1430728R-HSA-196849Reactome Name: Metabolism of water-soluble vitamins and cofactors 
                          See in Reactome R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-2993802
                             (
                               
                                 R-HSA-196780
                               
                              ) R-HSA-3065959
                             (
                               
                                 R-HSA-196780
                               
                              )
 
 
                          R-HSA-3323111
                             (
                               
                                 R-HSA-196780
                               
                              )R-HSA-3065959Reaction Name: An unknown protease degrades hCBXs 
                          See in Reactome 
 
                          R-HSA-70501
                             (
                               
                                 R-HSA-70263
                               
                              )R-HSA-3323111Reaction Name: Cytoslic carboxylases translocate to mitochondrial matrix 
                          See in Reactome 
                          R-HSA-70501Reaction Name: Pyruvate + CO2 + ATP => ADP + Orthophosphate + Oxaloacetate 
                          See in Reactome | 
                
                
                  | MONDO:0018142 | PC | P11498 | 6.4.1.1 | R-HSA-196780 
 
                          R-HSA-70263R-HSA-196780Reactome Name: Biotin transport and metabolism 
                          See in Reactome | R-HSA-196854 
 
                          R-HSA-196849R-HSA-196854Reactome Name: Metabolism of vitamins and cofactors 
                          See in Reactome 
 
                          R-HSA-1430728R-HSA-196849Reactome Name: Metabolism of water-soluble vitamins and cofactors 
                          See in Reactome R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-2993802
                             (
                               
                                 R-HSA-196780
                               
                              ) R-HSA-3065959
                             (
                               
                                 R-HSA-196780
                               
                              )
 
 
                          R-HSA-3323111
                             (
                               
                                 R-HSA-196780
                               
                              )R-HSA-3065959Reaction Name: An unknown protease degrades hCBXs 
                          See in Reactome 
 
                          R-HSA-70501
                             (
                               
                                 R-HSA-70263
                               
                              )R-HSA-3323111Reaction Name: Cytoslic carboxylases translocate to mitochondrial matrix 
                          See in Reactome 
                          R-HSA-70501Reaction Name: Pyruvate + CO2 + ATP => ADP + Orthophosphate + Oxaloacetate 
                          See in Reactome | 
                
                
                  | MONDO:0018143 | PC | P11498 | 6.4.1.1 | R-HSA-196780 
 
                          R-HSA-70263R-HSA-196780Reactome Name: Biotin transport and metabolism 
                          See in Reactome | R-HSA-196854 
 
                          R-HSA-196849R-HSA-196854Reactome Name: Metabolism of vitamins and cofactors 
                          See in Reactome 
 
                          R-HSA-1430728R-HSA-196849Reactome Name: Metabolism of water-soluble vitamins and cofactors 
                          See in Reactome R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-2993802
                             (
                               
                                 R-HSA-196780
                               
                              ) R-HSA-3065959
                             (
                               
                                 R-HSA-196780
                               
                              )
 
 
                          R-HSA-3323111
                             (
                               
                                 R-HSA-196780
                               
                              )R-HSA-3065959Reaction Name: An unknown protease degrades hCBXs 
                          See in Reactome 
 
                          R-HSA-70501
                             (
                               
                                 R-HSA-70263
                               
                              )R-HSA-3323111Reaction Name: Cytoslic carboxylases translocate to mitochondrial matrix 
                          See in Reactome 
                          R-HSA-70501Reaction Name: Pyruvate + CO2 + ATP => ADP + Orthophosphate + Oxaloacetate 
                          See in Reactome | 
                
                
                  | MONDO:0020091 | PGK1 | P00558 | 2.7.2.3 | R-HSA-70263 R-HSA-70171
 | R-HSA-1430728 R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-70486
                             (
                               
                                 R-HSA-70263
                               
                              ) 
 
                          R-HSA-71850
                             (
                               
                                 R-HSA-70171
                               
                              )R-HSA-70486Reaction Name: ATP + 3-Phospho-D-glycerate <=> ADP + 1,3-bisphospho-D-glycerate 
                          See in Reactome 
                          R-HSA-71850Reaction Name: 1,3-bisphospho-D-glycerate + ADP <=> 3-phospho-D-glycerate + ATP 
                          See in Reactome | 
                
                
                  | MONDO:0030072 | MDH1 | P40925 | 1.1.1.96 1.1.1.37
 | R-HSA-70263 | R-HSA-1430728 R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-198508
                             (
                               
                                 R-HSA-70263
                               
                              ) 
                          R-HSA-198508Reaction Name: malate + NAD+ <=> oxaloacetate + NADH + H+ 
                          See in Reactome | 
                
                
                  | MONDO:0032880 | GOT2 | P00505 | 2.6.1.7 2.6.1.1
 | R-HSA-389661 
 
                          R-HSA-8964539R-HSA-389661Reactome Name: Glyoxylate metabolism and glycine degradation 
                          See in Reactome 
 
                          R-HSA-1614558R-HSA-8964539Reactome Name: Glutamate and glutamine metabolism 
                          See in Reactome 
 
                          R-HSA-8963693R-HSA-1614558Reactome Name: Degradation of cysteine and homocysteine 
                          See in Reactome 
 
                          R-HSA-70263R-HSA-8963693Reactome Name: Aspartate and asparagine metabolism 
                          See in Reactome | R-HSA-71291 
 
                          R-HSA-1614635R-HSA-71291Reactome Name: Metabolism of amino acids and derivatives 
                          See in Reactome R-HSA-1430728
 R-HSA-70326
 R-HSA-71387
 | Metabolism | R-HSA-6784393
                             (
                               
                                 R-HSA-389661
                               
                              ) 
 
                          R-HSA-70596
                             (
                               
                                 R-HSA-8963693
                               
                              )R-HSA-6784393Reaction Name: PXLP-K279-GOT2 dimer transaminates 4-OH-L-glutamate to 4-hydroxy-2-oxoglutarate (HOG) 
                          See in Reactome R-HSA-70613
                             (
                               
                                 R-HSA-70263;
                               
                              
                               
                                 R-HSA-8964539
                               
                              )
 
 
                          R-HSA-9012597
                             (
                               
                                 R-HSA-1614558
                               
                              )R-HSA-70613Reaction Name: oxaloacetate + glutamate <=> aspartate + alpha-ketoglutarate [GOT2] 
                          See in Reactome 
                          R-HSA-9012597Reaction Name: GOT2 dimer transfers amino group from L-Cys to 2OG to form 3MPYR and Glu 
                          See in Reactome |