ABCC5
MRP5
UniProt:O15440 ABCC5
TREA
TREH
UniProt:O43280 TREH
G6PT
G6PT1
PRO0685
SLC37A4
TRG19
UniProt:O43826 SLC37A4
CHST1
UniProt:O43916 CHST1
GLCE
KIAA0836
UniProt:O94923 GLCE
PGLS
UniProt:O95336 PGLS
GALT
UniProt:P07902 GALT
GNS
UniProt:P15586 GNS
GLUT5
SLC2A5
UniProt:P22732 SLC2A5
IDUA
UniProt:P35475 IDUA
PCK1
PEPCK1
UniProt:P35558 PCK1
AGL
GDE
UniProt:P35573 AGL
G6PC
UniProt:P35575 G6PC
TAL
TALDO
TALDO1
TALDOR
UniProt:P37837 TALDO1
RPI
RPIA
UniProt:P49247 RPIA
GALK
GALK1
UniProt:P51570 GALK1
HSS
SGSH
UniProt:P51688 SGSH
SLC20A3
SLC25A1
UniProt:P53007 SLC25A1
NAGLU
UFHSD1
UniProt:P54802 NAGLU
GBE1
UniProt:Q04446 GBE1
HYAL1
LUCA1
UniProt:Q12794 HYAL1
PCK2
PEPCK2
UniProt:Q16822 PCK2
HFRC
SLC35D2
UGTREL8
UniProt:Q76EJ3 SLC35D2
BRAG
CHST15
GALNAC4S6ST
KIAA0598
UniProt:Q7LFX5 CHST15
HS2ST
HS2ST1
KIAA0448
UniProt:Q7LGA3 HS2ST1
CHST14
D4ST1
UniProt:Q8NCH0 CHST14
HPA2
HPSE2
UniProt:Q8WWQ2 HPSE2
B3GALT6
UniProt:Q96L58 B3GALT6
G6PC3
UGRP
UniProt:Q9BUM1 G6PC3
G6PC2
IGRP
UniProt:Q9NQR9 G6PC2
B4GALT7
UniProt:Q9UBV7 B4GALT7
XGALT1
DIC
SLC25A10
UniProt:Q9UBX3 SLC25A10
DSE
SART2
UniProt:Q9UL01 DSE
DS2ST
UST
UniProt:Q9Y2C2 UST
SLC26A1,2 transport extracellular SO4(2-) to cytosol
malate [mitochondrial matrix] + orthophosphate [cytosol] <=> malate [cytosol] + orthophosphate [mitochondrial matrix]
Transport (influx) of fructose by GLUT5
extracellular fructose => cytosolic fructose
Transport (influx) of glucose, galactose, and sodium ions by SGLT1
Transport (efflux) of fructose, galactose, and glucose by GLUT2
cytosolic fructose, galactose, or glucose => extracellular fructose, galactose, or glucose
Glucose is taken up across the plasma membrane by a glucose transport protein (GLUT)
alpha-D-glucose (extracellular) <=> alpha-D-glucose (cytosol)
Glucose is secreted across the plasma membrane
alpha-D-glucose (cytosol) <=> alpha-D-glucose (extracellular)
GLUT1 + ATP <=> GLUT1:ATP
GLUT1:ATP <=> GLUT1 + ATP
alpha-D-Glucose + ATP => alpha-D-glucose 6-phosphate + ADP
glucokinase (GCK1) + glucokinase regulatory protein (GKRP) <=> GCK1:GKRP complex
cytosolic GCK1:GKRP complex <=> glucokinase (GCK1) + glucokinase regulatory protein (GKRP)
GCK1:GKRP [cytosol] => GCK1:GKRP [nucleoplasm]
Transport of glucokinase (GCK1):GKRP complex to the nucleus
Dissociation of the nucleoplasmic GCK1:GKRP complex
nucleoplasmic GCK1:GKRP complex => glucokinase (GCK1) + glucokinase regulatory protein (GKRP)
glucokinase [nucleoplasm] => glucokinase [cytosol]
Cytosolic glucose 6-phosphate is exchanged for orthophosphate from the endoplasmic reticulum lumen by SLC37A4
alpha-D-glucose 6-phosphate (cytosol) + orthophosphate (ER lumen) => alpha-D-glucose 6-phosphate (ER lumen) + orthophosphate (cytosol) (SLC37A4)
G6PC hydrolyzes glucose 6-phosphate to form glucose and orthophosphate (liver)
alpha-D-Glucose 6-phosphate + H2O => alpha-D-Glucose + Orthophosphate (G6PC - liver)
G6PC2 hydrolyzes glucose 6-phosphate to form glucose and orthophosphate (islet)
alpha-D-Glucose 6-phosphate + H2O => alpha-D-Glucose + Orthophosphate (G6PC2 - islet)
G6PC3 hydrolyzes glucose 6-phosphate to form glucose and orthophosphate (ubiquitous)
alpha-D-Glucose 6-phosphate + H2O => alpha-D-Glucose + Orthophosphate (G6PC3 - ubiquitous)
Efflux of glucose from the endoplasmic reticulum
SLC35B2,3 transport cytosolic PAPS to Golgi lumen
SLC35D2 exchanges UDP-sugars for UMP
HAS1,2,3 mediate the polymerisation of HA
Growing HA is extruded from the cell by ABCC5
Receptor-mediated uptake of HA
HYAL2 interacts with NHE1 to create an acidic environment
Hyaluronidase 2 (HYAL2) hydrolyses HA into 20kDa fragments
20kDa HA fragments are translocated to lysosomes
Hyaluronidase 1 (HYAL1) hydrolyses HA from 20kDa to 800Da fragments
Beta-glucuronidase (GUSB) hydrolyses glucuronate from HA tetrasaccharides
Beta-hexosaminidase A (BHEXA) cleaves the terminal GalNAc from small HA fragments
Beta-glucuronidase (GUSB) hydrolyses glucuronate from the HA disaccharides
The single sugars GlcA and GlcNAc translocate from the lysosome to the cytosol
ATP + sulfate => adenylyl sulfate (APS) + pyrophosphate
Activation of inorganic sulfate forms APS
PAPSS1,2 transfer PO4(2-) group from ATP to form APS
APS reacts with another ATP molecule to form PAPS
PAPSS1,2 transfer PO4(2-) group from ATP to APS to form PAPS
adenylyl sulfate (APS) + ATP => PAPS + ADP
B4GALTs add galactose to the N-glycan precursor
B3GNT1,2,3,4,7 add GlcNAc to form Keratan-PG
B4GALTs add another galactose to the chain
B4GALTs can add galactose to a branch of keratan
The keratan chain can be capped by N-acetylneuraminic acid
Sulfation of GlcNAc residues on Keratan-PG forms KSPG
Further sulfation on galactose residues produces KSPG
KSPG is secreted from the cell
Extracellular KSPG translocates to the lysosome for degradation
Keratan sulfate is cleaved from its proteoglycan by an unknown galactosidase
N-acetylgalactosamine-6-sulfatase (GALNS) hydrolyses the sulfate groups of galactose 6-sulfate units of keratan sulfate
Beta-galactosidase (GLB1) can cleave galactose from a glycosaminoglycan
N-acetylglucosamine 6-sulfatase (GNS) hydrolyses 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of keratan sulfate
Beta-hexosaminidase A (BHEXA) cleaves the terminal GalNAc from keratan sulfate
GXYLTs transfer Xyl to core protein
Xylose is transferred to a serine residue on the core protein
B4GALT7 transfers Gal group to tetrasaccharide linker
Galactose is the second sugar added in the tetrasaccharide linker
A second galactose is added in the tetrasaccharide linker
B3GALT6 transfers Gal to the tetrasaccharide linker
A glucuronate moiety is the fourth addition to the tetrasaccharide linker
B3GATs transfer GlcA to tetrasaccharide linker
The addition of GlcNAc to the terminal glucuronate residue creates heparan
Glucuronate is added to the heparan chain
Another GlcNAc addition extends the heparan chain
Another GlcA addition extends the heparan chain
NDST1-4 N-deacetylates GlcNAc residues in heparan
NDST1-4 can sulfate a glucosamine residue in heparan to form heparan sulfate (HS)
GLCE epimerises GlcA to IdoA
HS2ST1 sulfates IdoA at C2 in heparan sulfate
HS3STs sulfate GlcN at C3 in heparan sulfate
HS3ST1 sulfates GlcN at C3 in heparan sulfate
GLCE epimerises more GlcA to IdoA as sulfate content rises
HS6STs sulfate GlcN at C6 in heparan sulfate/heparin
Some HSPGs are secreted to the plasma membrane
HS-GAGs translocate to the lysosome for degradation
Heparanase (HPSE) cleaves heparan sulfate from its proteoglycan (lysosome)
Heparanase 2 (HPSE2) cleaves heparan sulfate from its proteoglycan (plasma membrane)
Alpha-L-iduronidase (IDUA) hydrolyses the unsulfated alpha-L-iduronosidic linkage in heparan sulfate
N-sulphoglucosamine sulphohydrolase (SGSH) hydrolyses sulfate from the N-sulphoglucosamine residue of heparan sulfate
HGSNAT acetylates the terminal N-glucosamine in heparan sulfate
Alpha-N-acetylglucosaminidase (NAGLU) hydrolyses N-acetyl-D-glucosamine residues from heparan sulfate
Iduronate 2-sulfatase (IDS) hydrolyses sulfates from L-iduronate 2-sulfate units of heparan sulfate
Alpha-L-iduronidase (IDUA) hydrolyses another unsulfated alpha-L-iduronosidic linkage in heparan sulfate
N-sulphoglucosamine sulphohydrolase (SGSH) hydrolyses another sulfate from the N-sulphoglucosamine residue of heparan sulfate
HGSNAT acetylates another terminal N-glucosamine in heparan
Alpha-N-acetylglucosaminidase (NAGLU) hydrolyses another N-acetyl-D-glucosamine residue from heparan
Beta-glucuronidase (GUSB) hydrolyses glucuronate from the linker chain
Beta-galactosidase (GLB1) can cleave galactose from the linker chain
The addition of GalNAc to the terminal glucuronate residue forms chondroitin
A glucuronate moiety is added to chondroitin
GalNAc is added to chondroitin
Chondroitin can be sulfated on position 4 of GalNAc by CHST9, 11, 12 and 13
Chondroitin 4-sulfate (C4S) can be further sulfated on position 6 by CHST15
Chondroitin can be sulfated on position 6 of GalNAc by CHST3,7
CSPG is secreted
Dermatan-sulfate epimerase (DSE) converts chondroitin sulfate (CS) to dermatan sulfate (DS)
Dermatan sulfate can be further sulfated on position 2 of iduronate
CHST14 transfers SO4(2-) to DS
GalNAc is sulfated on position 4 once dermatan sulfate is formed
DSPGs are secreted
DSPG and CSPG translocate to the lysosome for degradation
DS is cleaved from its proteoglycan
CS is cleaved from its proteoglycan
Arylsulfatase B (ARSB) hydrolyses sulfate groups from GalNAc 4-sulfate units of DS
Iduronate 2-sulfatase (IDS) hydrolyses sulfates from L-iduronate 2-sulfate units of dermatan sulfate
Beta-hexosaminidase A (BHEXA) cleaves the terminal GalNAc from DS
Alpha-L-iduronidase (IDUA) hydrolyses the unsulfated alpha-L-iduronosidic linkage in DS
Arylsulfatase B (ARSB) hydrolyses sulfate groups from GalNAc 4-sulfate units of CS
Hyaluronidase 1 (HYAL1) hydrolyses 1-4 linkages between GalNAc and D-glucuronate residues in CS.
Digestion of linear starch (amylose) by extracellular amylase
Digestion of branched starch (amylopectin) by extracellular amylase
Digestion of 1-6 linkages of limit dextrins to yield maltose, maltotriose, longer maltosides, and glucose
Digestion of maltotriose by maltase-glucomylase to yield maltose and glucose
maltotriose + H2O => maltose + D-glucose (maltase-glucoamylase)
Digestion of maltotriose by sucrase-isomaltase to yield maltose and glucose
maltotriose + H2O => maltose + D-glucose (sucrase-isomaltase)
Digestion of maltose by maltase-glucoamylase to yield glucose
maltose + H2O => 2 D-glucose (maltase-glucoamylase)
Digestion of maltose by sucrase-isomaltase to yield glucose
maltose + H2O => 2 D-glucose (sucrase-isomaltase)
Digestion of lactose to yield glucose and galactose
lactose + H2O => D-glucose + D-galactose
Digestion of sucrose to yield glucose and fructose
sucrose + H2O => glucose + fructose
Digestion of trehalose to yield glucose
trehalose + H2O => 2 D-glucose
Glucose 6-phosphate is isomerized to form fructose-6-phosphate
alpha-D-glucose 6-phosphate <=> D-fructose 6-phosphate
Dephosphorylation of phosphoPFKFB1 by PP2A complex
D-fructose 6-phosphate + ATP => D-fructose 2,6-bisphosphate + ADP
Fructose 6-phosphate and ATP react to form fructose 2,6-bisphosphate and ADP
D-fructose 6-phosphate + ATP => D-fructose 1,6-bisphosphate + ADP
D-fructose 1,6-bisphosphate <=> dihydroxyacetone phosphate + D-glyceraldehyde 3-phosphate
dihydroxyacetone phosphate <=> D-glyceraldehyde 3-phosphate
D-glyceraldehyde 3-phosphate + orthophosphate + NAD+ <=> 1,3-bisphospho-D-glycerate + NADH + H+
Glyceraldehyde 3 phosphate, NAD+, and orthophosphate react to form 1,3 bisphosphoglycerate and NADH + H+
1,3-Bisphosphoglycerate and ADP react to form 3-phosphoglycerate and ATP
1,3-bisphospho-D-glycerate + ADP <=> 3-phospho-D-glycerate + ADP
3-Phospho-D-glycerate <=> 2-Phospho-D-glycerate
2-Phospho-D-glycerate <=> Phosphoenolpyruvate + H2O
phosphoenolpyruvate + ADP => pyruvate + ATP
Pyruvate + CO2 + ATP => ADP + Orthophosphate + Oxaloacetate
Pyruvate, CO2, and ATP react to form oxaloacetate, ADP, and orthophosphate
Oxaloacetate + NADH + H+ <=> (S)-Malate + NAD+
(S)-malate + NAD+ <=> oxaloacetate + NADH + H+
malate + NAD+ <=> oxaloacetate + NADH + H+
oxaloacetate + glutamate <=> aspartate + alpha-ketoglutarate [GOT2]
Exchange of malate and alpha-ketoglutarate (2-oxoglutarate) across the inner mitochondrial membrane
malate [mitochondrial matrix] + alpha-ketoglutarate [cytosol] <=> malate [cytosol] + alpha-ketoglutarate [mitochondrial matrix]
Exchange of cytosolic glutamate and mitochondrial aspartate
aspartate [mitochondrial matrix] + glutamate [cytosol] => aspartate [cytosol] + glutamate [mitochondrial matrix]
aspartate + alpha-ketoglutarate <=> oxaloacetate + glutamate [GOT1]
Oxaloacetate and GTP react to form phosphoenolpyruvate, CO2, and GDP [cytosol]
oxaloacetate + GTP => phosphoenolpyruvate + GDP + CO2 [cytosol]
oxaloacetate + GTP => phosphoenolpyruvate + GDP + CO2 [mitochondrial matrix]
Exchange of cytosolic citrate for mitochondrial phosphoenolpyruvate
phosphoenolpyruvate [mitochondrial matrix] + citrate [cytosol] => phosphoenolpyruvate [cytosol] + citrate [mitochondrial matrix]
Phosphoenolpyruvate + H2O <=> 2-Phospho-D-glycerate
2-Phospho-D-glycerate <=> 3-Phospho-D-glycerate
ATP + 3-Phospho-D-glycerate <=> ADP + 1,3-bisphospho-D-glycerate
1,3-bisphospho-D-glycerate + NADH + H+ <=> D-glyceraldehyde 3-phosphate + Orthophosphate + NAD+
D-glyceraldehyde 3-phosphate <=> dihydroxyacetone phosphate
dihydroxyacetone phosphate + D-glyceraldehyde 3-phosphate <=> D-fructose 1,6-bisphosphate
D-fructose 1,6-bisphosphate + H2O => D-fructose 6-phosphate + orthophosphate
Phosphorylation of PF2K-Pase by PKA catalytic subunit
Fructose 2,6-bisphosphate is hydrolyzed to form fructose-6-phosphate and orthophosphate
D-fructose 6-phosphate <=> alpha-D-Glucose 6-phosphate
alpha-D-Glucose 6-phosphate <=> D-Glucose 1-phosphate
UTP + D-glucose 1-phosphate <=> pyrophosphate + UDP-glucose
8 UDP-glucose + glycogenin dimer => 8 UDP + {(1,4)-alpha-D-glucosyl}4 glycogenin dimer
8 UDP-glucose + ((1,4)-alpha-D-glucosyl}4 glycogenin => 8 UDP + ((1,4)-alpha-D-glucosyl)8 glycogenin [GYS I form]
8 UDP-glucose + ((1,4)-alpha-D-glucosyl)4 glycogenin => 8 UDP + ((1,4)-alpha-D-glucosyl)8 glycogenin [GYS D form]
poly((1,4)-alpha-D-glucosyl) glycogenin => glycogen-glycogenin
glycogen phosphorylase (PYGB) dimer b + 2 ATP => glycogen phosphorylase (PYGB) dimer a + 2 ADP
glycogen phosphorylase (PYGM) dimer b + 2 ATP => glycogen phosphorylase (PYGM) dimer a + 2 ADP
glycogen phosphorylase (PYGL) dimer b + 2 ATP => glycogen phosphorylase (PYGL) dimer a + 2 ADP
PGYB dimer, b form + 2 AMP <=> PGYB b dimer:AMP complex
PGYB b dimer:AMP complex <=> PGYB dimer, b form + 2 AMP
PGYM dimer, b form + 2 AMP <=> PGYM b dimer:AMP complex
PGYM b dimer:AMP complex <=> PGYM dimer, b form + 2 AMP
glycogen-glycogenin-1 + n orthophosphate => limit dextrin-glycogenin-1 + n D-glucose 1-phosphate [PYGM,PYGB]
glycogen-glycogenin-2 + n orthophosphate => limit dextrin-glycogenin-2 + n D-glucose 1-phosphate [PYGL]
limit dextrin-glycogenin => ((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl) glycogenin
((1,6)-alpha-glucosyl)poly((1,4)-alpha-glucosyl)glycogenin => poly{(1,4)-alpha-glucosyl} glycogenin + alpha-D-glucose
poly((1,4)-alpha-glycosyl) glycogenin-1 + n orthophosphate => glycogenin-1 + n D-glucose 1-phosphate [PYGM,PYGB]
poly((1,4)-alpha-glucosyl) glycogenin-2 + n orthophosphate => glycogenin-2 + n D-glucose 1-phosphate [PYGL]
D-Glucose 1-phosphate <=> alpha-D-Glucose 6-phosphate
ATP + beta-D-fructose => ADP + D-fructose 1-phosphate
D-fructose 1-phosphate <=> D-glyceraldehyde + dihydroxyacetone phosphate
ATP + D-glyceraldehyde => ADP + D-glyceraldehyde 3-phosphate
ATP + D-galactose => ADP + D-galactose 1-phosphate
D-galactose 1-phosphate + UDP-glucose <=> D-glucose 1-phosphate + UDP-galactose
UDP-galactose <=> UDP-glucose
alpha-D-glucose 6-phosphate + NADP+ => D-glucono-1,5-lactone 6-phosphate + NADPH + H+
D-glucono-1,5-lactone 6-phosphate + H2O => 6-phospho-D-gluconate
6-phospho-D-gluconate + NADP+ => D-ribulose 5-phosphate + CO2 + NADPH + H+
D-ribulose 5-phosphate <=> xylulose 5-phosphate
xylulose 5-phosphate <=> D-ribulose 5-phosphate
ribose 5-phosphate + xylulose 5-phosphate <=> sedoheptulose 7-phosphate + D-glyceraldehyde 3-phosphate
D-glyceraldehyde 3-phosphate + sedoheptulose 7-phosphate<=> xylulose 5-phosphate+ribose 5-phosphate
sedoheptulose 7-phosphate + D-glyceraldehyde 3-phosphate <=> D-erythrose 4-phosphate + D-fructose 6-phosphate
D-fructose 6-phosphate + D-erythrose 4-phosphate <=> sedoheptulose 7-phosphate + D-glyceraldehyde 3-phosphate
xylulose 5-phosphate + D-erythrose 4-phosphate <=> D-glyceraldehyde 3-phosphate + D-fructose 6-phosphate
D-glyceraldehyde 3-phosphate + D-fructose 6-phosphate <=> xylulose 5-phosphate + D-erythrose 4-phosphate
D-ribulose 5-phosphate <=> ribose 5-phosphate
ribose 5-phosphate <=> D-ribulose 5-phosphate
D-ribose 5-phosphate + 2'-deoxyadenosine 5'-triphosphate (dATP) => 5-Phospho-alpha-D-ribose 1-diphosphate (PRPP) + 2'-deoxyadenosine 5'-monophosphate
D-ribose 5-phosphate + ATP => 5-phospho-alpha-D-ribose 1-diphosphate (PRPP) + adenosine 5'-monophosphate
addition of diphosphate (PP) to ribose 5-phosphate to form 5'-Phospho-alpha-D-ribose 1-diphosphate (PRPP)
Carbohydrate metabolism
Metabolism of carbohydrates
Digestion of dietary carbohydrate
Glucose metabolism
Glycolysis
Gluconeogenesis
Glycogen synthesis
Glycogen breakdown (glycogenolysis)
Fructose catabolism
Galactose catabolism
Pentose phosphate pathway (hexose monophosphate shunt)
5-Phosphoribose 1-diphosphate biosynthesis
PRPP biosynthesis
Synthesis of cytosolic 5-phospho-alpha-D-ribose 1-diphosphate (PRPP) from D-ribose 5-phosphate
Hexose transport
Hexose uptake
Glucose transport
Regulation of Glucokinase by Glucokinase Regulatory Protein
Glycosaminoglycan metabolism
Hyaluronan metabolism
Hyaluronan biosynthesis and export
Hyaluronan uptake and degradation
Transport and synthesis of PAPS
Keratan sulfate/keratin metabolism
Keratan sulfate biosynthesis
Keratan sulfate degradation
Heparan sulfate/heparin (HS-GAG) metabolism
A tetrasaccharide linker sequence is required for GAG synthesis
HS-GAG biosynthesis
HS-GAG degradation
Chondroitin sulfate/dermatan sulfate metabolism
Chondroitin sulfate biosynthesis
Dermatan sulfate biosynthesis
CS/DS degradation