Glycosaminoglycan metabolism (Homo sapiens)
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82, 97, 132
125
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40, 47, 84
5, 53, 60, 116, 126...
137, 154
53, 60, 138
2, 23, 115
143
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22, 67
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Golgi lumen
Golgi lumen
lysosomal lumen
cytosol
UDP-GlcNAc
OGN
Gal-Xyl-GPC2
aldehydo-L-iduronic
acid
CHST15
GlcA-Gal-Gal-Xyl-GPC2
VCAN
Heparan-GPC5
CMP
HS(5)-GPC5
Heparan-PGs
Gal-Gal-Xyl-GPC2
KS(2)-OMD
HS(6)-SDC1
(HA)50
UDP-GlcNAc
chondroitin(2)-BCAN
Gal-Xyl-NCAN
(HA)50
GPC2
B3GNT7
CHST13
aldehydo-L-iduronic
acid
GlcA
C6S-BCAN
GlcA-Gal-Gal-Xyl-BGN
GalNAc
PRELP
UDP-GlcA
p-CHP1
HS(4)-AGRN
Heparan(1)-GPC1
GPC2
HS(6)-SDC1
C4S-PG
KS(2)-LUM
XylS-SDC3
C4S-CSPG4
HS(6)-SDC4
HS(3)-GPC3
GUSB tetramer
HEXA
Gal-GlcNAc(S)-Gal-GlcNAc(S)-Gal
D4S-PGs
Heparan(3)-GPC3
Keratan(1)-KERA
Gal-Xyl-SDC2
GlcA-Gal-Gal-Xyl-VCAN
IDS dimer
HS/HPIN-PGs
GlcA-Gal-Gal-Xyl-proteins
HS(4)-GPC5
STAB2(1136-2551)
CHPF
H2O
dermatan-core proteins
UDP-GalNAc
Heparan(2)-GPC2
LUM
CHST6
Heparan(3)-HSPG2
Heparan(1)-PGs
Heparan(3)-GPC1
ChEBI:63515 chain
bHEXB
HS2ST1
CSE-BCAN
HS(3)-SDC2
ST3GAL1
B4GALT3
N-glycan PRELP
Heparan(1)-SDC2
CHST14 substrates
BCAN
Gal-GlcNAc(S)-Gal
KSPG(2)
Keratan(4)-ACAN
B4GALT4
UDP
GlcA-Gal-Gal-Xyl-DCN
dermatan-core proteins
HS(2)-SDC4
Heparan(4)-SDC1
chondroitin(1)-VCAN
CSE-BGN
HS(4)-SDC2
KS(1)-KERA
HS3ST1
ChEBI:63517 chain
UDP
HS(6)-GPC3
Gal-Gal-Xyl-SDC1
APS
L-Asp
Heparan(1)-SDC1
GlcNAc
HA:HAR:HYAL2
CSE-CSPG5
UDP
PAPS
GlcA-Gal-Gal-Xyl-SDC2
XylS-CSPG4
UDP
Heparan(1)-GPC2
HS(1)-GPC3
BGN
HARs
XylS-DCN
ABCC5
Gal-Gal-Xyl-SDC4
Gal-glycan KERA
HS core proteins
KS(2)-OGN
SLC9A1
Heparan-GPC6
H2O
HS(6)-HSPG2
H2O
B4GALT2
PAPS
HS(6)-GPC5
Ac-CoA
GlcA-Gal-Gal-Xyl-CSPG5
CSPGs
LYVE1
C6S chain
CHST14 products
HS/HPIN-PGs
B4GAT1
Heparan(1)-GPC4
Gal
GlcA-Gal-Gal-Xyl-SDC3
HPSE(158-543)
bHEXB
KS(2)-OGN
UDP
Keratan(3)-PG
PAPS
C6S-PGs
Heparan(3)-SDC4
HS(4)-GPC2
chondroitin(1)-BGN
HA polymer
HS(1)-HSPG2
HS(3)-PGs
GlcA-Gal-Gal-Xyl-CSPG5
CHST11
KS(2)-FMOD
BCAN
H2O
HS(5)-GPC6
Keratan(3)-LUM
Heparan(2)-PGs
HS(3)-AGRN
H2O
GlcNAc
PAP
Heparan-GPC4
GlcA-Gal-Gal-Xyl-GPC4
C6S-VCAN
Gal-Xyl-DCN
Heparan(3)-AGRN
SDC3
Gal-Xyl-AGRN
XYLT1
ST3GAL2
SDC3
HAS1,2,3
Heparan(3)-SDC3
D2,4(S)2-PG
PAP
HS(1)-PGs
KS(2)-LUM
Heparan-GPC1
HEXA
Mn2+
SO4(2-)
HEXA
HS(1)-SDC2
HS(6)-GPC4
HAS1
Ca2+
HS(5)-SDC4
H2O
KS(1)-FMOD
NCAN
KS(2)-KERA
PAPS
HS(2)-AGRN
HS(6)-GPC2
C4S-BCAN
CS/DS core proteins
Gal-Gal-Xyl-NCAN
H2O
HS(2)-GPC6
SO4(2-)
Keratan(4)-PRELP
KS(2)-OMD
C6S-NCAN
Heparan-SDC2
B4GALT1-6 homodimers
Ca2+
H2O
D2,4(S)2-CSPG5
H2O
GlcA-Gal-Gal-Xyl-GPC4
SDC4
HYAL1
CSGALNACT1
Ca2+
KSPG(1)
D4S-PGs
SLC26A1,2
HEXB(122-311)
HEXB(315-556)
HS(6)-SDC2
D2,4(S)2-VCAN
KS(2)-FMOD
Heparan(2)-HSPG2
Heparan sulfate
chain(7)
GPC3(25-?)
GlcA-Gal-Gal-Xyl-NCAN
STAB2(1136-2551)
AGRN(30-2045)
N-glycan KERA
ST3GAL1-4,6
GlcA-Gal-Gal-Xyl-SDC3
ST3GAL4
KS(2)-ACAN
UDP
HS3ST6
H+
SDC1
Heparan-GPC3
HEXB(315-556)
D4S-BCAN
Heparan(2)-GPC5
HS(6)-GPC2
Heparan-AGRN
CSPG5
Heparan sulfate
chain(6)
C6S-PG
Heparan(1)-HSPG2
Keratan(2)-PRELP
Gal-Xyl-CSPG5
HS3ST3A1
Gal-glycan-protein
SO4(2-)
HS(3)-GPC5
CHST2,5,6
B3GNT3
HS(4)-PGs
Gal-Gal-Xyl-GPC1
B3GAT3:Mn2+ dimer
Heparan(2)-SDC1
B3GAT3
Gal-glycan FMOD
chondroitin(1)-DCN
ATP
IDUA
GPC1
XylS-GPC1
Keratan(3)-ACAN
D2,4,4(S)3-PGs
CHEBI:63868 chain
HS3ST
sulfotransferases
CHST7
KS(2)-KERA
NCAN
Heparan(4)-GPC6
Heparan sulfate
chain(2)
HS(2)-SDC1
HS(6)-GPC1
DSE,DSEL substrates
IDUA
GPC1
D4S-DCN
HS3ST3B1
UDP-GlcNAc
PAPS
UDP-Gal
HS(6)-GPC1
ChEBI:63519 chain
Heparan(2)-GPC1
KS core proteins
UDP-Glc
CHPF,CHSY3
D4S-CSPG4
CH3COO-
HS(2)-HSPG2
KS(2)-PRELP
KS(2)-OMD
HS(3)-PGs
HS(3)-GPC2
NAGLU(59-743)
ChEBI:63516 chain
ADP
Keratan(3)-KERA
C6S chain
H2O
CSPGs
HS/HPIN-PGs
CSE-PG
KS(1)-OGN
UMP
Heparan(1)-GPC6
GlcA-Gal-Gal-Xyl-GPC6
GPC5(25-?)
Keratan(2)-FMOD
CSE-DCN
BGN
B3GALT6
Gal-Gal-Xyl-HSPG2
SO4(2-)
HS(5)-PGs
HS(6)-GPC6
Gal-Gal-Xyl-GPC5
XylS-SDC4
Gal-Gal-Xyl-AGRN
GlcA-Gal-Gal-Xyl-GPC3
UDP
C4S/C6S chains
UDP-GlcA
GPC6
GPC3(25-?)
HMMR
GlcA-Gal-Gal-Xyl-DCN
B3GNT2
SO4(2-)
GUSB
CHST14
Gal-Gal-Xyl-DCN
NDST2
D2,4(S)2-PGs
PAPSS2
HS(4)-GPC4
SLC35B3
GLCE
HS(1)-SDC4
DSE,DSEL products
EXT1:EXT2
Heparan(2)-SDC2
Heparan(4)-SDC4
NDST1
Keratan(4)-PG
Heparan(1)-SDC3
Gal-Xyl-proteins
HS(6)-GPC6
Heparan(3)-GPC5
chondroitin(3)-CSPG5
HS(4)-SDC4
C4S-BGN
Gal-Xyl-GPC3
Keratan(1)-PG
HS(4)-GPC1
HS(4)-SDC3
C4S-DCN
PAPS
chondroitin(1)-core
proteins
GlcA-Gal-Gal-Xyl-BCAN
CHSY3
C6S-CSPG5
Heparan(3)-GPC6
KS(2)-KERA
D4S-PGs
Keratan(2)-ACAN
SGSH
CHST9-2
Keratan(1)-LUM
HS(6)-HSPG2
HS(3)-PGs
Heparan(2)-GPC4
HAS3
HS3ST5
HEXA
D2,4(S)2-PG
HEXB(315-556)
Asparagine N-linked
glycosylation
HS(6)-SDC3
SDC2
GlcA-Gal-Gal-Xyl-SDC1
UDP-GlcNAc
Heparan-HSPG2
GlcA-Gal-Gal-Xyl-CS
proteins
Heparan(1)-GPC3
HS(3)-HSPG2
C6S-BGN
Heparan sulfate
chain(5)
B4GALT6
SDC1
OxA-GNS
NDST3
Gal-Xyl-CSPG4
HS/HPIN-PGs
OxA-ARSB
D2,4(S)2-CSPG4
chondroitin(2)-VCAN
Heparan sulfate
chain
Gal-Gal-Xyl-CSPG4
UDP-GlcNAc, UDP-Glc
SDC4
Keratan(4)-OMD
HPSE2(1-592)
UDP
CSPG5
HS(5)-GPC4
HS(6)-GPC1
Chondroitin chain
UDP-Gal
IDS(456-550)
Gal-Gal-Xyl-CSPG5
B4GALT7
HPSE(36-109)
Keratan(2)-LUM
KS(2)-OGN
OMD
HEXA
Gal-glycan ACAN
PAP
Gal-Gal-Xyl-SDC2
HYAL2
KS(2)-ACAN
Heparan(2)-SDC3
UDP-GlcA
HS6ST2
H2O
GAG core proteins
EXT2
Heparan-GPC2
KS(2)-PRELP
HS(5)-GPC1
KS(2)-PRELP
HYAL1-like proteins
UDP-Gal
HSPG2(22-4391)
GlcNAc
GlcA-Gal-Gal-Xyl-CSPG4
H2O
HEXB(315-556)
Keratan(3)-FMOD
Gal-Xyl-BGN
Heparan chain(2)
HSPGs
chondroitin(2)-CSPG4
C4S-CSPG5
D2,4(S)2-PG
N-glycan ACAN
GPC6
ST3GAL6
Chondroitin chain
H2O
CHSY3
SGSH
HS(6)-SDC4
PAPSS1,2
SDC2
HS(4)-PGs
Gal-Xyl-SDC4
C6S-PG
DSPGs
CHST2
HS(4)-PGs
HS(2)-GPC4
beta-xylosidase
ChEBI:63515 chain
HS(5)-PGs
chondroitin(1)-BCAN
KS(1)-PRELP
PPi
FMOD
XylS-BCAN
UDP-GalNAc
UDP-Gal
AGRN(30-2045)
CSPGs
CMP-Neu5Ac
Heparan(4)-GPC1
HS(6)-SDC2
UDP-GlcNAc
UDP
D4S-PGs
XylS-GPC2
CHPF2
B3GNT4
Heparan(3)-SDC1
Gal-Xyl-GPC6
N-glycan LUM
CSE-VCAN
IdoA-GalNAc(4S)-GlcA-Gal-Gal-Xyl
GlcA-Gal-Gal-Xyl-BCAN
D2,4(S)2-PGs
p-CHP1
Gal-Xyl-BCAN
C4S chain
KS(1)-OMD
D4S-PGs
H2O
GlcA
CSE-PG
D2,4,4(S)3-PGs
B3GAT2
Gal-Xyl-GPC5
GlcNAc
CHPF
HS(1)-SDC1
HS(2)-SDC2
UDP-Gal
Ac-CoA
D4S-VCAN
HS(1)-GPC4
HYAL1-like proteins
SDC1
NDSTs
CHST12
XYLT2
HA
HSPGs
Heparan(1)-AGRN
HS(2)-PGs
Heparan(4)-AGRN
D2,4,4(S)3-BCAN
CHST3,7
H2O
HS(1)-GPC2
D2,4,4(S)3-DCN
HS(6)-AGRN
GlcA-Gal-Gal-Xyl-AGRN
CHST3
HS3ST2
B4GALT5
HS(6)-AGRN
Heparan chain(1)
HS(2)-GPC3
XylS-VCAN
DSEL
Heparan sulfate
chain(4)
Gal-Xyl-VCAN
HS(5)-PGs
CSGALNACT
Mn2+
C4S-NCAN
HA:HAR:HYAL2:SLC9A1:pCHP:Ca2+
Heparan(4)-HSPG2
PAP
HYAL1
XylS-NCAN
HYAL2
Gal-Gal-Xyl-GPC6
N-glycan FMOD
Gal-Xyl-SDC3
Keratan(4)-OGN
D2,4,4(S)3-VCAN
SDC3
CoA-SH
Heparan(4)-GPC4
Gal-Xyl-HSPG2
Gal-glycan PRELP
Heparan(3)-PGs
C6S-CSPG4
C4S-PG
GALNS oligomer
D2,4,4(S)3-PGs
chondroitin(3)-core
proteins
GLB1
GlcA-Gal-Gal-Xyl-HSPG2
KSPG(2)
HS(5)-SDC3
XylS-GPC6
UDP
UDP
HS(3)-GPC4
Keratan(1)-OGN
H2O
D2,4,4(S)3-BGN
linker chain(2)
N-glycan OMD
D2,4,4(S)3-PGs
(HA)2
Heparan(2)-SDC4
keratan
sulfate
1,4-beta-D-galactosidase
C4S-PG
KERA
KS(1)-LUM
HS(3)-GPC6
H2O
HS6ST3
GlcA-Gal-Gal-Xyl-GPC2
GlcA-Gal-Gal-Xyl-GPC1
chondroitin(1)-CSPG5
chondroitin(3)-BCAN
BGAL
GPC5(25-?)
HS/HPIN-PGs
GPC3(25-?)
GlcA-Gal-Gal-Xyl-BGN
chondroitin(3)-BGN
UDP
GPC5(25-?)
Heparan-SDC1
PAP
DSE
CHST1
C4S-VCAN
HS(6)-GPC4
HS(4)-GPC3
GlcNAc-GlcA-GlcNAc
HS6ST1
SO4(2-)
XylS-HSPG2(22-4391)
HS(5)-SDC2
Ca2+
Heparan(2)-GPC3
Gal-Gal-Xyl-GPC4
XylS-SDC2
xylosyl-core
proteins
CSE-PG
GPC6
GlcA-Gal-Gal-Xyl-SDC1
HSPG2(22-4391)
CSPG4
SLC35B2
Keratan(3)-OGN
Heparan(4)-SDC2
HEXB(315-556)
UDP-GlcA
ST3GAL3
DSPGs
Keratan(1)-FMOD
Heparan(4)-GPC2
Heparan(2)-AGRN
chondroitin(3)-NCAN
HS(6)-GPC6
Gal
Heparan(4)-PGs
B3GAT3
GlcA-Gal-Gal-Xyl-VCAN
PAP
HS(6)-GPC2
PAPS
ACAN
Keratan(2)-PG
HS(1)-SDC3
D2,4,4(S)3-NCAN
HS3ST4
GPC4
HEXB(122-311)
Heparan-SDC3
VCAN
Heparan(4)-SDC3
Gal-Gal-Xyl-proteins
bHEXB
chondroitin(1)-CSPG4
H2O
Keratan(4)-FMOD
Heparan(4)-GPC3
CSE-PGs
HS(5)-AGRN
CSGALNACT2
HS3STs
Keratan(2)-OGN
HS(5)-HSPG2
KS(2)-FMOD
HS core proteins
XYLT1, XYLT2
XylS-CSPG5
HPSE dimer
DSPGs
LYVE1
UST
UDP
GPC1
D4S-CSPG5
PAP
PAP
H2O
GlcA-b1,3-GlcNAc
HYAL2
HS(4)-HSPG2
Gal(S)-GlcNAc(S)-Gal-GlcNAc(S)-Gal
HEXB(122-311)
BGAL
SDC4
PAP
HEXB(122-311)
PAPSS1
GlcA-b1,3-GlcNAc
C4S-PGs
Heparan(1)-SDC4
HS3ST1
GlcNAc-Gal-GlcNAc(S)-Gal
GlcA-β1,3-GlcNAc
DSE,DSEL
HS(1)-AGRN
HS(3)-GPC1
GlcA-Gal-Gal-Xyl-GPC6
SLC26A1
D2,4(S)2-NCAN
GlcA-Gal-Gal-Xyl-HSPG2
H+
chondroitin(3)-core proteins
GLB1L
GLB1
HEXA
CD44
HS(6)-GPC5
PAP
H2O
D2,4,4(S)3-PGs
B4GALT1
SO4(2-)
Gal-Xyl-GPC4
D4S-NCAN
CS/HS precursor
HS(2)-PGs
HS(2)-GPC5
H2O
HMMR
HSPG2(22-4391)
Keratan(2)-OMD
IDS(34-455)
D4S-BGN
ARSB:Ca2+
C6S-DCN
CHSY1
D2,4(S)2-BGN
GlcA-Gal-Gal-Xyl-AGRN
HS(6)-SDC3
Gal-Xyl-SDC1
Gal-Gal-Xyl-VCAN
GPC4
Keratan(4)-LUM
B3GNT1,2,3,4,7
GlcA-Gal-Gal-Xyl-GPC3
HS(6)-AGRN
Keratan(1)-ACAN
PAPS
Gal-Gal-Xyl-SDC3
uridine
5'-monophosphate
Gal-Gal-Xyl-BGN
Heparan(1)-GPC5
CD44
CEMIP
Chondroitin chains
HA
HS(5)-GPC3
D4S-PGs
HA
GlcNAc(S)-Gal-GlcNAc(S)-Gal
HS(3)-SDC1
Gal-Gal-Xyl-GPC3
CHST9,11,12,13
CoA-SH
DCN
XylS-GPC4
PAPS
Keratan(1)-OMD
XylS-GPC3(25-?)
CHPF,CHPF2,CHSY3
GalNAc
H2O
GlcA-Gal-Gal-Xyl-GPC5
CSE-CSPG4
GlcA-Gal-Gal-Xyl-SDC4
Heparan(4)-GPC5
CSPG4
Gal-glycan OGN
SLC9A1
GPC4
NAGLU(59-743)
HYAL3
Heparan sulfate
chain(1)
UDP-Xyl
HAS2
HMMR
HYAL3
GlcA-Gal-Gal-Xyl-SDC4
KSPG(2)
chondroitin(2)-core
proteins
GlcA-Gal-Gal-Xyl-NCAN
HS(6)-GPC3
HS(3)-SDC4
chondroitin(3)-DCN
C4S chain
STAB2(1136-2551)
HS(2)-PGs
chondroitin(2)-DCN
UDP
NDST4
chondroitin(1)-NCAN
Gal-glycan LUM
D-xylose
HS(1)-GPC1
N-glycan-protein
Gal-Xyl-GPC1
HGSNAT oligomer
chondroitin(2)-BGN
HEXB(315-556)
GlcA-Gal-Gal-Xyl-HS
proteins
Gal-glycan OMD
XylS-GPC5(25-?)
SLC26A2
Keratan(3)-PRELP
HS(4)-GPC6
XylS-SDC1
PAPS
KS(1)-ACAN
AGRN(30-2045)
HS(1)-GPC6
B3GAT1
HS(2)-SDC3
HS(6)-SDC1
H2O
C6S-PG
C4S-PG
DCN
HS(2)-GPC2
CHST5
HS(6)-GPC5
H2O
GlcA-Gal-Gal-Xyl-SDC2
HS(5)-SDC1
Gal-Gal-Xyl-BCAN
H2O
GPC2
KS(2)-LUM
Keratan(2)-KERA
ATP
HS(6)-SDC4
HS(6)-HSPG2
HS(6)-GPC4
HS(2)-GPC1
EXT1
HS(1)-GPC5
SO4(2-)
chondroitin(3)-CSPG4
SLC35B2,3
GlcA
Keratan(1)-PRELP
D2,4(S)2-BCAN
GLB1L
Heparan(3)-SDC2
HS(3)-SDC3
Keratan(4)-KERA
HEXB(122-311)
N-glycan OGN
XylS-BGN
chondroitin(2)-CSPG5
D2,4,4(S)3-CSPG4
B3GAT dimers
chondroitin(2)-NCAN
Heparan(2)-GPC6
HS(6)-GPC3
CD44
Heparan(3)-GPC2
SDC2
CSE-NCAN
LYVE1
HS3ST5
HS(6)-SDC3
Heparan(3)-GPC4
UDP-GlcNAc, UDP-Glc
Keratan(3)-OMD
GlcA-Gal-Gal-Xyl-CSPG4
UDP
GlcA-Gal-Gal-Xyl-GPC5
SLC35D2
Heparan-SDC4
GlcA-Gal-Gal-Xyl-GPC1
SLC9A1:p-CHP:Ca2+
UDP-Glc
D2,4,4(S)3-CSPG5
XylS-AGRN(30-2045)
HEXB(122-311)
Heparan sulfate
chain(3)
HS(5)-GPC2
D2,4(S)2-DCN
HS(6)-SDC2
HS6STs
chondroitin(3)-VCAN
HS(1)-PGs
HS(1)-PGs
PAPS
HS(4)-SDC1
KS(2)-ACAN
54, 88, 90, 150