AGM1
PGM3
UniProt:O95394 PGM3
GCNT3
UniProt:O95395 GCNT3
MCD4
PIGN
UniProt:O95427 PIGN
FUR
FURIN
PACE
PCSK3
UniProt:P09958 FURIN
SIAT1
ST6GAL1
UniProt:P15907 ST6GAL1
GGNT1
GLCT1
GLYT1
MGAT
MGAT1
UniProt:P26572 MGAT1
MPI
PMI1
UniProt:P34949 MPI
GGCX
UniProt:P38435 GGCX
GGNT3
MGAT3
UniProt:Q09327 MGAT3
GGNT5
MGAT5
UniProt:Q09328 MGAT5
MGAT2
UniProt:Q10469 MGAT2
CGS23
NANTA3
SIAT4C
ST3GAL4
STZ
UniProt:Q11206 ST3GAL4
GCS1
MOGS
UniProt:Q13724 MOGS
ALG11
GT8
UniProt:Q2TAA5 ALG11
MANEA
UniProt:Q5SRI9 MANEA
PGAP1
UniProt:Q75T13 PGAP1
PIGW
UniProt:Q7Z7B1 PIGW
DOLPP1
LSFR2
UniProt:Q86YN1 DOLPP1
FGE
PSEC0152
SUMF1
UniProt:Q8NBK3 SUMF1
PIGB
UniProt:Q92521 PIGB
ALG3
NOT
NOT56L
UniProt:Q92685 ALG3
RFT1
UniProt:Q96AA3 RFT1
MSTP134
MSTP576
UniProt:Q9BQB6 VKORC1
VKOR
VKORC1
ALG1
HMAT1
HMT1
PSEC0061
UniProt:Q9BT22 ALG1
ALG12
UniProt:Q9BV10 ALG12
ALG8
HUSSY-02
UniProt:Q9BVK2 ALG8
FUT8
UniProt:Q9BYC5 FUT8
DPAGT1
DPAGT2
UniProt:Q9H3H5 DPAGT1
ALG2
UniProt:Q9H553 ALG2
ALG9
DIBD1
UniProt:Q9H6U8 ALG9
UniProt:Q9H9G5 cDNA FLJ12769 fis, clone NT2RP2001581
cDNA FLJ12769 fis, clone NT2RP2001581
PREB
SEC12
UniProt:Q9HCU5 PREB
PIGV
UniProt:Q9NUD9 PIGV
SIAT7B
SIATL1
ST6GALNAC2
STHM
UniProt:Q9UJ37 ST6GALNAC2
MAN1B1
UniProt:Q9UKM7 MAN1B1
DOLK
KIAA1094
TMEM15
UniProt:Q9UPQ8 DOLK
PIGL
UniProt:Q9Y2B2 PIGL
ALG6
My046
UniProt:Q9Y672 ALG6
ALG5
HSPC149
UniProt:Q9Y673 ALG5
Sar1p Activation And Membrane Binding
Coat Assembly
Loss of Sar1b GTPase
Cargo, Sec31p:Sec13p, and v-SNARE recruitment
Vesicle Budding
Vesicle Uncoating
vitamin K epoxide -> vitamin K hydroquinone
pro-factor IX, uncarboxylated + 12 CO2 + 12 O2 + 12 vitamin K hydroquinone -> pro-factor IX + 12 H2O + 12 vitamin K epoxide
pro-factor VII, uncarboxylated + 10 CO2 + 10 O2 + 10 vitamin K hydroquinone -> pro-factor VII + 10 H2O + 10 vitamin K epoxide
pro-factor X, uncarboxylated + 11 CO2 + 11 O2 + 11 vitamin K hydroquinone -> pro-factor X + 11 H2O + 11 vitamin K epoxide
pro-prothrombin, uncarboxylated + 10 CO2 + 10 O2 + 10 vitamin K hydroquinone -> pro-prothrombin + 10 H2O + 10 vitamin K epoxide
pro-protein C, uncarboxylated + 8 CO2 + 8 O2 + 8 vitamin K hydroquinone -> pro-protein C + 8 H2O + 8 vitamin K epoxide
pro-protein S, uncarboxylated + 11 CO2 + 11 O2 + 11 vitamin K hydroquinone -> pro-protein S + 11 H2O + 11 vitamin K epoxide
pro-protein Z, uncarboxylated + 13 CO2 + 13 O2 + 13 vitamin K hydroquinone -> pro-protein Z + 13 H2O + 13 vitamin K epoxide
pro-GAS6, uncarboxylated + 11 CO2 + 11 O2 + 11 vitamin K hydroquinone -> pro-GAS6 + 11 H2O + 11 vitamin K epoxide
Pro-factor IX is transported from the endoplasmic reticulum to the Golgi apparatus
Pro-factor VII is transported from the endoplasmic reticulum to the Golgi apparatus
Pro-factor X is transported from the endoplasmic reticulum to the Golgi apparatus
Pro-prothrombin is transported from the endoplasmic reticulum to the Golgi apparatus
Pro-protein C is transported from the endoplasmic reticulum to the Golgi apparatus
Pro-protein S is transported from the endoplasmic reticulum to the Golgi apparatus
Pro-protein Z is transported from the endoplasmic reticulum to the Golgi apparatus
Pro-GAS6 is transported from the endoplasmic reticulum to the Golgi apparatus
pro-factor IX -> factor IX + factor IX propeptide
pro-factor VII -> factor VII + factor VII propeptide
pro-factor X -> factor X + factor X propeptide
pro-prothrombin -> prothrombin + prothrombin propeptide
pro-protein C -> protein C + protein C propeptide
pro-protein S -> protein S + protein S propeptide
pro-protein Z -> protein Z + protein Z propeptide
pro-GAS6 -> GAS6 + GAS6 propeptide
EIF5A + spermidine <=> EIF5A(Dhp) + 1,3-diaminopropane
EIF5A(Dhp) + 1,3-diaminopropane <=> EIF5A + spermidine
EIF5A(Dhp) + O2 => EIF5A(Hyp)
SUMF1 mediates the oxidation of cysteine to formylglycine, producing active arylsulfatases
SUMF2 inhibits SUMF1-mediated activation of arylsulfatases
dolichyl phosphate + GDP-alpha-D-mannose -> dolichyl phosphate D-mannose
Reorientation of dolichyl phosphate D-mannose in the endoplasmic reticulum membrane
phosphatidylinositol + UDP-N-acetyl-D-glucosamine -> N-acetylglucosaminyl-PI + UDP
N-acetylglucosaminyl-PI + H2O -> glucosaminyl-PI + acetate
Reorientation of glucosaminyl-acyl-PI in the endoplasmic reticulum membrane
glucosaminyl-PI + fatty acyl-CoA -> glucosaminyl-acyl-PI + CoA-SH
glucosaminyl-acyl-PI + dolichol phosphate D-mannose -> mannose(al1-4)glucosaminyl-acyl-PI + dolichol phosphate
mannose(a1-4)glucosaminyl-acyl-PI + phosphatidylethanolamine -> (ethanolamineP) mannose(al1-4)glucosaminyl-acyl-PI + diacylglycerol
(ethanolamineP) mannose (a1-4) glucosaminyl-acyl-PI + dolichol phosphate D-mannose -> mannose (a1-6) (ethanolamineP) mannose (a1-4) glucosaminyl-acyl-PI + dolichol phosphate
mannose (a1-6) (ethanolamineP) mannose (a1-4) glucosaminyl-acyl-PI + dolichol phosphate D-mannose -> mannose (a1-2) mannose (a1-6) (ethanolamineP) mannose (a1-4) glucosaminyl-acyl-PI + dolichol phosphate
mannose (a1-2) mannose (a1-6) (ethanolamineP) mannose (a1-4) glucosaminyl-acyl-PI + phosphatidylethanolamine -> (ethanolamineP) mannose (a1-2) mannose (a1-6) (ethanolamineP) mannose (a1-4) glucosaminyl-acyl-PI (acyl-GPI) + diacylglycerol
mannose (a1-2) mannose (a1-6) (ethanolamineP) mannose (a1-4) glucosaminyl-acyl-PI -> mannose (a1) mannose (a1-2) mannose (a1-6) (ethanolamineP) mannose (a1-4) glucosaminyl-acyl-PI
(ethanolamineP) mannose (a1-2) mannose (a1-6) (ethanolamineP) mannose (a1-4) glucosaminyl-acyl-PI -> (ethanolamineP) mannose (a1-2) (ethanolamineP) mannose (a1-6) (ethanolamineP) mannose (a1-4) glucosaminyl-acyl-PI
uPAR precursor + acyl-GPI -> uPAR-acyl-GPI + uPAR propeptide
uPAR-acyl-GPI + H2O -> uPAR + long-chain fatty acid
Phosphorylation of dolichol
Dephosphorylation of dolichyl diphosphate
Formation of glucosamine 6-phosphate from fructose 6-phosphate and glutamine
Acetylation of glucosamine 6-phosphate to GlcNAc6P
Isomerization of GlcNAc6P to GlcNAc1P
GlcNAc1P is dephosphorylated to UDP-N-acetyl-glucosamine
Fructose 6-phosphate isomerizes to Mannose 6-phosphate
Mannose 6-phosphate isomerizes to Mannose 1-phosphate
Mannose-1-phosphate converted to GDP-Mannose
Synthesis of dolichyl-phosphate-glucose
Flipping of dolichyl-phosphate-glucose into the ER lumen
Addition of N-acetyl-D-glucosamine to Dolichyl phosphate
Addition of the second N-acetyl-glucosamine to the N-glycan precursor
Addition of the first mannose to the N-glycan precursor by ALG13/14
Addition of a second mannose to the N-glycan precursor by ALG2
Addition of a third mannose to the N-glycan precursor by ALG2
Addition of a fourth and fifth mannose to the N-glycan precursor by ALG11
Flipping of the N-glycan precursor to inside the ER
Addition of the sixth mannose to the N-glycan precursor by ALG3.
Addition of the seventh mannose to the N-glycan precursor by ALG9, inside the ER lumen
Addition of the eighth mannose to the N-glycan precursor by ALG12, inside the ER lumen
Addition of the last mannose to the N-glycan precursor by ALG3, inside the ER lumen.
Addition of the first glucose to the N-glycan precursor by ALG6
Addition of a second glucose to the N-glycan precursor by ALG8
Addition of a third glucose to the N-glycan precursor by an ALG10 homologue
Transfer of N-glycan to the protein
Trimming of the first glucose by glucosidase I
Binding of Malectin
Removal of the second glucose by glucosidase II
Binding of calnexin/calreticulin to the unfolded protein
Binding of ERp57
Removal of the third glucose by glucosidase II and release from the chaperone
Incorrectly folded glycoproteins translocate to the ERQC
Trimming of the terminal mannose on the B branch
Trimming of the terminal mannose on the C branch
Trimming of the first mannose on the A branch
Trimming of the second mannose on the A branch
Re-addition of a glucose on the alpha 1,3 branch
Glycoproteins with major folding defects are sent to degradation by EDEMs
Glycoproteins with lesser folding defects get transported back to the ER and the CNX/CRT complex
Correctly folded glycoproteins translocate to the Golgi
Transport of glycoproteins with Man8 (or Man9) N-glycans to the Golgi
Alternative endo-mannosidase I route
Progressive trimming of alpha-1,2-linked mannose residues from Man9GlcNAc2 to produce Man5GlcNAc2
Progressive trimming of alpha-1,2-linked mannose residues from Man8GlcNAc2 to produce Man5GlcNAc2
Progressive trimming of alpha-1,2-linked mannose residues from Man7GlcNAc2 to produce Man5GlcNAc2
Addition of GlcNAc to the glycan on the A arm
Addition of a bifurcating GlcNAc to the N-glycan by MGAT3
Trimming of mannoses on the alpha1,6 arm by MAN2A1
Addition of a GlcNAc on the alpha 1,4 branch by MGAT2
Addition of alpha 1,6 fucose to the first GlcNAc of the N-glycan
Addition of GlcNAc to position 4 by N-acetylglucosaminyltransferase (GnT)-IV
Addition of GlcNAc to position 5 by MGAT5
Addition of galactose by beta 4-galactosyltransferases
Addition of alpha-2,6-sialic acid to terminal galactose of N-glycans by ST6GAL1
Addition of alpha-2,8-sialic acid to N-glycan over a galactose by ST8SIAs
Addition of alpha-2,3-sialic acid to N-glycan over a galactose by ST3GAL4
Addition of GalNAc to mucins to form the Tn antigen
C1GALT1 transfers galactose to the Tn antigen forming Core 1 glycoproteins (T antigens)
Addition of GlcNAc to the T antigen forms a Core 2 glycoprotein
Addition of GlcNAc to the Tn antigen forms a Core 3 glycoprotein
Addition of GlcNAc to Core 3 forms a Core 4 glycoprotein
Addition of GlcNAc to the Tn antigen via an alpha-1,3 linkage forms a Core 5 glycoprotein
Addition of GlcNAc to the Tn antigen via a beta-1,6 linkage forms a Core 6 glycoprotein
Addition of galactose to Core 6 glycoprotein
Addition of GalNAc to the Tn antigen via an alpha-1,6 linkage forms a Core 7 glycoprotein
Addition of galactose to the Tn antigen via an alpha-1,3 linkage forms a Core 8 glycoprotein
Sialyltransferase I can add sialic acid to the Tn antigen at the alpha 6 position
Sialyltransferase I can add sialic acid to the T antigen at the alpha 6 position
GalNAc alpha-2,6-sialyltransferase II can add a sialic acid to the T antigen at the alpha 6 position
ST3GAL1-4 can add a sialic acid to the T antigen at the alpha 3 position
ST6GALNAC3/4 can add a sialic acid to the sialyl T antigen to form the disialyl T antigen
ER to Golgi Transport
COPII (Coat Protein 2) Mediated Vesicle Transport
Post-translational protein modification
PTM: gamma carboxylation, hypusine formation and arylsulfatase activation
Gamma-carboxylation, transport, and amino-terminal cleavage of proteins
Gamma-carboxylation of protein precursors
Transport of gamma-carboxylated protein precursors from the endoplasmic reticulum to the Golgi apparatus
Removal of aminoterminal propeptides from gamma-carboxylated proteins
Hypusine synthesis from eIF5A-lysine
Hypusinylation
The activation of arylsulfatases
Post-translational modification: synthesis of GPI-anchored proteins
Synthesis of dolichyl-phosphate mannose
Synthesis of glycosylphosphatidylinositol (GPI)
Attachment of GPI anchor to uPAR
Asparagine N-linked glycosylation
Biosynthesis of the N-glycan precursor (dolichol lipid-linked oligosaccharide, LLO) and transfer to a nascent protein
Synthesis of substrates in N-glycan biosythesis
Synthesis of Dolichyl-phosphate
Synthesis of UDP-N-acetyl-glucosamine
Synthesis of GDP-mannose
N-glycan trimming in the ER and Calnexin/Calreticulin cycle
Calnexin/calreticulin cycle
ER Quality Control Compartment (ERQC)
Transport to the Golgi and subsequent modification
N-glycan trimming and elongation in the cis-Golgi
Progressive trimming of alpha-1,2-linked mannose residues from Man9/8/7GlcNAc2 to produce Man5GlcNAc2
N-glycan antennae elongation in the medial/trans-Golgi
Reactions specific to the hybrid N-glycan synthesis pathway
Reactions specific to the complex N-glycan synthesis pathway
N-Glycan antennae elongation
O-linked glycosylation of mucins
Termination of O-glycan biosynthesis