Pathway
Synthesis of substrates in N-glycan biosythesis
Reactions for the synthesis of the small nucleotide-linked sugar substrates that are used in the synthesis of the N-glycan precursor and in the later steps of glycosylation are annotated here.
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All these nucleotide-linked sugar donors are synthesized in the cytosol; however, to participate in the later reactions of N-glycan precursor biosynthesis (when the glycan is oriented toward the lumen of the endoplasmic reticulum (ER)), these substrates must be attached to a dolichyl-phosphate molecule and then flipped toward the luminal side of the ER, through a mechanism which is still not known but which involves a different protein than the one that mediates the flipping of the LLO itself. Two of the genes encoding enzymes involved in these reactions, MPI and PMM2, are known to be associated with Congenital Disorders of Glycosylation (CDG) diseases of type I. Of these, CDG-Ia, associated with defects in PMM2, is the most frequent CDG disease reported.
REACT:R-HSA-446219Reactome
What the reference databases state
Statements
65 statements
65 statements · 1 reference database · Subject: Synthesis of substrates in N-glycan biosythesis · Sources: Reactome
- ALG5 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- AMDHD2 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- CMAS participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- CTSA participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- DHDDS participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- DHRSX participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- DOLK participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- DOLPP1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- DPM1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- DPM2 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- DPM3 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- FCSK participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
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- FPGT participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- FUOM participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- GFPT1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- GFPT2 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- GFUS participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- GLB1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- GMDS participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- GMPPA participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- GMPPB participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- GNE participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- GNPNAT1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- HK1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- MPI participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- MVD participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- NAGK participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- NANP participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- NANS participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- NEU1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- NEU2 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- NEU3 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- NEU4 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- NPL participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- NUDT14 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- NUS1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- PGM3 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- PMM1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- PMM2 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- RENBP participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- SLC17A5 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- SLC35A1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- SLC35C1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- SRD5A3 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST3GAL1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST3GAL2 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST3GAL3 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST3GAL4 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST3GAL5 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST3GAL6 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST6GAL1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST6GAL2 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST6GALNAC1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST6GALNAC2 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST6GALNAC3 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST6GALNAC4 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST6GALNAC5 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST6GALNAC6 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST8SIA1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST8SIA2 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST8SIA3 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST8SIA4 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST8SIA5 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- ST8SIA6 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only
- UAP1 participates in Synthesis of substrates in N-glycan biosythesisProteinReactome R-HSA-446219Inferred only