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ProteomicsA pseudoautosomal glycosylation disorder prompts the revision of dolichol biosynthesis
Dolichol is a lipid critical for N-glycosylation as a carrier for activated sugars and nascent oligosaccharides. It is commonly thought to be directly produced from polyprenol by the enzyme SRD5A3. Instead, we found that dolichol synthesis requires a three-step detour involving additional metabolite...
ORGANISM(S): Homo sapiens (Human) 
2024-06-12 | PXD049174 | Pride
Lipid droplets Congenital disorders of glycosylation Polyprenal N-glycosylation Dolichal Pseudoautosomal region Dolichol Polyprenol 
ProteomicsAbsence of the dolichol synthesis gene DHRSX leads to N-glycosylation defects in Lec5 and Lec9 Chinese hamster ovary cells
Glycosylation-deficient Chinese hamster ovary (CHO) cell lines have been instrumental in the discovery of N-glycosylation machinery. Yet, the molecular causes of the glycosylation defects in the Lec5 and Lec9 mutants have been elusive, even though for both cell lines a defect in dolichol formation f...
ORGANISM(S): Cricetulus griseus (Chinese hamster) (Cricetulus barabensis griseus) 
2024-10-14 | PXD052706 | Pride
Congenital disorders of glycosylation Lec9 Dhrsx N-glycosylation Dolichal Polyprenal Lec5 Polyprenol Dolichol 
UnknownA pseudoautosomal glycosylation disorder prompts the revision of dolichol biosynthesis.
Not available
S-EPMC11250103 | biostudies-literature
UnknownFrom glycosylation disorders to dolichol biosynthesis defects: a new class of metabolic diseases.
Not available
S-EPMC3137772 | biostudies-literature
UnknownDolichol-linked oligosaccharide selection by the oligosaccharyltransferase in protist and fungal organisms.
Not available
S-EPMC2064103 | biostudies-literature
UnknownCharacterization of age-related dolichol increases in the retina of the C57BL/6 mouse.
Not available
S-EPMC12749842 | biostudies-literature
UnknownMetabolically programmed quality control system for dolichol-linked oligosaccharides.
Not available
S-EPMC3845098 | biostudies-literature
UnknownDeficiency of GDP-Man:GlcNAc2-PP-dolichol mannosyltransferase causes congenital disorder of glycosylation type Ik.
Not available
S-EPMC1182261 | biostudies-literature
UnknownA predicted geranylgeranyl reductase reduces the ω-position isoprene of dolichol phosphate in the halophilic archaeon, Haloferax volcanii.
Not available
S-EPMC3340491 | biostudies-literature
UnknownStereoselective transbilayer translocation of mannosyl phosphoryl dolichol by an endoplasmic reticulum flippase.
Not available
S-EPMC2895134 | biostudies-literature
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