Table 2.

Phylogenetic distribution of COG complex subunits and pathology/glycosylation defects associated with mutations/deletions of COG complex subunits

Budding yeasts S. cerevisiae	Worms C. elegans	Fruit fly D. melanogaster	Humans mammals	Pathology/glycosylation defects
Cog1 Sec36 Cod3	Cogc-1 Mig30	dCog1	Cog1 ldlB	CDG-II/Cog1 human disorder.53 Sialylation and galactosylation defects. Decrease in sugar-transporters and glycosyltransferases. In Chinese hamster ovary ldlB cells, defects in sialylation and galactosylation (O- and N-linked glycans).16,50,60 Man II is mislocalized.42 In C. elegans, Cogc-1 mutation causes dysglycosylation of Mig-17 (ADAM protease) with gonadal dysmorphia.48 Glycosylation defects in yeast cells lacking COG1.15,18,21
Cog2 Sec35	Cogc-2	dCog2	Cog2 ldlC	In Chinese hamster ovary ldlC cells, defects in sialylation and galactosylation (O- and N-linked glycans).16,47,60 Man II is mislocalized.42 Glycosylation defects in yeast cells with mutant COG215,18
Cog3 Sec34 Grd20	Cogc-3 Mig29	dCog3	Cog3 hSec34	In siRNA-induced COG3 knockdown HeLa cells both GlcNAcT1 and Man II are mislocalized leading to dysglycosylation of Lamp2, and CD44.41 In C. elegans, Cogc-3 mutation causes dysglycosylation of Mig-17 (ADAM protease) with gonadal dysmorphia.48 Glycosylation defects in yeast cells with mutant COG3.15,18 Golgi mammosyltransferase Och1p is mislocalized.58
Cog4 Sec38 Cod1 Sgf1	Cogc-4	dCog4	Cog4 Cod1	Glycosylation defects in yeast cells lacking COG4.18
Cog5 Cod4	Cogc-5	dCog5 fws	hCog5 GTC-90	In shRNA-induced COG5 knockdown HeLa cells LDLR is slightly dysglycosylated. Cells were stained with PNA lectin, indicating sialylation defect.28 In Drosophila, defects in male spermatozoa (multiple nuclei and non-functional acroblast).46
Cog6 Sec37 Cod2	Cogc-6	dCog6	Cog6 hCod2	Glycosylation defects in yeast cells lacking COG6.15,18,21
Cog7 Cod5	Cogc-7	dCog7	Cog7	CDG-IIe human disorder. Sialylation and galactosylation defects. Decrease in sugar-transporters and glycosyltransferases.51,54,55,61 In siRNA-induced COG7 knockdown HeLa cells both GlcNAcT1 and ManII are mislocalized leading to dysglycosylation of Lamp2, and CD44.41
Cog8 Dor1	Cogc-8	dCog8	Cog8 hDor1	CDG-IIh (CDG-II/COG8) human disorder. Patient’s fibroblasts stain with PNA lectin, indicating sialylation defects. Abnormal N-glycosylation in serum.56,57 Glycosylation defects in yeast cells lacking COG815