Neuronal function critically depends on coordinated delivery of properly modified ion channels, transporters and components of the synaptic apparatus at the appropriate rates and over long distances, to specific subcellular compartments. Remarkably, the localization of the synaptobrevin homolog Snc1 is altered in COG deficient yeast cells (Whyte and Munro, 2002). In addition, underglycosylated low density lipoprotein receptor is severely destabilized in CHO cells deficient for COG1 or COG2 proteins (Kingsley et al., 1986). Since glycosylation of channels, transporters and transport regulators is essential for their correct delivery, stability and/or activity (Gong et al., 2002; Watanabe et al., 2004; Scott and Panin, 2014), it is reasonable to predict that a majority of underglycosylated ion channels and transporters may similarly be destabilized, thus altering the functionality of COG-CDG patient neurons.