Dissection of TMEM165 function in Golgi glycosylation and its Mn²⁺ sensitivity.

ABSTRACT: Since 2012, the interest for TMEM165 increased due to its implication in a rare genetic human disease named TMEM165-CDG (Congenital Disorder(s) of Glycosylation). TMEM165 is a Golgi localized protein, highly conserved through evolution and belonging to the uncharacterized protein family 0016 (UPF0016). Although the precise function of TMEM165 in glycosylation is still controversial, our results highly suggest that TMEM165 would act as a Golgi Ca²⁺/Mn²⁺ transporter regulating both Ca²⁺ and Mn²⁺ Golgi homeostasis, the latter is required as a major cofactor of many Golgi glycosylation enzymes. Strikingly, we recently demonstrated that besides its role in regulating Golgi Mn²⁺ homeostasis and consequently Golgi glycosylation, TMEM165 is se

SUBMITTER: Lebredonchel E 

PROVIDER: S-EPMC6857530 | biostudies-literature | 2019 Oct

REPOSITORIES: biostudies-literature