Additional features include facial dysmorphism (midface hypoplasia, internal strabism of the right eye, low-set ears, moderately high arched palate, small teeth), nephrotic syndrome, cardiac defects, and feeding problems.” Given the importance of ionic homeostasis for glycosylation and cardiomyocyte activity, this review will focus on the TMEM165 protein, highlighting its structure and function to emphasize the main properties in the context of cardiomyopathies associated with CDG. The gene discussed is TMEM165; the disease is congenital disorder of glycosylation.