Over 12 NGLY1 deficiency-causing variants are known to reside within the highly conserved transglutaminase and mannose-binding domains; therefore, study of the C. elegans ortholog png-1 represents a good potential model for this disease (https://rarediseases.org/rare-diseases/ngly1-deficiency/). The gene discussed is NGLY1; the disease is hyperinsulinemic hypoglycemia, familial, 4.