TRAPPC11 encodes a crucial subunit of the trafficking protein particle (TRAPP) complex, which functions as a tethering factor in intracellular membrane trafficking, specifically facilitating endoplasmic reticulum to Golgi transport 1. Beyond its established role in secretory pathway trafficking, TRAPPC11 also functions upstream of autophagosome formation by recruiting ATG2B and WIPI4/WDR45 to preautophagosomal membranes, where it participates in isolation membrane expansion 2. Pathogenic variants in TRAPPC11 cause a spectrum of muscular dystrophies, ranging from limb-girdle muscular dystrophy (LGMD-R18/LGMD2S) to congenital muscular dystrophy with systemic involvement 1. The disease mechanism involves defective membrane trafficking and abnormal glycosylation, specifically hypoglycosylation of α-dystroglycan, establishing TRAPPC11-opathies as congenital disorders of glycosylation (CDG) 31. Patients typically present with muscle weakness, elevated creatine kinase levels, and dystrophic muscle changes, with severe cases showing additional features including developmental delay, seizures, microcephaly, liver disease, and movement disorders 1. Cellular studies in patient fibroblasts demonstrate impaired ER-Golgi transport and decreased expression of glycoproteins, supporting the glycosylation defect as the underlying pathogenic mechanism 1.