ASNSD1 (asparagine synthetase domain containing 1) is a conserved protein with emerging roles in cellular metabolism and disease pathogenesis. The gene produces a bicistronic mRNA encoding both a canonical protein and a functional upstream open reading frame (uORF)-derived microprotein called ASDURF 1. ASNSD1 expression is maximal in skeletal muscle 2 and functions through protein-protein interactions [NCBI annotation]. ASNSD1 has multiple disease associations: it is dysregulated in childhood medulloblastoma where the ASNSD1-uORF microprotein ASDURF promotes cell survival via prefoldin-like chaperone complex engagement 3, drives hepatic fibrosis through TGFβ1/Smad3 and NF-κB signaling pathways 4, and functions as a glutamine metabolism-associated gene implicated in diabetic foot ulcer pathogenesis 5 and Alzheimer's disease 6. Loss of ASNSD1 function causes progressive degenerative myopathy with severe sarcopenia and myosteatosis in mice, characterized by muscle-to-adipose tissue transdifferentiation 2. ASNSD1 mutations have been identified in severe early-onset obesity, representing monogenic variants disrupting metabolic homeostasis 7. Additionally, genetic variants in ASNSD1 modulate statin-induced gene expression changes 8. These findings establish ASNSD1 as a critical regulator of skeletal muscle integrity and metabolic disease.