NHLH2 and Prader-Willi syndrome: A decrease in NHLH2 (Nescient Helix-Loop-Helix 2) and PCSK1 (ProConvertase Subtilisin/Kexin type 1) expression in the iPSC-derived neurons resulted in altered prohormone processing.8 Hypothalamic loss of the Snord116 cluster in mice was also shown to reproduce the hyperphagia phenotype observed in PWS, providing the first adult mouse model of PWS.9 Analysis of the hypothalamic transcriptome of PWS patients suggested that putative SNORD116-regulated genes influence neuronal loss.10