GLUD1 and hyperinsulinism: By preventing c-Myc ubiquitination-mediated degradation, XLOC_006390 promotes the transcription of glutamate dehydrogenase 1 (GDH1), a gene that encodes a mitochondrial enzyme that converts glutamate into alpha-ketoglutarate and ammonia by oxidative deamination, and whose germline pathogenic variants cause a form of Familial Hyperinsulinism [107].