Rate My Professor Johan Van Hove

Van Hove's academic interests encompass neurometabolic diseases causing seizures, with particular emphasis on non-ketotic hyperglycinemia (NKH), exploring its genetic basis primarily in GLDC and AMT genes, clinical spectrum from neonatal epileptic encephalopathy to later-onset developmental delays, genotype-phenotype correlations for outcome prediction, pathogenesis involving glycine toxicity and folate methylation deficits in brain cells using patient samples and mouse models, and therapeutic developments such as protein stabilization with chaperones, methylation enhancement, and optimization of current therapies through clinical studies. His research also addresses pyridoxine-dependent epilepsy and metabolic epilepsies, mitochondrial energy disorders through clinical test development, new genetic cause identification, and treatment exploration, as well as disorders of lipoate metabolism. He has produced over 100 publications, including key works such as "Selvanathan A et al., The History and Nosology of the Glycine Disorders: A Framework for Clinicians" (J Inherit Metab Dis, 2026), "Friederich MW, Van Hove JLK, Clinical utility of the ATP hydrolysis assay for the diagnosis of complex V deficiency in cultured skin fibroblasts" (Mol Genet Metab, 2025), "Karaa A et al., RePOWER: An International, Prospective, Non-Interventional Registry of Patients With Primary Mitochondrial Myopathy" (Clin Genet, 2026), and the influential "Pompe disease diagnosis and management guideline" (Genet Med, 2006). Van Hove's contributions have advanced diagnosis and treatment strategies for these rare disorders.