Mutations at the flavin binding site of ETF:QO yield a MADD-like severe phenotype in Drosophila

► Fly mutants defective for embryonic cuticle allelic to ETF:QO were identified. ► Three missense mutations identified in ETF:QO (G65E, A68V and S104F). ► Raised acylcarnitine levels in fly embryos as in severe MADD. ► Mutations map at FAD motif within Rossmann fold inactivating enzyme. ► Severe MADD-like phenotype in the fruit fly sets proof of concept of suitability as disease model.