Accelerated clinical disease and pathology in mucopolysaccharidosis type IIIB and GalNAc transferase double knockout mice

► MPS IIIB is a neuropathic disease with incompletely understood pathology. ► Secondary ganglioside accumulation is present but has an unknown clinical impact. ► A double knockout model was used to study MPS IIIB mice with no GM2 gangliosides. ► Double knockout animals had a novel and much accelerated disease. ► The model may aid studies of pathology and therapy directed at MPS IIIB.