The rhesus macaque model of Krabbe disease has a dinucleotide deletion that abolishes galactosylceramidase (GALC) activity and shows clinical signs (muscle tremors of head and limbs, ataxia, hypertonia, and incoordination) and immunopathologic alterations (central and peripheral demyelination, the presence of multinucleated globoid cells, and psychosine accumulation) resembling those found in humans (Baskin et al, 1998; Weimer et al, 2005; Borda et al, 2008). The gene discussed is GALC; the disease is Krabbe disease.