ABCD3 and congenital bile acid synthesis defect 5: In contrast to the Abcd1 and Abcd2 knock-out models, the Abcd3 knock-out mice do not develop peripheral or central neurodegeneration (like ABCD3 deficiency in humans), but exhibit hepatomegaly associated with abnormalities in peroxisomal FA metabolism, which seems to represent a suitable model for CBAS5 [67].