TUBB4A and leukodystrophy: Taiep rat is the most reliable animal model for studying H-ABC because it has a face validity of the main pyramidal and extrapyramidal motor signs of this leukodystrophy, they had characteristic imaging features in the white matter, basal ganglia, and cerebellum using MRI analysis and they had a point mutation in the tubulin β 4a gene and its corresponding TUBB4A protein (Fig 9, left panel).