TABLE 1.

A summary of the main findings of the model systems used to study ALD

Model system	Gene	Protein	Main findings
Fibroblasts	ABCD1	ABCD1	Impaired β‐oxidation and accumulation of VLCFA
Yeast	pxa1 and pxa2	Pxa1p and Pxa2p	Impaired β‐oxidation and accumulation of VLCFA
C. elegans	pmp‐4	PMP‐4	Motor defects, axonal damage, VLCFA accumulation, and impaired mitochondrial redox
Drosophila	dABCD	dABCD	Retina neurodegeneration
Zebrafish	abcd1	Abcd1	Motor defects, developmental deficiencies in olig2 + progenitors of oligodendrocyte and motor neuron, VLCFA, and cholesterol accumulation
Mouse	Abcd1	ABCD1	Late onset axonopathy, motor defects, elevated levels of VLCFA in tissues, and decreased VLCFA β‐oxidation capacity
Chimpanzee	ABCD1	ABCD1	Cerebral leukodystrophy and elevated VLCFA plasma levels
Human iPSCs	ABCD1	ABCD1	Elevated VLCFA levels of iPSC‐derived oligodendrocytes, astrocytes, and neurons from AMN and cerebral ALD patients
Arabidopsis thaliana	At_ABCD1	At_ABCD1	Seedling deficiencies in the absence of sucrose and accumulation of fatty acyl CoAs