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. Author manuscript; available in PMC: 2022 Nov 14.
Published in final edited form as: J Neurosci Res. 2021 Oct 29;99(12):3170–3181. doi: 10.1002/jnr.24953

TABLE 1.

Phenotype, biochemical findings, pathophysiological insights, and limitations of each animal model of X-ALD

Species Gene k/o (% identity to ABCD1) Phenotype / Pathology
C26:0 elevation Locomotion (test) Life span
Mouse (hemizygotes) Abcd1 (92%)

  • Adrenal: × 2–8

  • Sciatic nerve and spinal cord: × 6

  • CNS: × 2–5

  • Decreased coordination (rotarod)

  • Decreased exploration (open field)

 Unaltered
Zebrafish abcd1 (68%) × 1.4–1.9 (whole animal extracts) Reduced (evoked) swimming distance 20%–30% reduction in competitive survival to 30 days. Unaltered life span when reared separately
Caenorhabditis elegans Pmp-4 (57%) 25% increase

  • 10% reduction in lateral swimming motion

  • Increased lethality when exposed to oxidative stress

 Unaltered
Drosophila dABCD (53%) Not reported Not reported Unaltered
Selected additional phenotypes models with deficiency in genes related to ABCD1
Mouse Abcd1 and Abcd2 double k/o

  • Adrenal: × 16

  • Sciatic nerve and spinal cord: × 6

  • Locomotion defects begins at 12 months instead of 20 months

  • Reversibility of symptoms with antioxidant treatment

 Unaltered
Drosophila bgm and dbb (VLCFA acyl-CoA synthases)

  • ×2 increase for bgm (× 1.5 for C26:1)

  • 50% reduction (beam breaks assay)

  • bgm: 0%–30% reduction

  • dbb: 15% reduction