Translational and Clinical Pharmacology Considerations in Drug
Repurposing for X-linked Adrenoleukodystrophy-A Rare Peroxisomal
Disorder
Abstract
X-linked adrenoleukodystrophy (X-ALD) is an inherited, neurodegenerative
rare disease that can result in devastating symptoms of blindness, gait
disturbances, and spastic quadriparesis due to progressive
demyelination. Typically, the disease progresses rapidly, causing death
within the first decade of life. With limited treatments available,
efforts to determine an effective therapy that can alter disease
progression or mitigate symptoms have been undertaken for many years,
particularly through drug repurposing. Repurposing has generally been
guided through clinical experience and small trials. At this time, none
of the drug candidates have been approved for use, which may be due, in
part, to the lack of pharmacokinetic/pharmacodynamic (PK/PD) information
on the repurposed medications in the target patient population. Greater
consideration for the disease pathophysiology, drug pharmacology, and
potential drug-target interactions, specifically at the site of action,
would improve drug repurposing and facilitate development. Although
there is a good understanding of X-ALD pathophysiology, the absence of
information on drug targets, pharmacokinetics, and pharmacodynamics
hinders the repurposing of drugs for this condition. Incorporating
advanced translational and clinical pharmacological approaches in
preclinical studies and early stages clinical trials will improve the
success of repurposed drugs for X-ALD as well as other rare diseases.