Abcd1 KO in mice is a validated disease model for ALD (ALD mice); these mice manifest VLCFA increases that are similar to levels observed in patients (McGuinness et al., 2003; Pujol et al., 2004); in older mice, a late onset neurologic phenotype is also evident (Pujol et al., 2002). The gene discussed is ABCD1; the disease is adrenoleukodystrophy.