Abstract
Patients with the inherited disorders of propionate and methylmalonate metabolism such as propionic acidaemia (PA; McKusick 23200) and methylmalonic acidurias (MMA; McKusick 25100) accumulate odd-numbered long-chain fatty acids (OLCFA) in lipids of various organs (Hommes et al., 1968; Gompertz, 1970; Gompertz et al., 1970; Kishimoto et al., 1973). The accumulation of OLCFA also occurs in cultured skin fibroblasts of PA and MMA patients (Giudici et al., 1986). This is due to the fact that intracellularly accumulated propionyl-CoA can replace acetyl-CoA as a ‘primer’ for long-chain fatty acid synthesis.
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