The aetiology of neurological complications of organic acidaemias—A role for the blood–brain barrier

@article{Koelker2006TheAO,
  title={The aetiology of neurological complications of organic acidaemias—A role for the blood–brain barrier},
  author={Stefan Koelker and Sven Wolfgang Sauer and Robert A H Surtees and James V. Leonard},
  journal={Journal of Inherited Metabolic Disease},
  year={2006},
  volume={29},
  pages={701-704}
}
The blood–brain barrier (BBB) metabolically isolates the central nervous system (CNS) from the circulation and protects it against fluctuations of hydrophilic nutrients in plasma and from intoxication. Recent studies have shown that dicarboxylic acids (DCAs) are transported across the blood–brain barrier at very low rates. In organic acidaemias, neurological complications are common. We hypothesize that, as a result of the very limited efflux, in certain organic acidaemias there is pathological… CONTINUE READING