Views And Reviews

Mitochondrial Disorders in Neurology

BMJ 1994; 309 doi: (Published 15 October 1994) Cite this as: BMJ 1994;309:1026
  1. L Bromley

    Ed A H V Schapria, S DiMauro Butterworth-Heinemann, £45, pp 254 ISBN 0-7506-0585-5

    Once upon a time, aeons ago, a small bacterium-like prokaryotic organism invaded a nucleated cell. After a stormy relationship they settled down and became devoted to one another. Each kept its own DNA and ribosomes; the cell provided a comfortable home, while the prokaryote became a wonderful source of energy and evolved into a mitochondrion.

    But, just as cells suffer genetic mutations and mishaps in their nuclear DNA, so do mitochondria in the mitochondrial DNA. Inheritance of mitochondrial genetic disorders is maternal because the sperm gets its nuclear DNA into the egg but leaves the mitochondria outside. Sons and daughters may suffer the …

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