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Polyunsaturated fatty acids reverse the lysosomal storage and accumulation of subunit 9 of mitochondrial F1F0-ATP synthase in cultured lymphoblasts from patients with Batten disease.

AuthorsM J Bennett, R L Boriack, R M Boustany
JournalJournal of inherited metabolic disease (J Inherit Metab Dis) Vol. 20 Issue 3 Pg. 457-60 (Jul 1997) ISSN: 0141-8955 [Print] United States
PMID9266381 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Enzyme Inhibitors
  • Fatty Acids, Unsaturated
  • Gentamicins
  • Proton-Translocating ATPases
Topics
  • Cell Line
  • Enzyme Inhibitors (pharmacology)
  • Fatty Acids, Unsaturated (pharmacology)
  • Gentamicins (pharmacology)
  • Humans
  • Infant, Newborn
  • Lymphocytes (drug effects, enzymology, metabolism)
  • Mitochondria (drug effects, enzymology, metabolism)
  • Neuronal Ceroid-Lipofuscinoses (enzymology)
  • Proton-Translocating ATPases (antagonists & inhibitors, metabolism)

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