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.
Bennett
M J
MJ
Department of Pathology and Pediatrics, University of Texas Southwestern Medical Center, Dallas, USA.
Boriack
R L
RL
Boustany
R M
RM
D002460:Cell Line; D004791:Enzyme Inhibitors / Q000494:pharmacology; D005231:Fatty Acids, Unsaturated / Q000494:pharmacology*; D005839:Gentamicins / Q000494:pharmacology; D006801:Humans; D007231:Infant, Newborn; D008214:Lymphocytes / Q000187:drug effects / Q000201:enzymology* / Q000378:metabolism; D008928:Mitochondria / Q000187:drug effects / Q000201:enzymology / Q000378:metabolism*; D009472:Neuronal Ceroid-Lipofuscinoses / Q000201:enzymology*; D006180:Proton-Translocating ATPases / Q000037:antagonists & inhibitors / Q000378:metabolism*
D004791:Enzyme Inhibitors; D005231:Fatty Acids, Unsaturated; D005839:Gentamicins; D006180:Proton-Translocating ATPases