[Analysis of SNPs and enzymatic disorder in the patients of influenza-associated encephalopathy: disorder of fatty acid metabolism in mitochondria induced by high fever].
To assess the etiology of influenza-associated encephalopathy(IAE), a surveillance effort was conducted during 2000-2005 in Japan. Over half of fatal and handicapped IAE patients exhibited a disorder of mitochondrial beta-oxidation and ATP generation evoked by the thermolabile phenotype of carnitine palmitoyltransferase II variations with transiently elevated serum acylcarnitine during high-grade fever. Model mice having impaired mitochondrial beta-oxidation exhibited significant accumulation of mini-plasmin and up-regulation of trypsin in the cerebral capillaries after infection with influenza A virus, resulting in the destruction of blood-brain barrier and increased brain vascular permeability. Trypsin up-regulation was also evident in the neuronal cells in the hippocampus, suggesting a severe neurologic complication of IAE.
Kido
Hiroshi
H
Division of Enzyme Chemistry, Institute for Enzyme Research, The University of Tokushima.
Yao
Dengbing
D
Le Trong
Quang
Q
Tsukane
Mariko
M
Chida
Junji
J
D000818:Animals; D001927:Brain Diseases / Q000209:etiology*; D002334:Carnitine O-Palmitoyltransferase / Q000235:genetics / Q000378:metabolism*; D005227:Fatty Acids / Q000378:metabolism*; D006801:Humans; D007251:Influenza, Human / Q000150:complications*; D051379:Mice; D008928:Mitochondria / Q000378:metabolism*; D020641:Polymorphism, Single Nucleotide
D005227:Fatty Acids; D002334:Carnitine O-Palmitoyltransferase