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Primary coenzyme Q 10 deficiency and the brain
Author(s) -
Naini Ali,
Lewis VerniceJackson,
Hirano Michio,
Dimauro Salvatore
Publication year - 2003
Publication title -
biofactors
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.204
H-Index - 94
eISSN - 1872-8081
pISSN - 0951-6433
DOI - 10.1002/biof.5520180217
Subject(s) - cerebellum , ataxia , coenzyme q – cytochrome c reductase , medicine , endocrinology , atrophy , human brain , cerebellar ataxia , coenzyme q10 , biology , biochemistry , neuroscience , mitochondrion , cytochrome c
Our findings in 19 new patients with cerebellar ataxia establish the existence of an ataxic syndrome due to primary CoQ 10 deficiency and responsive to CoQ 10 therapy. As all patients presented cerebellar ataxia and cerebellar atrophy, this suggests a selective vulnerability of the cerebellum to CoQ 10 deficiency. We investigated the regional distribution of coenzyme Q 10 in the brain of adult rats and in the brain of one human subject. We also evaluated the levels of coenzyme Q 9 (CoQ 9 ) and CoQ 10 in different brain regions and in visceral tissues of rats before and after oral administration of CoQ 10 . Our results show that in rats, amongst the seven brain regions studied, cerebellum contains the lowest level of CoQ. However, the relative proportion of CoQ 10 was the same (about 30% of total CoQ) in all regions studied. The level of CoQ 10 is much higher in brain than in blood or visceral tissue, such as liver, heart, or kidney. Daily oral administration of CoQ 10 led to substantial increases of CoQ 10 concentrations only in blood and liver. Of the four regions of one human brain studied, cerebellum again had the lowest CoQ 10y concentration.