Affiliations: Department of Neurology, Columbia University College
of Physicians & Surgeons, New York, NY, USA
Note: [] Address for correspondence: Ali Naini, Ph.D., Department of
Neurology, Columbia University, 630 West 168th Street, New York, NY 10032, USA.
Tel.: +1 212 305 2118; Fax: +1 212 305 3986; E-mail: [email protected]
Abstract: 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_{10}y concentration.
Journal: BioFactors, vol. 18, no. 1-4, pp. 145-152, 2003
