Coenzyme Q10 in neurodegenerative disorders: Potential benefit of CoQ10 supplementation for multiple system atrophy

Table 1 Genotype-phenotype correlation in primary coenzyme Q10 deficiencies

	Clinical features	Age at onset	Response to CoQ10 supplementation	Ref.
PDSS1	Severe infantile multi-systemic disease	1-2 year	Improved and alive	[12]
PDSS2	Severe infantile multi-systemic disease	3 mo	No clinical response	[13]
	Leigh syndrome
COQ2	Nephropathy Severe infantile multi-systemic disease	Infantile-or early childhood-onset	Dramatic improvement of neurological manifestations and nephritic syndrome	[12,14-17]
	Multiple system atrophy	Adult-onset	Unknown	[8]
COQ4	Encephalomyopathy	< 3 yr	Significant improvement of neuromuscular symptoms	[18]
COQ6	Nephropathy with sensorineural deafness	Infantile-or early childhood-onset	Improvement of nephritic syndrome and hearing loss	[19]
	Severe infantile multi-systemic disease	< 3 yr
COQ8 (CABC1)	Cerebellar ataxia	Juvenile-or adult-onset	Severe neurological deficit with epilepsy ~mild improvement of ataxia	[20-23]
COQ9	Severe infantile multi-systemic disease	Birth	No clinical response	[24]

CoQ10: Coenzyme Q10; PDSS: Prenyldiphosphate synthase subunit; CABC: Chaperone-activity of bc1.