TABLE 2.

Reported partial effects of CoQ₁₀ treatment in primary CoQ₁₀ deficiency patients

Gene	No. of patients included in the analysis	Responding		Not responding
		Objective description	Subjective description
PDSS1	0	–	–	–
PDSS2	2	0	0	2
COQ2	7	1	0	6
COQ4	19	3	2	14
COQ5	3	3	0	0
COQ6	3	1	0	2
COQ7	3	0	0	3
COQ8A/ADCK3	41	9	2	30
COQ8B/ADCK4	9	3	0	6
COQ9	2	0	0	2

Note: Treatment effects established by quantitative or semi‐quantitative measures to describe the response to CoQ₁₀ treatment were counted as responding with objective description, while descriptions of positive effects but without relying on quantitative or semi‐quantitative measures were counted as responding with subjective description. ‘Not responding’ include the patients who were reported not to respond to CoQ₁₀ treatment or whose responses we consider lacking a convincing demonstration of a response to CoQ₁₀ supplementation.