Table 1. .

Summary of Clinical and Molecular Data for 14 Chinese Families with LHON

Pedigree	Ratio of Affected Males to Affected Females	Average Age at Onset (y)	No. of Matrilineal Relatives	Penetrance (%)*	G11778A Mutation	T3866C Mutation	Mitochondrial DNA Haplogroup
WZ4116	1:0	18	7	14.3	+		M8a2
WZ42	2:0	14.5	25	8	+		D4g2
WZ43	1:0	15	9	11.1	+		B4a1c
WZ44	1:0	15	17	5.9	+		B5b
WZ45	2:0	16	12	16.7	+		N9a1
WZ46	2:1	14	19	15.8	+		D4b2b
WZ47	2:1	27	11	27.3	+		C
WZ48	1:0	16	19	5.3	+		M7b1
WZ141	6:0	22	38	15.8	+		B5
WZ442	3:0	17	9	33.3	+		F1
WZ5	4:1	20	14	35.7	+		D4b2b
WZ510	3:1	24	27	29.6		+	D4a
WZ511	0:1	11	8	12.5		+	M10a
WZ512	0:1	5	6	16.7		+	R

Superscript reference citation numbers refer to studies from which data were derived: Qu et al.,⁴¹ Qian et al.,⁴² and Qu et al.¹⁶

^*Penetrance: affected matrilineal relatives/total matrilineal relatives.