Table 1.

Interactions between mitochondria and proteins encoded by genes that are mutated in Mendelian Parkinson’s Disease.

Locus	Gene product	Inheritance & comments	Direct or indirect interaction with mitochondria
PARK1/4	α-Synuclein	AD	Mutant α-synuclein sensitizes neurons to oxidative stress and damage.
PARK2	Parkin	AR, most common cause of recessive juvenile PD	Parkin mutations lead to increased oxidative stress and in turn mitochondrial dysfunction can affect parkin function.
PARK6	PINK1	AR, second most common cause of recessive juvenile PD	A mitochondria-localized kinase; its deficiency sensitizes mitochondria to rotenone and induces degeneration of dopaminergic neurons.
PARK7	DJ-1	AR	A possible redox sensor; binds to mitochondrial complex I and maintain its activity.
PARK8	LRRK2	AD, most common cause of dominant PD	Associates with the outer mitochondrial membrane and can bind parkin.
PARK13	OMI/HTRA2	* AD?	A mitochondrial protease; acts downstream of PINK1; loss of HtrA2 results in the accumulation of unfolded proteins in the mitochondria and increased production of ROS.

AD=autosomal dominant; AR=autosomal recessive.

^*Not uniformly accepted.