Table 1.

Mitochondrial Lipid-Metabolism-Related Proteins

name
in yeast	in mammals	description	localization
Mdm35	TRIAP1	obligate binding partner of Ups1 and Ups2; transports PA or PS across the IMS	IMS
Pgs1	PGS1	phosphatidylglycerophosphate synthase; catalyzes the committed step in CL biosynthesis	matrix
Psd1	PISD	Phosphatidylserine decarboxylase; generates PE from PS; active site faces the IMS.	IM
	STARD7	transports PC to the OM and from the OM to the IM; localization and function controlled by the context of PARL-mediated proteolysis	IMS and OM
Taz1	TAZ	remodels the acyl chain composition of CL; lyso-lipid transacylase; mutations cause Barth syndrome	IM and OM
Ups1	PRELID1	Ups1/Mdm35 heterodimer transports PA from the OM to the IM; transported PA fuels the CL biosynthetic pathway; requires Mdm35 for stability	IMS
Ups2	PRELID3b	Ups2/Mdm35 heterodimer transports PS from the OM to the IM; transported PS is converted to PE by Psd1/PISD; requires Mdm35 for stability	IMS