TABLE 2.

Genes whose inactivation results in decreased fitness in cells lacking tafazzin function

Standard name	Systematic name	SGD description
Mitochondrial quality control
YME1	YPR024W	Catalytic subunit of the i-AAA protease complex; responsible for degradation of unfolded or misfolded mitochondrial gene products; also has a role in intermembrane space protein folding; mutation causes an elevated rate of mitochondrial turnover
Arginine metabolism
ORT1	YOR130C	Ornithine transporter of the mitochondrial inner membrane; exports ornithine from mitochondria as part of arginine biosynthesis; human ortholog is associated with hyperammonemia-hyperornithinemia-homocitrullinuria (HHH) syndrome
ARG3	YJL088W	Ornithine carbamoyltransferase; also known as carbamoylphosphate:l-ornithine carbamoyltransferase; catalyzes the biosynthesis of the arginine precursor citrulline.
CPA1	YOR303W	Small subunit of carbamoyl phosphate synthetase; carbamoyl phosphate synthetase catalyzes a step in the synthesis of citrulline, an arginine precursor; translationally regulated by an attenuator peptide encoded by YOR302W within the CPA1 mRNA 5′-leader.
CPA2	YJR109C	Large subunit of carbamoyl phosphate synthetase; carbamoyl phosphate synthetase catalyzes a step in the synthesis of citrulline, an arginine precursor
ARG1	YOL058W	Arginosuccinate synthetase; catalyzes the formation of l-argininosuccinate from citrulline and l-aspartate in the arginine biosynthesis pathway; potential Cdc28p substrate.
Mitochondrial iron metabolism
ISA2	YPR067W	Protein required for maturation of mitochondrial [4Fe-4S] proteins; functions in a complex with Isa1p and possibly Iba57p; localizes to the mitochondrial intermembrane space; overexpression of ISA2 suppresses grx5 mutations.
GGC1	YDL198C	Mitochondrial GTP/GDP transporter; essential for mitochondrial genome maintenance; has a role in mitochondrial iron transport.
Mitochondrial iron metabolism
FRE4	YNR060W	Ferric reductase; reduces a specific subset of siderophore-bound iron prior to uptake by transporters; expression induced by low iron levels.
SIT1	YEL065W	Ferrioxamine B transporter; member of the ARN family of transporters that specifically recognize siderophore-iron chelates; transcription is induced during iron deprivation and diauxic shift; potentially phosphorylated by Cdc28p
Mitochondrial protein import
TOM5	YPR133W-A	Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import of all mitochondrially directed proteins; involved in transfer of precursors from the Tom70p and Tom20p receptors to the Tom40p pore