Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

GrpE protein homolog, mitochondrial; Mitochondrial matrix cochaperone; nucleotide release factor for Ssc1p in protein translocation and folding; also acts as cochaperone for Ssq1p in folding of Fe-S cluster proteins; acts as oxidative sensor to regulate mitochondrial Ssc1p; in presence of oxidative stress, dimeric Mge1p becomes a monomer and unable to regulate Ssc1p function; homolog of E. coli GrpE and human Mge1 (GRPEL1), which also responds to oxidative stress; Belongs to the GrpE family.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Protein MGR2; Subunit of the TIM23 translocase complex; acts to couple Tim21p with the core Tim23 translocase; provides quality control for insertion of membrane proteins by regulating their release into the inner membrane by the TIM23 complex; absolutely required for mitochondrial import of presequence-containing proteins at elevated temperature; required for viability of cells lacking the mitochondrial genome (petite-negative phenotype); Belongs to the MGR2 family.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Import and assembly protein in mitochondrial intermembrane space; component of MIA pathway which mediates import and oxidative folding of substrates including small proteins containing twin cysteine motifs; acts in concert with Erv1p, which oxidizes the cysteine residues of Mia40p to comprise a disulfide relay system that catalyzes import; also mediates folding of Atp23p via a chaperone-like activity; forms a dimer that binds iron-sulfur cluster in vitro.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Subunit of the import motor (PAM complex); the PAM complex is a component of the Translocase of the Inner Mitochondrial membrane (TIM23 complex); essential J-protein cochaperone that stimulates Ssc1p ATPase activity to drive import; inhibited by Pam16p.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Heat shock protein SSC1, mitochondrial; Hsp70 family ATPase; constituent of the import motor component of the Translocase of the Inner Mitochondrial membrane (TIM23 complex); involved in protein translocation and folding; subunit of SceI endonuclease; SSC1 has a paralog, ECM10, that arose from the whole genome duplication.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Mitochondrial import inner membrane translocase subunit TIM10; Essential protein of the mitochondrial intermembrane space; forms a complex with Tim9p (TIM10 complex) that delivers hydrophobic proteins to the TIM22 complex for insertion into the inner membrane.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Mitochondrial import inner membrane translocase subunit TIM12; Essential protein of the inner mitochondrial membrane; peripherally localized; component of the TIM22 complex, which is a twin-pore translocase that mediates insertion of numerous multispanning inner membrane proteins.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Mitochondrial import inner membrane translocase subunit TIM13; Mitochondrial intermembrane space protein; forms a complex with Tim8p that delivers a subset of hydrophobic proteins to the TIM22 complex for insertion into the inner membrane.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Mitochondrial import inner membrane translocase subunit TIM17; Essential component of the TIM23 complex; with Tim23p, contributes to the architecture and function of the import channel; may link the import motor to the core Translocase of the Inner Mitochondrial membrane (TIM23 complex); Belongs to the Tim17/Tim22/Tim23 family.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Mitochondrial import inner membrane translocase subunit TIM21; Nonessential component of the TIM23 complex; interacts with the Translocase of the Outer Mitochondrial membrane (TOM complex) and with respiratory enzymes; may regulate the Translocase of the Inner Mitochondrial membrane (TIM23 complex) activity.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Essential component of the TIM23 complex; involved in protein import into mitochondrial matrix and inner membrane; with Tim17p, contributes to architecture and function of the import channel; TIM23 complex is short for the translocase of the inner mitochondrial membrane.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Mitochondrial import inner membrane translocase subunit TIM44; Essential component of the TIM23 complex; tethers the import motor and regulatory factors (PAM complex) to the translocation channel (Tim23p-Tim17p core complex); TIM23 complex is short for the translocase of the inner mitochondrial membrane; Belongs to the Tim44 family.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Essential component of the TIM23 complex; acts as receptor for the translocase of the inner mitochondrial membrane (TIM23) complex guiding incoming precursors from the TOM complex; may control the gating of the Tim23p-Tim17p channel.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Mitochondrial import inner membrane translocase subunit TIM54; Component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Mitochondrial import inner membrane translocase subunit TIM8; Mitochondrial intermembrane space protein; forms a complex with Tim13p that delivers a subset of hydrophobic proteins to the TIM22 complex for inner membrane insertion; homolog of human TIMM8A, implicated in Mohr-Tranebjaerg syndrome, also known as deafness-dystonia-optic neuronopathy (DDON) syndrome; human TIMM8A can complement yeast null mutant.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Mitochondrial import inner membrane translocase subunit TIM9; Essential protein of the mitochondrial intermembrane space; forms a complex with Tim10p (TIM10 complex) that delivers hydrophobic proteins to the TIM22 complex for insertion into the inner membrane.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

Protein co-chaperone with a zinc finger motif; essential for protein import into mitochondria; may act with Pam18p to facilitate recognition and folding of imported proteins by Ssc1p (mtHSP70) in the mitochondrial matrix; required for the maintenance of Ssc1p solubility and assists in the functional interaction of Ssc1p with substrate proteins.

GrpE protein homolog, mitochondrial; Mitochondrial matrix cochaperone; nucleotide release factor for Ssc1p in protein translocation and folding; also acts as cochaperone for Ssq1p in folding of Fe-S cluster proteins; acts as oxidative sensor to regulate mitochondrial Ssc1p; in presence of oxidative stress, dimeric Mge1p becomes a monomer and unable to regulate Ssc1p function; homolog of E. coli GrpE and human Mge1 (GRPEL1), which also responds to oxidative stress; Belongs to the GrpE family.

Flavin-linked sulfhydryl oxidase of the mitochondrial IMS; N-terminus is an intrinsically disordered domain that in the cytosol helps target Erv1p to mitochondria, and in the intermembrane space oxidizes Mia40p as part of a disulfide relay system that promotes intermembrane space retention of imported proteins; functional ortholog of human GFER (ALR); human GFER carrying N-terminal 21 amino acids of Erv1p functionally complements the lethality of the erv1 null mutation.

GrpE protein homolog, mitochondrial; Mitochondrial matrix cochaperone; nucleotide release factor for Ssc1p in protein translocation and folding; also acts as cochaperone for Ssq1p in folding of Fe-S cluster proteins; acts as oxidative sensor to regulate mitochondrial Ssc1p; in presence of oxidative stress, dimeric Mge1p becomes a monomer and unable to regulate Ssc1p function; homolog of E. coli GrpE and human Mge1 (GRPEL1), which also responds to oxidative stress; Belongs to the GrpE family.

Protein MGR2; Subunit of the TIM23 translocase complex; acts to couple Tim21p with the core Tim23 translocase; provides quality control for insertion of membrane proteins by regulating their release into the inner membrane by the TIM23 complex; absolutely required for mitochondrial import of presequence-containing proteins at elevated temperature; required for viability of cells lacking the mitochondrial genome (petite-negative phenotype); Belongs to the MGR2 family.

GrpE protein homolog, mitochondrial; Mitochondrial matrix cochaperone; nucleotide release factor for Ssc1p in protein translocation and folding; also acts as cochaperone for Ssq1p in folding of Fe-S cluster proteins; acts as oxidative sensor to regulate mitochondrial Ssc1p; in presence of oxidative stress, dimeric Mge1p becomes a monomer and unable to regulate Ssc1p function; homolog of E. coli GrpE and human Mge1 (GRPEL1), which also responds to oxidative stress; Belongs to the GrpE family.

Import and assembly protein in mitochondrial intermembrane space; component of MIA pathway which mediates import and oxidative folding of substrates including small proteins containing twin cysteine motifs; acts in concert with Erv1p, which oxidizes the cysteine residues of Mia40p to comprise a disulfide relay system that catalyzes import; also mediates folding of Atp23p via a chaperone-like activity; forms a dimer that binds iron-sulfur cluster in vitro.