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| TIM44 | 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. (431 aa) | ||||
| MDM10 | Mitochondrial distribution and morphology protein 10; Subunit of both the ERMES and the SAM complex; component of ERMES complex which acts as a molecular tether between the mitochondria and the ER, necessary for efficient phospholipid exchange between organelles and for mitophagy; SAM/TOB complex component that functions in the assembly of outer membrane beta-barrel proteins; involved in mitochondrial inheritance and morphology; ERMES complex is often co-localized with peroxisomes and concentrated areas of pyruvate dehydrogenase; Belongs to the MDM10 family. (493 aa) | ||||
| DER1 | Degradation in the endoplasmic reticulum protein 1; ER membrane protein that promotes export of misfolded polypeptides; required for ER-associated protein degradation of misfolded or unassembled proteins; initiates export of aberrant polypeptides from ER lumen by threading them into ER membrane and routing them to Hrd1p for ubiquitination; function normally requires N-terminal acetylation by NatB; N- and C- termini protrude into cytoplasm; similar to Dfm1p; homolog of mammalian derlin-1. (211 aa) | ||||
| EMC1 | Member of conserved endoplasmic reticulum membrane complex; involved in efficient folding of proteins in the ER; null mutant displays induction of the unfolded protein response; interacts with Gal80p; homologous to worm H17B01.4/EMC-1, fly CG2943, and human KIAA0090; Belongs to the EMC1 family. (760 aa) | ||||
| ATG9 | Autophagy-related protein 9; Transmembrane protein involved in forming Cvt and autophagic vesicles; cycles between the phagophore assembly site (PAS) and other cytosolic punctate structures, not found in autophagosomes; may be involved in membrane delivery to the PAS; Belongs to the ATG9 family. (997 aa) | ||||
| SEC1 | Sm-like protein involved in docking and fusion of exocytic vesicles; binds to assembled SNARE complexes at the membrane and stimulates membrane fusion; localization to sites of secretion (bud neck and bud tip) is dependent on SNARE function; interacts directly with essential exocyst subunit Sec6p. (724 aa) | ||||
| GET1 | Golgi to ER traffic protein 1; Subunit of the GET complex; involved in insertion of proteins into the ER membrane; required for the retrieval of HDEL proteins from the Golgi to the ER in an ERD2 dependent fashion and for normal mitochondrial morphology and inheritance. (235 aa) | ||||
| EMC4 | Member of conserved ER transmembrane complex; required for efficient folding of proteins in the ER; null mutant displays induction of the unfolded protein response; homologous to worm ZK616.6/EMC-4 and fly CG11137; mutation is functionally complemented by human EMC4. (190 aa) | ||||
| COX18 | Cytochrome c oxidase assembly protein COX18, mitochondrial; Protein required for membrane insertion of C-terminus of Cox2p; mitochondrial integral inner membrane protein; interacts genetically and physically with Mss2p and Pnt1p; similar to S. cerevisiae Oxa1, N. crassa Oxa2p, and E. coli YidC; respiratory defect of the null mutant is functionally complemented by human COX18 carrying the N-terminal 54 amino acids of S. cerevisiae Cox18p; Belongs to the OXA1/ALB3/YidC family. (316 aa) | ||||
| SAM35 | Component of the sorting and assembly machinery (SAM) complex; the SAM (or TOB) complex is located in the mitochondrial outer membrane; the complex binds precursors of beta-barrel proteins and facilitates their insertion into the outer membrane. (329 aa) | ||||
| POR2 | Mitochondrial outer membrane protein porin 2; Putative mitochondrial porin (voltage-dependent anion channel); not required for mitochondrial membrane permeability or mitochondrial osmotic stability; POR2 has a paralog, POR1, that arose from the whole genome duplication. (281 aa) | ||||
| TIM54 | Mitochondrial import inner membrane translocase subunit TIM54; Component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane. (478 aa) | ||||
| TIM17 | 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. (158 aa) | ||||
| SDH3 | Subunit of succinate dehydrogenase and of TIM22 translocase; functions as cytochrome b subunit of succinate dehydrogenase, which couples oxidation of succinate to transfer of electrons to ubiquinone as part of the TCA cycle and the mitochondrial respiratory chain; also required for mitochondrial inner membrane protein import as part of the TIM22 complex; SDH3 has a paralog, SHH3, that arose from the whole genome duplication. (198 aa) | ||||
| EMC3 | Member of conserved ER transmembrane complex; required for efficient folding of proteins in the ER; required for respiratory growth; null mutant displays induction of the unfolded protein response; homologous to worm Y62E10A.10/EMC-3, fly CG6750, human TMEM111. (253 aa) | ||||
| EMC6 | Member of conserved ER transmembrane complex; required for efficient folding of proteins in the ER; null mutant displays induction of the unfolded protein response; homologous to worm F33D4.7/EMC-6, fly CG11781, human TMEM93. (108 aa) | ||||
| SEC61 | Conserved ER protein translocation channel; essential subunit of Sec61 complex (Sec61p, Sbh1p, and Sss1p); forms channel for SRP-dependent protein import; with Sec63 complex allows SRP-independent protein import into ER; involved in posttranslational soluble protein import into the ER, ERAD of soluble substrates, and misfolded soluble protein export from the ER. (480 aa) | ||||
| USA1 | U1 SNP1-associating protein 1; Scaffold subunit of the Hrd1p ubiquitin ligase; also promotes ligase oligomerization; involved in ER-associated protein degradation (ERAD); interacts with the U1 snRNP-specific protein, Snp1p. (838 aa) | ||||
| SAM37 | Component of the Sorting and Assembly Machinery (SAM) complex; the SAM (or TOB) complex is located in the mitochondrial outer membrane; binds precursors of beta-barrel proteins and facilitates their outer membrane insertion; contributes to SAM complex stability. (327 aa) | ||||
| TOM40 | Mitochondrial import receptor subunit TOM40; Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import steps for all mitochondrially directed proteins; constitutes the core element of the protein conducting pore; pre-Tom40p is phosphorylated by PKA, which impairs its import into mitochondria under non-respiratory conditions. (387 aa) | ||||
| SAM50 | Component of the Sorting and Assembly Machinery (SAM) complex; the SAM (or TOB) complex is located in the mitochondrial outer membrane; the complex binds precursors of beta-barrel proteins and facilitates their outer membrane insertion; homologous to bacterial Omp85. (484 aa) | ||||
| POR1 | Mitochondrial porin (voltage-dependent anion channel); outer membrane protein required for maintenance of mitochondrial osmotic stability and mitochondrial membrane permeability; couples the glutathione pools of the intermembrane space (IMS) and the cytosol; interacts with Om45 and Om14 in the outer membrane; phosphorylated; protein abundance increases in response to DNA replication stress. (283 aa) | ||||
| TOM7 | Mitochondrial import receptor subunit TOM7; Component of the TOM (translocase of outer membrane) complex; responsible for recognition and initial import steps for all mitochondrially directed proteins; promotes assembly and stability of the TOM complex; Belongs to the Tom7 family. (60 aa) | ||||
| TOM22 | Mitochondrial import receptor subunit TOM22; Component of the TOM (Translocase of Outer Membrane) complex; responsible for initial import of mitochondrially directed proteins; mediates interaction between TOM and TIM complexes and acts as a receptor for precursor proteins; Belongs to the Tom22 family. (152 aa) | ||||
| ATG2 | Autophagy-related protein 2; Peripheral membrane protein required for autophagic vesicle formation; also required for vesicle formation during pexophagy and the cytoplasm-to-vaucole targeting (Cvt) pathway; involved in Atg9p cycling between the phagophore assembly site and mitochondria; contains an APT1 domain that binds phosphatidylinositol-3-phosphate; essential for cell cycle progression from G2/M to G1 under nitrogen starvation; forms cytoplasmic foci upon DNA replication stress. (1592 aa) | ||||
| TIM18 | Mitochondrial import inner membrane translocase subunit TIM18; Component of the mitochondrial TIM22 complex; involved in insertion of polytopic proteins into the inner membrane; may mediate assembly or stability of the complex; Belongs to the CybS family. (192 aa) | ||||
| TIM50 | 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. (476 aa) | ||||
| MGR2 | 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. (113 aa) | ||||