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| FASTKD2 | FAST kinase domain-containing protein 2, mitochondrial; Plays an important role in assembly of the mitochondrial large ribosomal subunit; FASTK mitochondrial RNA binding family (710 aa) | |||
| MRPL4 | Mitochondrial ribosomal protein L4; Belongs to the universal ribosomal protein uL4 family (311 aa) | |||
| ZC3H3 | Zinc finger CCCH domain-containing protein 3; Required for the export of polyadenylated mRNAs from the nucleus. Enhances ACVR1B-induced SMAD-dependent transcription. Binds to single-stranded DNA but not to double- stranded DNA in vitro. Involved in RNA cleavage (By similarity); Zinc fingers CCCH-type (948 aa) | |||
| RBM42 | RNA-binding protein 42; Binds (via the RRM domain) to the 3’-untranslated region (UTR) of CDKN1A mRNA; RNA binding motif containing (480 aa) | |||
| FASTKD3 | FAST kinase domain-containing protein 3, mitochondrial; Required for normal mitochondrial respiration; Belongs to the FAST kinase family (662 aa) | |||
| RNF157 | E3 ubiquitin ligase RNF157; E3 ubiquitin ligase that ubiquitinates APBB1 for its degradation by the proteasome and thus prevents apoptosis and promotes survival of neurons. Has a dual role in neurons as it is also required for dendrite growth and maintenance for which its ligase activity is not critical. May act as a scaffold molecule to regulate this process. Acts as a downstream effector of the interconnected PI3K and MAPK signaling pathways and thus participates in the regulation of the cell cycle; Ring finger proteins (679 aa) | |||
| MTERF3 | Transcription termination factor 3, mitochondrial; Binds promoter DNA and regulates initiation of transcription. Required for normal mitochondrial transcription and translation, and for normal assembly of mitochondrial respiratory complexes. Required for normal mitochondrial function (By similarity). Maintains 16S rRNA levels and functions in mitochondrial ribosome assembly by regulating the biogenesis of the 39S ribosomal subunit (By similarity); Belongs to the mTERF family (417 aa) | |||
| PTCD1 | Pentatricopeptide repeat-containing protein 1, mitochondrial; Mitochondrial protein implicated in negative regulation of leucine tRNA levels, as well as negative regulation of mitochondria-encoded proteins and COX activity. Affects also the 3’-processing of mitochondrial tRNAs; Belongs to the PTCD1 family (700 aa) | |||
| RPUSD4 | Mitochondrial RNA pseudouridine synthase RPUSD4; Converts specific uridines to pseudouridine (PSI) in different mitochondrial RNA substrates. Acts on position 1397 in 16S mitochondrial ribosomal RNA (16S mt-rRNA). This modification is required for the assembly of 16S mt-rRNA into a functional mitochondrial ribosome. Acts on position 39 in mitochondrial tRNA(Phe); Belongs to the pseudouridine synthase RluA family (377 aa) | |||
| ICT1 | Peptidyl-tRNA hydrolase ICT1, mitochondrial; Essential peptidyl-tRNA hydrolase component of the mitochondrial large ribosomal subunit. Acts as a codon-independent translation release factor that has lost all stop codon specificity and directs the termination of translation in mitochondrion, possibly in case of abortive elongation. May be involved in the hydrolysis of peptidyl-tRNAs that have been prematurely terminated and thus in the recycling of stalled mitochondrial ribosomes (206 aa) | |||
| YARS2 | Tyrosine--tRNA ligase, mitochondrial; Catalyzes the attachment of tyrosine to tRNA(Tyr) in a two-step reaction- tyrosine is first activated by ATP to form Tyr- AMP and then transferred to the acceptor end of tRNA(Tyr); Belongs to the class-I aminoacyl-tRNA synthetase family (477 aa) | |||
| DDX28 | Probable ATP-dependent RNA helicase DDX28; Plays an essential role in facilitating the proper assembly of the mitochondrial large ribosomal subunit and its helicase activity is essential for this function. May be involved in RNA processing or transport. Has RNA and Mg(2+)-dependent ATPase activity; DEAD-box helicases (540 aa) | |||
| ZNF324B | Zinc finger protein 324B; May be involved in transcriptional regulation; Zinc fingers C2H2-type (544 aa) | |||
| HNRNPA1 | Heterogeneous nuclear ribonucleoprotein A1; Involved in the packaging of pre-mRNA into hnRNP particles, transport of poly(A) mRNA from the nucleus to the cytoplasm and may modulate splice site selection. May bind to specific miRNA hairpins; RNA binding motif containing (372 aa) | |||
| TRUB2 | Mitochondrial mRNA pseudouridine synthase TRUB2; Minor enzyme contributing to the conversion of uridine to pseudouridine (PSI) at position 390 in mitochondrial COXI (MT- CO1) mRNA and at position 697-699 in mitochondrial COXIII (MT-CO3) mRNA, modulating the efficiency of mitochondrial protein synthesis without changes in transcript abundance or stability (331 aa) | |||
| MRPL50 | Mitochondrial ribosomal protein L50 (158 aa) | |||
| NGRN | Neugrin; May be involved in neuronal differentiation (291 aa) | |||
| APIP | Methylthioribulose-1-phosphate dehydratase; Catalyzes the dehydration of methylthioribulose-1- phosphate (MTRu-1-P) into 2,3-diketo-5-methylthiopentyl-1- phosphate (DK-MTP-1-P). Functions in the methionine salvage pathway, which plays a key role in cancer, apoptosis, microbial proliferation and inflammation. May inhibit the CASP1-related inflammatory response (pyroptosis), the CASP9-dependent apoptotic pathway and the cytochrome c-dependent and APAF1-mediated cell death; Belongs to the aldolase class II family. MtnB subfamily (242 aa) | |||
| TSC22D1 | TSC22 domain family protein 1; Transcriptional repressor. Acts on the C-type natriuretic peptide (CNP) promoter (1073 aa) | |||
| CLTC | Clathrin heavy chain 1; Clathrin is the major protein of the polyhedral coat of coated pits and vesicles. Two different adapter protein complexes link the clathrin lattice either to the plasma membrane or to the trans-Golgi network. Acts as component of the TACC3/ch- TOG/clathrin complex proposed to contribute to stabilization of kinetochore fibers of the mitotic spindle by acting as inter- microtubule bridge. The TACC3/ch-TOG/clathrin complex is required for the maintenance of kinetochore fiber tension. Plays a role in early autophagosome formation; Belongs to the clathrin heavy chain [...] (1679 aa) | |||
| WBSCR16 | RCC1-like G exchanging factor-like protein; Williams-Beuren syndrome chromosome region 16 (464 aa) | |||
