Polyribonucleotide 5’-hydroxyl-kinase Clp1; Polynucleotide kinase that can phosphorylate the 5’- hydroxyl groups of double-stranded RNA (dsRNA), single-stranded RNA (ssRNA), double-stranded DNA (dsDNA) and double-stranded DNA-RNA hybrids. dsRNA is phosphorylated more efficiently than dsDNA, and the RNA component of a DNA-RNA hybrid is phosphorylated more efficiently than the DNA component. Plays a key role in both tRNA splicing and mRNA 3’-end formation. Component of the tRNA splicing endonuclease complex- phosphorylates the 5’-terminus of the tRNA 3’-exon during tRNA splicing; this ph [...]

Microprocessor complex subunit DGCR8; Component of the microprocessor complex that acts as a RNA- and heme-binding protein that is involved in the initial step of microRNA (miRNA) biogenesis. Component of the microprocessor complex that is required to process primary miRNA transcripts (pri-miRNAs) to release precursor miRNA (pre-miRNA) in the nucleus. Within the microprocessor complex, DGCR8 function as a molecular anchor necessary for the recognition of pri-miRNA at dsRNA-ssRNA junction and directs DROSHA to cleave 11 bp away form the junction to release hairpin-shaped pre-miRNAs that [...]

Polyribonucleotide 5’-hydroxyl-kinase Clp1; Polynucleotide kinase that can phosphorylate the 5’- hydroxyl groups of double-stranded RNA (dsRNA), single-stranded RNA (ssRNA), double-stranded DNA (dsDNA) and double-stranded DNA-RNA hybrids. dsRNA is phosphorylated more efficiently than dsDNA, and the RNA component of a DNA-RNA hybrid is phosphorylated more efficiently than the DNA component. Plays a key role in both tRNA splicing and mRNA 3’-end formation. Component of the tRNA splicing endonuclease complex- phosphorylates the 5’-terminus of the tRNA 3’-exon during tRNA splicing; this ph [...]

Serine/threonine-protein kinase/endoribonuclease IRE1; Senses unfolded proteins in the lumen of the endoplasmic reticulum via its N-terminal domain which leads to enzyme auto- activation. The active endoribonuclease domain splices XBP1 mRNA to generate a new C-terminus, converting it into a potent unfolded-protein response transcriptional activator and triggering growth arrest and apoptosis

Polyribonucleotide 5’-hydroxyl-kinase Clp1; Polynucleotide kinase that can phosphorylate the 5’- hydroxyl groups of double-stranded RNA (dsRNA), single-stranded RNA (ssRNA), double-stranded DNA (dsDNA) and double-stranded DNA-RNA hybrids. dsRNA is phosphorylated more efficiently than dsDNA, and the RNA component of a DNA-RNA hybrid is phosphorylated more efficiently than the DNA component. Plays a key role in both tRNA splicing and mRNA 3’-end formation. Component of the tRNA splicing endonuclease complex- phosphorylates the 5’-terminus of the tRNA 3’-exon during tRNA splicing; this ph [...]

3-hydroxyacyl-CoA dehydrogenase type-2; Mitochondrial dehydrogenase that catalyzes the beta- oxidation at position 17 of androgens and estrogens and has 3- alpha-hydroxysteroid dehydrogenase activity with androsterone. Catalyzes the third step in the beta-oxidation of fatty acids. Carries out oxidative conversions of 7-alpha-OH and 7-beta-OH bile acids. Also exhibits 20-beta- OH and 21-OH dehydrogenase activities with C21 steroids. By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD). Essential for structur [...]

Polyribonucleotide 5’-hydroxyl-kinase Clp1; Polynucleotide kinase that can phosphorylate the 5’- hydroxyl groups of double-stranded RNA (dsRNA), single-stranded RNA (ssRNA), double-stranded DNA (dsDNA) and double-stranded DNA-RNA hybrids. dsRNA is phosphorylated more efficiently than dsDNA, and the RNA component of a DNA-RNA hybrid is phosphorylated more efficiently than the DNA component. Plays a key role in both tRNA splicing and mRNA 3’-end formation. Component of the tRNA splicing endonuclease complex- phosphorylates the 5’-terminus of the tRNA 3’-exon during tRNA splicing; this ph [...]

Mitochondrial ribonuclease P catalytic subunit; Catalytic ribonuclease component of mitochondrial ribonuclease P, a complex composed of TRMT10C/MRPP1, HSD17B10/MRPP2 and MRPP3, which cleaves tRNA molecules in their 5’-ends. The presence of TRMT10C/MRPP1, HSD17B10/MRPP2 is required to catalyze tRNA molecules in their 5’-ends

Polyribonucleotide 5’-hydroxyl-kinase Clp1; Polynucleotide kinase that can phosphorylate the 5’- hydroxyl groups of double-stranded RNA (dsRNA), single-stranded RNA (ssRNA), double-stranded DNA (dsDNA) and double-stranded DNA-RNA hybrids. dsRNA is phosphorylated more efficiently than dsDNA, and the RNA component of a DNA-RNA hybrid is phosphorylated more efficiently than the DNA component. Plays a key role in both tRNA splicing and mRNA 3’-end formation. Component of the tRNA splicing endonuclease complex- phosphorylates the 5’-terminus of the tRNA 3’-exon during tRNA splicing; this ph [...]

Ribonucleases P/MRP protein subunit POP1; Component of ribonuclease P, a protein complex that generates mature tRNA molecules by cleaving their 5’-ends. Also a component of RNase MRP

Polyribonucleotide 5’-hydroxyl-kinase Clp1; Polynucleotide kinase that can phosphorylate the 5’- hydroxyl groups of double-stranded RNA (dsRNA), single-stranded RNA (ssRNA), double-stranded DNA (dsDNA) and double-stranded DNA-RNA hybrids. dsRNA is phosphorylated more efficiently than dsDNA, and the RNA component of a DNA-RNA hybrid is phosphorylated more efficiently than the DNA component. Plays a key role in both tRNA splicing and mRNA 3’-end formation. Component of the tRNA splicing endonuclease complex- phosphorylates the 5’-terminus of the tRNA 3’-exon during tRNA splicing; this ph [...]

Ribonuclease P/MRP protein subunit POP5; Component of ribonuclease P, a protein complex that generates mature tRNA molecules by cleaving their 5’-ends. Also a component of RNase MRP

Polyribonucleotide 5’-hydroxyl-kinase Clp1; Polynucleotide kinase that can phosphorylate the 5’- hydroxyl groups of double-stranded RNA (dsRNA), single-stranded RNA (ssRNA), double-stranded DNA (dsDNA) and double-stranded DNA-RNA hybrids. dsRNA is phosphorylated more efficiently than dsDNA, and the RNA component of a DNA-RNA hybrid is phosphorylated more efficiently than the DNA component. Plays a key role in both tRNA splicing and mRNA 3’-end formation. Component of the tRNA splicing endonuclease complex- phosphorylates the 5’-terminus of the tRNA 3’-exon during tRNA splicing; this ph [...]

Ribonuclease P protein subunit p14; Part of ribonuclease P, a protein complex that generates mature tRNA molecules by cleaving their 5’-ends

Polyribonucleotide 5’-hydroxyl-kinase Clp1; Polynucleotide kinase that can phosphorylate the 5’- hydroxyl groups of double-stranded RNA (dsRNA), single-stranded RNA (ssRNA), double-stranded DNA (dsDNA) and double-stranded DNA-RNA hybrids. dsRNA is phosphorylated more efficiently than dsDNA, and the RNA component of a DNA-RNA hybrid is phosphorylated more efficiently than the DNA component. Plays a key role in both tRNA splicing and mRNA 3’-end formation. Component of the tRNA splicing endonuclease complex- phosphorylates the 5’-terminus of the tRNA 3’-exon during tRNA splicing; this ph [...]

Ribonuclease P protein subunit p21; Component of ribonuclease P, a protein complex that generates mature tRNA molecules by cleaving their 5’-ends; Belongs to the eukaryotic/archaeal RNase P protein component 4 family

Polyribonucleotide 5’-hydroxyl-kinase Clp1; Polynucleotide kinase that can phosphorylate the 5’- hydroxyl groups of double-stranded RNA (dsRNA), single-stranded RNA (ssRNA), double-stranded DNA (dsDNA) and double-stranded DNA-RNA hybrids. dsRNA is phosphorylated more efficiently than dsDNA, and the RNA component of a DNA-RNA hybrid is phosphorylated more efficiently than the DNA component. Plays a key role in both tRNA splicing and mRNA 3’-end formation. Component of the tRNA splicing endonuclease complex- phosphorylates the 5’-terminus of the tRNA 3’-exon during tRNA splicing; this ph [...]

Ribonuclease P protein subunit p25; Component of ribonuclease P, a protein complex that generates mature tRNA molecules by cleaving their 5’-ends. Also a component of RNase MRP. This subunit binds to RNA

Polyribonucleotide 5’-hydroxyl-kinase Clp1; Polynucleotide kinase that can phosphorylate the 5’- hydroxyl groups of double-stranded RNA (dsRNA), single-stranded RNA (ssRNA), double-stranded DNA (dsDNA) and double-stranded DNA-RNA hybrids. dsRNA is phosphorylated more efficiently than dsDNA, and the RNA component of a DNA-RNA hybrid is phosphorylated more efficiently than the DNA component. Plays a key role in both tRNA splicing and mRNA 3’-end formation. Component of the tRNA splicing endonuclease complex- phosphorylates the 5’-terminus of the tRNA 3’-exon during tRNA splicing; this ph [...]

Ribonuclease P protein subunit p30; Component of ribonuclease P, a protein complex that generates mature tRNA molecules by cleaving their 5’-ends

Polyribonucleotide 5’-hydroxyl-kinase Clp1; Polynucleotide kinase that can phosphorylate the 5’- hydroxyl groups of double-stranded RNA (dsRNA), single-stranded RNA (ssRNA), double-stranded DNA (dsDNA) and double-stranded DNA-RNA hybrids. dsRNA is phosphorylated more efficiently than dsDNA, and the RNA component of a DNA-RNA hybrid is phosphorylated more efficiently than the DNA component. Plays a key role in both tRNA splicing and mRNA 3’-end formation. Component of the tRNA splicing endonuclease complex- phosphorylates the 5’-terminus of the tRNA 3’-exon during tRNA splicing; this ph [...]

Ribonuclease P protein subunit p40; Component of ribonuclease P, a protein complex that generates mature tRNA molecules by cleaving their 5’-ends

Polyribonucleotide 5’-hydroxyl-kinase Clp1; Polynucleotide kinase that can phosphorylate the 5’- hydroxyl groups of double-stranded RNA (dsRNA), single-stranded RNA (ssRNA), double-stranded DNA (dsDNA) and double-stranded DNA-RNA hybrids. dsRNA is phosphorylated more efficiently than dsDNA, and the RNA component of a DNA-RNA hybrid is phosphorylated more efficiently than the DNA component. Plays a key role in both tRNA splicing and mRNA 3’-end formation. Component of the tRNA splicing endonuclease complex- phosphorylates the 5’-terminus of the tRNA 3’-exon during tRNA splicing; this ph [...]

tRNA methyltransferase 10 homolog C; Mitochondrial tRNA N(1)-methyltransferase involved in mitochondrial tRNA maturation. Component of mitochondrial ribonuclease P, a complex composed of TRMT10C/MRPP1, HSD17B10/MRPP2 and MRPP3, which cleaves tRNA molecules in their 5’-ends. Together with HSD17B10/MRPP2, forms a subcomplex of the mitochondrial ribonuclease P, named MRPP1-MRPP2 subcomplex, which displays functions that are independent of the ribonuclease P activity. The MRPP1-MRPP2 subcomplex catalyzes the formation of N(1)- methylguanine and N(1)-methyladenine at position 9 (m1G9 and m1 [...]

Polyribonucleotide 5’-hydroxyl-kinase Clp1; Polynucleotide kinase that can phosphorylate the 5’- hydroxyl groups of double-stranded RNA (dsRNA), single-stranded RNA (ssRNA), double-stranded DNA (dsDNA) and double-stranded DNA-RNA hybrids. dsRNA is phosphorylated more efficiently than dsDNA, and the RNA component of a DNA-RNA hybrid is phosphorylated more efficiently than the DNA component. Plays a key role in both tRNA splicing and mRNA 3’-end formation. Component of the tRNA splicing endonuclease complex- phosphorylates the 5’-terminus of the tRNA 3’-exon during tRNA splicing; this ph [...]

tRNA-splicing endonuclease subunit Sen2; Constitutes one of the two catalytic subunit of the tRNA-splicing endonuclease complex, a complex responsible for identification and cleavage of the splice sites in pre-tRNA. It cleaves pre-tRNA at the 5’- and 3’-splice sites to release the intron. The products are an intron and two tRNA half-molecules bearing 2’,3’-cyclic phosphate and 5’-OH termini. There are no conserved sequences at the splice sites, but the intron is invariably located at the same site in the gene, placing the splice sites an invariant distance from the constant structural [...]

Polyribonucleotide 5’-hydroxyl-kinase Clp1; Polynucleotide kinase that can phosphorylate the 5’- hydroxyl groups of double-stranded RNA (dsRNA), single-stranded RNA (ssRNA), double-stranded DNA (dsDNA) and double-stranded DNA-RNA hybrids. dsRNA is phosphorylated more efficiently than dsDNA, and the RNA component of a DNA-RNA hybrid is phosphorylated more efficiently than the DNA component. Plays a key role in both tRNA splicing and mRNA 3’-end formation. Component of the tRNA splicing endonuclease complex- phosphorylates the 5’-terminus of the tRNA 3’-exon during tRNA splicing; this ph [...]

tRNA-splicing endonuclease subunit Sen54; Non-catalytic subunit of the tRNA-splicing endonuclease complex, a complex responsible for identification and cleavage of the splice sites in pre-tRNA. It cleaves pre-tRNA at the 5’ and 3’ splice sites to release the intron. The products are an intron and two tRNA half-molecules bearing 2’,3’ cyclic phosphate and 5’-OH termini. There are no conserved sequences at the splice sites, but the intron is invariably located at the same site in the gene, placing the splice sites an invariant distance from the constant structural features of the tRNA bo [...]

Derlin-1; Functional component of endoplasmic reticulum-associated degradation (ERAD) for misfolded lumenal proteins. May act by forming a channel that allows the retrotranslocation of misfolded proteins into the cytosol where they are ubiquitinated and degraded by the proteasome. May mediate the interaction between VCP and the misfolded protein. Also involved in endoplasmic reticulum stress-induced pre-emptive quality control, a mechanism that selectively attenuates the translocation of newly synthesized proteins into the endoplasmic reticulum and reroutes them to the cytosol for prot [...]

Derlin-3; Functional component of endoplasmic reticulum-associated degradation (ERAD) for misfolded lumenal glycoproteins, but not that of misfolded nonglycoproteins. May act by forming a channel that allows the retrotranslocation of misfolded glycoproteins into the cytosol where they are ubiquitinated and degraded by the proteasome. May mediate the interaction between VCP and the misfolded glycoproteins. May be involved in endoplasmic reticulum stress-induced pre-emptive quality control, a mechanism that selectively attenuates the translocation of newly synthesized proteins into the e [...]

Derlin-1; Functional component of endoplasmic reticulum-associated degradation (ERAD) for misfolded lumenal proteins. May act by forming a channel that allows the retrotranslocation of misfolded proteins into the cytosol where they are ubiquitinated and degraded by the proteasome. May mediate the interaction between VCP and the misfolded protein. Also involved in endoplasmic reticulum stress-induced pre-emptive quality control, a mechanism that selectively attenuates the translocation of newly synthesized proteins into the endoplasmic reticulum and reroutes them to the cytosol for prot [...]

Serine/threonine-protein kinase/endoribonuclease IRE1; Senses unfolded proteins in the lumen of the endoplasmic reticulum via its N-terminal domain which leads to enzyme auto- activation. The active endoribonuclease domain splices XBP1 mRNA to generate a new C-terminus, converting it into a potent unfolded-protein response transcriptional activator and triggering growth arrest and apoptosis

Derlin-1; Functional component of endoplasmic reticulum-associated degradation (ERAD) for misfolded lumenal proteins. May act by forming a channel that allows the retrotranslocation of misfolded proteins into the cytosol where they are ubiquitinated and degraded by the proteasome. May mediate the interaction between VCP and the misfolded protein. Also involved in endoplasmic reticulum stress-induced pre-emptive quality control, a mechanism that selectively attenuates the translocation of newly synthesized proteins into the endoplasmic reticulum and reroutes them to the cytosol for prot [...]

Serine/threonine-protein kinase/endoribonuclease IRE2; Induces translational repression through 28S ribosomal RNA cleavage in response to ER stress. Pro-apoptotic. Appears to play no role in the unfolded-protein response, unlike closely related proteins; Belongs to the protein kinase superfamily. Ser/Thr protein kinase family

Derlin-1; Functional component of endoplasmic reticulum-associated degradation (ERAD) for misfolded lumenal proteins. May act by forming a channel that allows the retrotranslocation of misfolded proteins into the cytosol where they are ubiquitinated and degraded by the proteasome. May mediate the interaction between VCP and the misfolded protein. Also involved in endoplasmic reticulum stress-induced pre-emptive quality control, a mechanism that selectively attenuates the translocation of newly synthesized proteins into the endoplasmic reticulum and reroutes them to the cytosol for prot [...]

Heat shock protein HSP 90-alpha; Molecular chaperone that promotes the maturation, structural maintenance and proper regulation of specific target proteins involved for instance in cell cycle control and signal transduction. Undergoes a functional cycle that is linked to its ATPase activity which is essential for its chaperone activity. This cycle probably induces conformational changes in the client proteins, thereby causing their activation. Interacts dynamically with various co-chaperones that modulate its substrate recognition, ATPase cycle and chaperone function. Engages with a ra [...]

Derlin-1; Functional component of endoplasmic reticulum-associated degradation (ERAD) for misfolded lumenal proteins. May act by forming a channel that allows the retrotranslocation of misfolded proteins into the cytosol where they are ubiquitinated and degraded by the proteasome. May mediate the interaction between VCP and the misfolded protein. Also involved in endoplasmic reticulum stress-induced pre-emptive quality control, a mechanism that selectively attenuates the translocation of newly synthesized proteins into the endoplasmic reticulum and reroutes them to the cytosol for prot [...]

78 kDa glucose-regulated protein; Plays a role in facilitating the assembly of multimeric protein complexes inside the endoplasmic reticulum. Involved in the correct folding of proteins and degradation of misfolded proteins via its interaction with DNAJC10, probably to facilitate the release of DNAJC10 from its substrate (By similarity); Belongs to the heat shock protein 70 family

Derlin-1; Functional component of endoplasmic reticulum-associated degradation (ERAD) for misfolded lumenal proteins. May act by forming a channel that allows the retrotranslocation of misfolded proteins into the cytosol where they are ubiquitinated and degraded by the proteasome. May mediate the interaction between VCP and the misfolded protein. Also involved in endoplasmic reticulum stress-induced pre-emptive quality control, a mechanism that selectively attenuates the translocation of newly synthesized proteins into the endoplasmic reticulum and reroutes them to the cytosol for prot [...]

E3 ubiquitin-protein ligase synoviolin; Acts as an E3 ubiquitin-protein ligase which accepts ubiquitin specifically from endoplasmic reticulum-associated UBC7 E2 ligase and transfers it to substrates, promoting their degradation. Component of the endoplasmic reticulum quality control (ERQC) system also called ER-associated degradation (ERAD) involved in ubiquitin-dependent degradation of misfolded endoplasmic reticulum proteins. Also promotes the degradation of normal but naturally short-lived proteins such as SGK. Protects cells from ER stress-induced apoptosis. Protects neurons from [...]