| IED ID | IndEnz0002015395 |
| Enzyme Type ID | protease015395 |
| Protein Name |
Exosome component 10 EC 3.1.13.- Autoantigen PM/Scl 2 P100 polymyositis-scleroderma overlap syndrome-associated autoantigen Polymyositis/scleroderma autoantigen 100 kDa PM/Scl-100 Polymyositis/scleroderma autoantigen 2 |
| Gene Name | EXOSC10 PMSCL PMSCL2 RRP6 |
| Organism | Homo sapiens (Human) |
| Taxonomic Lineage | cellular organisms Eukaryota Opisthokonta Metazoa Eumetazoa Bilateria Deuterostomia Chordata Craniata Vertebrata Gnathostomata (jawed vertebrates) Teleostomi Euteleostomi Sarcopterygii Dipnotetrapodomorpha Tetrapoda Amniota Mammalia Theria Eutheria Boreoeutheria Euarchontoglires Primates Haplorrhini Simiiformes Catarrhini Hominoidea (apes) Hominidae (great apes) Homininae Homo Homo sapiens (Human) |
| Enzyme Sequence | MAPPSTREPRVLSATSATKSDGEMVLPGFPDADSFVKFALGSVVAVTKASGGLPQFGDEYDFYRSFPGFQAFCETQGDRLLQCMSRVMQYHGCRSNIKDRSKVTELEDKFDLLVDANDVILERVGILLDEASGVNKNQQPVLPAGLQVPKTVVSSWNRKAAEYGKKAKSETFRLLHAKNIIRPQLKFREKIDNSNTPFLPKIFIKPNAQKPLPQALSKERRERPQDRPEDLDVPPALADFIHQQRTQQVEQDMFAHPYQYELNHFTPADAVLQKPQPQLYRPIEETPCHFISSLDELVELNEKLLNCQEFAVDLEHHSYRSFLGLTCLMQISTRTEDFIIDTLELRSDMYILNESLTDPAIVKVFHGADSDIEWLQKDFGLYVVNMFDTHQAARLLNLGRHSLDHLLKLYCNVDSNKQYQLADWRIRPLPEEMLSYARDDTHYLLYIYDKMRLEMWERGNGQPVQLQVVWQRSRDICLKKFIKPIFTDESYLELYRKQKKHLNTQQLTAFQLLFAWRDKTARREDESYGYVLPNHMMLKIAEELPKEPQGIIACCNPVPPLVRQQINEMHLLIQQAREMPLLKSEVAAGVKKSGPLPSAERLENVLFGPHDCSHAPPDGYPIIPTSGSVPVQKQASLFPDEKEDNLLGTTCLIATAVITLFNEPSAEDSKKGPLTVAQKKAQNIMESFENPFRMFLPSLGHRAPVSQAAKFDPSTKIYEISNRWKLAQVQVQKDSKEAVKKKAAEQTAAREQAKEACKAAAEQAISVRQQVVLENAAKKRERATSDPRTTEQKQEKKRLKISKKPKDPEPPEKEFTPYDYSQSDFKAFAGNSKSKVSSQFDPNKQTPSGKKCIAAKKIKQSVGNKSMSFPTGKSDRGFRYNWPQR |
| Enzyme Length | 885 |
| Uniprot Accession Number | Q01780 |
| Absorption | |
| Active Site | |
| Activity Regulation | |
| Binding Site | |
| Calcium Binding | |
| catalytic Activity | |
| DNA Binding | |
| EC Number | 3.1.13.- |
| Enzyme Function | FUNCTION: Putative catalytic component of the RNA exosome complex which has 3'->5' exoribonuclease activity and participates in a multitude of cellular RNA processing and degradation events. In the nucleus, the RNA exosome complex is involved in proper maturation of stable RNA species such as rRNA, snRNA and snoRNA, in the elimination of RNA processing by-products and non-coding 'pervasive' transcripts, such as antisense RNA species and promoter-upstream transcripts (PROMPTs), and of mRNAs with processing defects, thereby limiting or excluding their export to the cytoplasm. The RNA exosome may be involved in Ig class switch recombination (CSR) and/or Ig variable region somatic hypermutation (SHM) by targeting AICDA deamination activity to transcribed dsDNA substrates. In the cytoplasm, the RNA exosome complex is involved in general mRNA turnover and specifically degrades inherently unstable mRNAs containing AU-rich elements (AREs) within their 3' untranslated regions, and in RNA surveillance pathways, preventing translation of aberrant mRNAs. It seems to be involved in degradation of histone mRNA. EXOSC10 has 3'-5' exonuclease activity (By similarity). EXOSC10 is required for nucleolar localization of C1D and probably mediates the association of MTREX, C1D and MPHOSPH6 with the RNA exosome involved in the maturation of 5.8S rRNA. {ECO:0000250, ECO:0000269|PubMed:14527413, ECO:0000269|PubMed:16455498, ECO:0000269|PubMed:17412707, ECO:0000269|PubMed:17545563, ECO:0000269|PubMed:18172165, ECO:0000269|PubMed:19056938, ECO:0000269|PubMed:20368444, ECO:0000269|PubMed:20699273}. |
| Temperature Dependency | |
| PH Dependency | |
| Pathway | |
| nucleotide Binding | |
| Features | Alternative sequence (1); Beta strand (12); Chain (1); Compositional bias (2); Cross-link (8); Domain (1); Helix (24); Modified residue (1); Region (1); Turn (2) |
| Keywords | 3D-structure;Alternative splicing;Cytoplasm;Exonuclease;Exosome;Hydrolase;Isopeptide bond;Nonsense-mediated mRNA decay;Nuclease;Nucleus;Phosphoprotein;RNA-binding;Reference proteome;Ubl conjugation;rRNA processing |
| Interact With | Q13901; P17844; Q9Y2L1; Q9NPD3; Q9NQT4; Q99547 |
| Induction | |
| Subcellular Location | SUBCELLULAR LOCATION: Cytoplasm. Nucleus, nucleolus. Nucleus. Note=Strongly enriched in the nucleolus and a small amount has been found in cytoplasm supporting the existence of a nucleolar RNA exosome complex form. |
| Modified Residue | MOD_RES 821; /note=Phosphoserine; /evidence=ECO:0007744|PubMed:23186163 |
| Post Translational Modification | |
| Signal Peptide | |
| Structure 3D | NMR spectroscopy (1); Electron microscopy (3); X-ray crystallography (3) |
| Cross Reference PDB | 2CPR; 3SAF; 3SAG; 3SAH; 6D6Q; 6D6R; 7MQA; |
| Mapped Pubmed ID | 11719186; 11812149; 12150911; 12419256; 12788944; 14743216; 14872500; 16396833; 18065403; 18438418; 19103309; 19220911; 20195357; 20467437; 20562859; 20711500; 20805244; 21182203; 21182205; 21255825; 21705430; 21907836; 22261302; 22980978; 23143267; 23414517; 23439679; 23482395; 23756462; 23902751; 24189400; 25609649; 25665578; 25969445; 26496610; 26612606; 26687677; 26857222; 30575725; 30840897; 31086179; 32350160; 33580257; 34059908; 34516797; |
| Motif | |
| Gene Encoded By | |
| Mass | 100,831 |
| Kinetics | |
| Metal Binding | |
| Rhea ID | |
| Cross Reference Brenda |