Javascript is not enabled on this browser. This site will not work properly without Javascript.
PhosphoSitePlus Homepage Cell Signaling Technology
Home | Login
About PhosphoSiteUsing PhosphoSiteprivacy & cookiesCuration ProcessContact
logos LINCs Logo Mt Sinai Logo NIH Logo NCI Logo
Search / Browse Functions
Protein Page:

NOP10 Required for ribosome biogenesis and telomere maintenance. Part of the H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which catalyzes pseudouridylation of rRNA. This involves the isomerization of uridine such that the ribose is subsequently attached to C5, instead of the normal N1. Each rRNA can contain up to 100 pseudouridine (psi) residues, which may serve to stabilize the conformation of rRNAs. May also be required for correct processing or intranuclear trafficking of TERC, the RNA component of the telomerase reverse transcriptase (TERT) holoenzyme. Defects in NOP10 are a cause of dyskeratosis congenita autosomal recessive type 1 (DKCB1). A rare multisystem disorder caused by defective telomere maintenance. It is characterized by progressive bone marrow failure, and the clinical triad of reticulated skin hyperpigmentation, nail dystrophy, and mucosal leukoplakia. Common but variable features include premature graying, aplastic anemia, low platelets, osteoporosis, pulmonary fibrosis, and liver fibrosis among others. Early mortality is often associated with bone marrow failure, infections, fatal pulmonary complications, or malignancy. Belongs to the NOP10 family. Note: This description may include information from UniProtKB.
Protein type: Nucleolus
Chromosomal Location of Human Ortholog: 15q14
Cellular Component: nuclear body; nucleoplasm; small nucleolar ribonucleoprotein complex; telomerase holoenzyme complex
Molecular Function: protein binding; RNA binding
Biological Process: pseudouridine synthesis; rRNA pseudouridine synthesis; snoRNA guided rRNA pseudouridine synthesis; snRNA pseudouridine synthesis; telomere maintenance via telomerase
Disease: Dyskeratosis Congenita, Autosomal Recessive, 1
Reference #:  Q9NPE3 (UniProtKB)
Alt. Names/Synonyms: homolog of yeast Nop10p; MGC70651; NOLA3; NOP10; NOP10 ribonucleoprotein homolog (yeast); NOP10P; nucleolar protein family A, member 3; nucleolar protein family A, member 3 (H/ACA small nucleolar RNPs); Q9NPE3
Gene Symbols: NOP10
Molecular weight: 7,706 Da
Basal Isoelectric point: 10.01  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

STRING  |  cBioPortal  |  Wikipedia  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Scansite  |  Pfam  |  Phospho.ELM  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene  |  Ensembl Protein