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Protein Page:
NOP10 (human)

Overview
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
Cellular Component: Cajal body; small nucleolar ribonucleoprotein complex; nucleolus
Molecular Function: snoRNA binding; protein binding
Biological Process: pseudouridine synthesis; rRNA processing
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

NOP10

Protein Structure Not Found.


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Modification Sites and Domains  

Modification Sites in Parent Protein, Orthologs, and Isoforms  
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
0 1 T16 EQGDRVYTLKKFDPM
0 1 S36-p SAHPARFsPDDKYSR
  mouse

 
T16-p EQGDRVYtLKKFDPM
S36 SAHPARFSPDDKYSR
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