NP_061118.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Related Accession #
Molecular Weight
7,706 Da
NCBI Official Full Name
H/ACA ribonucleoprotein complex subunit 3
NCBI Official Synonym Full Names
NOP10 ribonucleoprotein
NCBI Protein Information
H/ACA ribonucleoprotein complex subunit 3
UniProt Protein Name
H/ACA ribonucleoprotein complex subunit 3
UniProt Synonym Protein Names
Nucleolar protein 10; Nucleolar protein family A member 3; snoRNP protein NOP10
UniProt Synonym Gene Names
UniProt Entry Name
NOP10_HUMAN
NCBI Summary for NOP10
This gene is a member of the H/ACA snoRNPs (small nucleolar ribonucleoproteins) gene family. snoRNPs are involved in various aspects of rRNA processing and modification and have been classified into two families: C/D and H/ACA. The H/ACA snoRNPs also include the DKC1, NOLA1 and NOLA2 proteins. These four H/ACA snoRNP proteins localize to the dense fibrillar components of nucleoli and to coiled (Cajal) bodies in the nucleus. Both 18S rRNA production and rRNA pseudouridylation are impaired if any one of the four proteins is depleted. The four H/ACA snoRNP proteins are also components of the telomerase complex. This gene encodes a protein related to Saccharomyces cerevisiae Nop10p. [provided by RefSeq, Jul 2008]
UniProt Comments for NOP10
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.
Protein type: Nucleolus
Chromosomal Location of Human Ortholog: 15q14-q15
Cellular Component: nucleus; small nucleolar ribonucleoprotein complex; telomerase holoenzyme complex
Molecular Function: protein binding
Biological Process: pseudouridine synthesis; rRNA processing; telomere maintenance via telomerase
Disease: Dyskeratosis Congenita, Autosomal Recessive, 1
Research Articles on NOP10
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Products associated with anti-NOP10 antibody
Pathways associated with anti-NOP10 antibody
Diseases associated with anti-NOP10 antibody
Organs/Tissues associated with anti-NOP10 antibody
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