NOP10

Functionⁱ

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.1 Publication

GO - Molecular functionⁱ

box H/ACA snoRNA binding
Source: BHF-UCL
Inferred from physical interactionⁱ
- 18082603
RNA binding
Source: BHF-UCL
Inferred from physical interactionⁱ
- 18082603
telomerase RNA binding
Source: BHF-UCL
Inferred from physical interactionⁱ
- 18082603

View the complete GO annotation on QuickGO ...

GO - Biological processⁱ

positive regulation of telomerase RNA localization to Cajal body
Source: BHF-UCL
Inferred from high throughput mutant phenotypeⁱ
- 25467444
pseudouridine synthesis
Source: UniProtKB
Non-traceable author statementⁱ
- 9843512
rRNA pseudouridine synthesis
Source: GO_Central
Inferred from biological aspect of ancestorⁱ
- 21873635
snoRNA guided rRNA pseudouridine synthesis Source: Ensembl
snRNA pseudouridine synthesis
Source: GO_Central
Inferred from biological aspect of ancestorⁱ
- 21873635
telomere maintenance via telomerase
Source: UniProtKB
Inferred from direct assayⁱ
- 29695869

View the complete GO annotation on QuickGO ...

Keywordsⁱ

Molecular function	Ribonucleoprotein
Biological process	Ribosome biogenesis, rRNA processing

Enzyme and pathway databases

Reactomeⁱ	R-HSA-6790901 rRNA modification in the nucleus and cytosol

Reactomeⁱ

R-HSA-6790901 rRNA modification in the nucleus and cytosol

Names & Taxonomyⁱ

Protein namesⁱ	Recommended name: H/ACA ribonucleoprotein complex subunit 3 Alternative name(s): Nucleolar protein 10 Nucleolar protein family A member 3 snoRNP protein NOP10
Gene namesⁱ	Name:NOP10 Synonyms:NOLA3
Organismⁱ	Homo sapiens (Human)
Taxonomic identifierⁱ	9606 [NCBI]
Taxonomic lineageⁱ	cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Dipnotetrapodomorpha › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Boreoeutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo
Proteomesⁱ	UP000005640 Componentⁱ: Chromosome 15

Organism-specific databases

EuPathDBⁱ	HostDB:ENSG00000182117.5
Human Gene Nomenclature Database More...HGNCⁱ	HGNC:14378 NOP10
MIMⁱ	606471 gene
neXtProtⁱ	NX_Q9NPE3

Keywords - Cellular componentⁱ

Nucleus

Pathology & Biotechⁱ

Involvement in diseaseⁱ

Dyskeratosis congenita, autosomal recessive, 1 (DKCB1)1 Publication

The disease is caused by mutations affecting the gene represented in this entry.

Disease descriptionA 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.

Keywords - Diseaseⁱ

Disease mutation, Dyskeratosis congenita

Organism-specific databases

DisGeNET More...DisGeNETⁱ	55505
GeneReviewsⁱ	NOP10
MalaCards human disease database More...MalaCardsⁱ	NOP10
MIMⁱ	224230 phenotype
Open Targets More...OpenTargetsⁱ	ENSG00000182117
Orphanetⁱ	1775 Dyskeratosis congenita
PharmGKBⁱ	PA164723973

Polymorphism and mutation databases

BioMutaⁱ	NOP10
DMDMⁱ	54036209

PTM / Processingⁱ

Molecule processing

Feature key	Position(s)	DescriptionActions	Graphical view	Length
ChainⁱPRO_0000149001	1 – 64	H/ACA ribonucleoprotein complex subunit 3Add BLAST		64

Proteomic databases

EPDⁱ	Q9NPE3
MaxQB - The MaxQuant DataBase More...MaxQBⁱ	Q9NPE3
PaxDbⁱ	Q9NPE3
PeptideAtlas More...PeptideAtlasⁱ	Q9NPE3
PRIDEⁱ	Q9NPE3
ProteomicsDBⁱ	81979
TopDownProteomicsⁱ	Q9NPE3

PTM databases

iPTMnetⁱ	Q9NPE3
PhosphoSitePlusⁱ	Q9NPE3

Expressionⁱ

Gene expression databases

Bgeeⁱ	ENSG00000182117 Expressed in 228 organ(s), highest expression level in oocyte
CleanExⁱ	HS_NOP10
ExpressionAtlasⁱ	Q9NPE3 baseline and differential
Genevisibleⁱ	Q9NPE3 HS

Organism-specific databases

Human Protein Atlas More...HPAⁱ	HPA059198

HPAⁱ

HPA059198

Interactionⁱ

Subunit structureⁱ

Part of the H/ACA small nucleolar ribonucleoprotein (H/ACA snoRNP) complex, which contains NHP2/NOLA2, GAR1/NOLA1, NOP10/NOLA3, and DKC1/NOLA4, which is presumed to be the catalytic subunit (PubMed:11074001). The complex contains a stable core formed by binding of one or two NOP10-DKC1 heterodimers to NHP2; GAR1 subsequently binds to this core via DKC1 (PubMed:11074001). The complex binds a box H/ACA small nucleolar RNA (snoRNA), which may target the specific site of modification within the RNA substrate (PubMed:16601202). During assembly, the complex contains NAF1 instead of GAR1/NOLA1 (PubMed:11074001, PubMed:16601202). The complex also interacts with TERC, which contains a 3'-terminal domain related to the box H/ACA snoRNAs (PubMed:11074001, PubMed:16601202). Specific interactions with snoRNAs or TERC are mediated by GAR1 and NHP2 (PubMed:11074001, PubMed:16601202). Associates with NOLC1/NOPP140 (PubMed:11074001, PubMed:16601202). H/ACA snoRNPs interact with the SMN complex, consisting of SMN1 or SMN2, GEMIN2/SIP1, DDX20/GEMIN3, and GEMIN4 (PubMed:11074001, PubMed:16601202). This is mediated by interaction between GAR1 and SMN1 or SMN2 (PubMed:11074001, PubMed:16601202). The SMN complex may be required for correct assembly of the H/ACA snoRNP complex (PubMed:11074001, PubMed:16601202). Component of the telomerase holoenzyme complex composed of one molecule of TERT, one molecule of WRAP53/TCAB1, two molecules of H/ACA ribonucleoprotein complex subunits DKC1, NOP10, NHP2 and GAR1, and a telomerase RNA template component (TERC) (PubMed:19179534, PubMed:20351177, PubMed:29695869). The telomerase holoenzyme complex is associated with TEP1, SMG6/EST1A and POT1 (PubMed:19179534).5 Publications

Binary interactionsⁱ

With	Entry	#Exp.	IntAct	Notes
NHP2	Q9NX24	8	EBI-1642169,EBI-1050064

With

Entry

#Exp.

IntAct

Notes

NHP2

Q9NX24

8

EBI-1642169,EBI-1050064

Protein-protein interaction databases

BioGridⁱ	120685, 32 interactors
ComplexPortalⁱ	CPX-265 Telomerase holoenzyme complex
CORUMⁱ	Q9NPE3
Database of interacting proteins More...DIPⁱ	DIP-40092N
IntActⁱ	Q9NPE3, 24 interactors
Molecular INTeraction database More...MINTⁱ	Q9NPE3
STRINGⁱ	9606.ENSP00000332198

Structureⁱ

3D structure databases

ProteinModelPortalⁱ	Q9NPE3
SMRⁱ	Q9NPE3
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Family & Domainsⁱ

Sequence similaritiesⁱ

Belongs to the NOP10 family.Curated

Phylogenomic databases

eggNOGⁱ	KOG3503 Eukaryota COG2260 LUCA
Ensembl GeneTree More...GeneTreeⁱ	ENSGT00390000012563
HOGENOMⁱ	HOG000159681
HOVERGENⁱ	HBG080280
InParanoidⁱ	Q9NPE3
KEGG Orthology (KO) More...KOⁱ	K11130
OMAⁱ	HLMYTLG
Database of Orthologous Groups More...OrthoDBⁱ	EOG091G1AP5
PhylomeDBⁱ	Q9NPE3
TreeFamⁱ	TF300211

Family and domain databases

Gene3Dⁱ	2.20.28.40, 1 hit
InterProⁱ	View protein in InterPro IPR007264 H/ACA_rnp_Nop10 IPR036756 H/ACA_rnp_Nop10_sf
PANTHERⁱ	PTHR13305 PTHR13305, 1 hit
Pfam protein domain database More...Pfamⁱ	View protein in Pfam PF04135 Nop10p, 1 hit
SUPFAMⁱ	SSF144210 SSF144210, 1 hit

Sequence (1+)ⁱ

Sequence statusⁱ: Complete.

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show all Align All

Q9NPE3-1 [UniParc]FASTA Add to basket

« Hide

        10         20         30         40         50
MFLQYYLNEQ GDRVYTLKKF DPMGQQTCSA HPARFSPDDK YSRHRITIKK 
        60 
RFKVLMTQQP RPVL

Length:64

Mass (Da):7,706

Last modified:October 1, 2000 - v1

Checksum:ⁱ62E9BCFFB27036FA

GO

Computationally mapped potential isoform sequencesⁱ

There is 1 potential isoform mapped to this entry.BLAST Align Show all Add to basket

	Entry	Entry name		Protein names	Gene names	Length	Annotation

	H0YM60	H0YM60_HUMAN		H/ACA ribonucleoprotein complex sub... H/ACA ribonucleoprotein complex subunit 3	NOP10	36	Annotation score:

Natural variant

Feature key	Position(s)	DescriptionActions	Graphical view	Length
Natural variantⁱVAR_043725	34	R → W in DKCB1. 1 PublicationCorresponds to variant dbSNP:rs121908092EnsemblClinVar.		1

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AB043103 Genomic DNA Translation: BAA96107.1 AB043104 mRNA Translation: BAA96133.1 BC008886 mRNA Translation: AAH08886.1 BC063023 mRNA Translation: AAH63023.1
The Consensus CDS (CCDS) project More...CCDSⁱ	CCDS10037.1
NCBI Reference Sequences More...RefSeqⁱ	NP_061118.1, NM_018648.3
UniGeneⁱ	Hs.14317

Genome annotation databases

Ensemblⁱ	ENST00000328848; ENSP00000332198; ENSG00000182117
GeneIDⁱ	55505
KEGGⁱ	hsa:55505
UCSC genome browser More...UCSCⁱ	uc001zie.2 human

Similar proteinsⁱ

100% Identity
90% Identity
50% Identity

Protein	Similar proteins		Species	Score	Length	Source
Q9NPE3	H/ACA ribonucleoprotein complex subunit 3		MOUSE		64	UniRef100_Q9NPE3
NOP10 isoform 1		PANTR		64
NOP10 isoform 1		PONAB		64
Uncharacterized protein		PANPA		64
Uncharacterized protein		NOMLE		64
+3

Protein	Similar proteins		Species	Score	Length	Source
Q9NPE3	H/ACA ribonucleoprotein complex subunit 3		MOUSE		64	UniRef90_Q9NPE3
NOP10 isoform 1		PANTR		64
NOP10 isoform 1		PONAB		64
Uncharacterized protein		PANPA		64
Uncharacterized protein		NOMLE		64
+80

Protein	Similar proteins		Species	Score	Length	Source
Q9NPE3	H/ACA ribonucleoprotein complex subunit 3		MOUSE		64	UniRef50_Q9NPE3
NOP10 isoform 1		PANTR		64
NOP10 isoform 1		PONAB		64
Uncharacterized protein		PANPA		64
Uncharacterized protein		NOMLE		64
+107

Cross-referencesⁱ

Sequence databases

Select the link destinations: EMBLⁱ GenBankⁱ DDBJⁱ Links Updated	AB043103 Genomic DNA Translation: BAA96107.1 AB043104 mRNA Translation: BAA96133.1 BC008886 mRNA Translation: AAH08886.1 BC063023 mRNA Translation: AAH63023.1
CCDSⁱ	CCDS10037.1
RefSeqⁱ	NP_061118.1, NM_018648.3
UniGeneⁱ	Hs.14317

3D structure databases

ProteinModelPortalⁱ	Q9NPE3
SMRⁱ	Q9NPE3
ModBaseⁱ	Search...
MobiDBⁱ	Search...

Protein-protein interaction databases

BioGridⁱ	120685, 32 interactors
ComplexPortalⁱ	CPX-265 Telomerase holoenzyme complex
CORUMⁱ	Q9NPE3
DIPⁱ	DIP-40092N
IntActⁱ	Q9NPE3, 24 interactors
MINTⁱ	Q9NPE3
STRINGⁱ	9606.ENSP00000332198

PTM databases

iPTMnetⁱ	Q9NPE3
PhosphoSitePlusⁱ	Q9NPE3

Polymorphism and mutation databases

BioMutaⁱ	NOP10
DMDMⁱ	54036209

Proteomic databases

EPDⁱ	Q9NPE3
MaxQBⁱ	Q9NPE3
PaxDbⁱ	Q9NPE3
PeptideAtlasⁱ	Q9NPE3
PRIDEⁱ	Q9NPE3
ProteomicsDBⁱ	81979
TopDownProteomicsⁱ	Q9NPE3

Protocols and materials databases

Structural Biology Knowledgebase	Search...

Structural Biology Knowledgebase

Search...

Genome annotation databases

Ensemblⁱ	ENST00000328848; ENSP00000332198; ENSG00000182117
GeneIDⁱ	55505
KEGGⁱ	hsa:55505
UCSCⁱ	uc001zie.2 human

Organism-specific databases

CTDⁱ	55505
DisGeNETⁱ	55505
EuPathDBⁱ	HostDB:ENSG00000182117.5
GeneCardsⁱ	NOP10
GeneReviewsⁱ	NOP10
HGNCⁱ	HGNC:14378 NOP10
HPAⁱ	HPA059198
MalaCardsⁱ	NOP10
MIMⁱ	224230 phenotype 606471 gene
neXtProtⁱ	NX_Q9NPE3
OpenTargetsⁱ	ENSG00000182117
Orphanetⁱ	1775 Dyskeratosis congenita
PharmGKBⁱ	PA164723973
GenAtlas: human gene database More...GenAtlasⁱ	Search...

Phylogenomic databases

eggNOGⁱ	KOG3503 Eukaryota COG2260 LUCA
GeneTreeⁱ	ENSGT00390000012563
HOGENOMⁱ	HOG000159681
HOVERGENⁱ	HBG080280
InParanoidⁱ	Q9NPE3
KOⁱ	K11130
OMAⁱ	HLMYTLG
OrthoDBⁱ	EOG091G1AP5
PhylomeDBⁱ	Q9NPE3
TreeFamⁱ	TF300211

Enzyme and pathway databases

Reactomeⁱ	R-HSA-6790901 rRNA modification in the nucleus and cytosol

Reactomeⁱ

R-HSA-6790901 rRNA modification in the nucleus and cytosol

Miscellaneous databases

ChiTaRSⁱ	NOP10 human
GenomeRNAiⁱ	55505
Protein Ontology More...PROⁱ	PR:Q9NPE3
SOURCEⁱ	Search...

Gene expression databases

Bgeeⁱ	ENSG00000182117 Expressed in 228 organ(s), highest expression level in oocyte
CleanExⁱ	HS_NOP10
ExpressionAtlasⁱ	Q9NPE3 baseline and differential
Genevisibleⁱ	Q9NPE3 HS

Family and domain databases

Gene3Dⁱ	2.20.28.40, 1 hit
InterProⁱ	View protein in InterPro IPR007264 H/ACA_rnp_Nop10 IPR036756 H/ACA_rnp_Nop10_sf
PANTHERⁱ	PTHR13305 PTHR13305, 1 hit
Pfamⁱ	View protein in Pfam PF04135 Nop10p, 1 hit
SUPFAMⁱ	SSF144210 SSF144210, 1 hit
ProtoNetⁱ	Search...

Entry informationⁱ

Entry nameⁱ	NOP10_HUMAN
Accessionⁱ	Q9NPE3Primary (citable) accession number: Q9NPE3
Entry historyⁱ	Integrated into UniProtKB/Swiss-Prot:	October 11, 2004
	Last sequence update:	October 1, 2000
	Last modified:	November 7, 2018
	This is version 140 of the entry and version 1 of the sequence. See complete history.
Entry statusⁱ	Reviewed (UniProtKB/Swiss-Prot)
Annotation program	Chordata Protein Annotation Program
Disclaimer	Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousⁱ

Keywords - Technical termⁱ

Complete proteome, Reference proteome

Documents

SIMILARITY comments
Index of protein domains and families
Human entries with polymorphisms or disease mutations
List of human entries with polymorphisms or disease mutations
Human polymorphisms and disease mutations
Index of human polymorphisms and disease mutations
MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
Human chromosome 15
Human chromosome 15: entries, gene names and cross-references to MIM

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UniProtKB - Q9NPE3 (NOP10_HUMAN)

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H/ACA ribonucleoprotein complex subunit 3

Select a section on the left to see content.

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

GO - Biological processi

Enzyme and pathway databases

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

Organism-specific databases

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Nucleus

Nucleus

Other locations

Keywords - Cellular componenti

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

Keywords - Diseasei

Organism-specific databases

Polymorphism and mutation databases

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Proteomic databases

PTM databases

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Organism-specific databases

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

Protein-protein interaction databases

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Phylogenomic databases

Family and domain databases

Natural variant

Sequence databases

Genome annotation databases

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

3D structure databases

Protein-protein interaction databases

PTM databases

Polymorphism and mutation databases

Proteomic databases

Protocols and materials databases

Genome annotation databases

Organism-specific databases

Phylogenomic databases

Enzyme and pathway databases

Miscellaneous databases

Gene expression databases

Family and domain databases

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

Documents

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Functionⁱ

GO - Molecular functionⁱ

GO - Biological processⁱ

Names & Taxonomyⁱ

Subcellular locationⁱ

Keywords - Cellular componentⁱ

Pathology & Biotechⁱ

Keywords - Diseaseⁱ

PTM / Processingⁱ

Expressionⁱ

Interactionⁱ

Structureⁱ

Family & Domainsⁱ

Sequence similaritiesⁱ

Cross-referencesⁱ

Entry informationⁱ

Miscellaneousⁱ

Keywords - Technical termⁱ