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UniProtKB - Q9NQH7 (XPP3_HUMAN)

Entry version 170 (17 Jun 2020)
Sequence version 1 (01 Oct 2000)
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Protein

Xaa-Pro aminopeptidase 3

Gene

XPNPEP3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the 'protein existence' evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<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

Catalyzes the removal of a penultimate prolyl residue from the N-termini of peptides, such as Leu-Pro-Ala (PubMed:25609706, PubMed:28476889). Also shows low activity towards peptides with Ala or Ser at the P1 position (PubMed:28476889).2 Publications
Promotes TNFRSF1B-mediated phosphorylation of MAPK8/JNK1 and MAPK9/JNK2, suggesting a function as an adapter protein for TNFRSF1B; the effect is independent of XPNPEP3 peptidase activity. May inhibit apoptotic cell death induced via TNF-TNFRSF1B signaling.1 Publication

<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<p>This subsection of the 'Function' section provides information relevant to cofactors. A cofactor is any non-protein substance required for a protein to be catalytically active. Some cofactors are inorganic, such as the metal atoms zinc, iron, and copper in various oxidation states. Others, such as most vitamins, are organic.<p><a href='/help/cofactor' target='_top'>More...</a></p>Cofactori

Mn2+1 PublicationNote: Binds 2 manganese ions per subunit.1 Publication

<p>This subsection of the 'Function' section describes biophysical and chemical properties, such as maximal absorption, kinetic parameters, pH dependence, redox potentials and temperature dependence.<p><a href='/help/biophysicochemical_properties' target='_top'>More...</a></p>Kineticsi

  1. KM=1.62 mM for Met-Pro-Ala1 Publication
  2. KM=2.45 mM for Leu-Pro-Ala1 Publication
  3. KM=3.06 mM for Phe-Pro-Ala1 Publication
  4. KM=0.55 mM for Tyr-Pro-Ala1 Publication
  5. KM=4.91 mM for Tyr-Ser-Ser1 Publication
  6. KM=4.20 mM for Tyr-Ala-Ala1 Publication
  7. KM=3.74 mM for Tyr-Ser-Ser-Ala-Ala-Ala-Ala1 Publication

    Sites

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section describes the interaction between a single amino acid and another chemical entity. Priority is given to the annotation of physiological ligands.<p><a href='/help/binding' target='_top'>More...</a></p>Binding sitei300SubstrateCombined sources1 Publication1
    <p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section indicates at which position the protein binds a given metal ion. The nature of the metal is indicated in the 'Description' field.<p><a href='/help/metal' target='_top'>More...</a></p>Metal bindingi331Manganese 2Combined sources1 Publication1
    Binding sitei331SubstrateCombined sources1 Publication1
    Metal bindingi342Manganese 1Combined sources1 Publication1
    Metal bindingi342Manganese 2Combined sources1 Publication1
    Binding sitei342SubstrateCombined sources1 Publication1 Publication1
    Metal bindingi424Manganese 1Combined sources1 Publication1
    Binding sitei424SubstrateCombined sources1 Publication1
    Binding sitei431SubstrateCombined sources1 Publication1
    Metal bindingi451Manganese 1Combined sources1 Publication1
    Binding sitei451SubstrateCombined sources1 Publication1
    Metal bindingi475Manganese 1Combined sources1 Publication1
    Metal bindingi475Manganese 2Combined sources1 Publication1
    Binding sitei475SubstrateCombined sources1 Publication1 Publication1

    <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

    Complete GO annotation on QuickGO ...

    GO - Biological processi

    Complete GO annotation on QuickGO ...

    <p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

    Molecular functionAminopeptidase, Hydrolase, Metalloprotease, Protease
    LigandManganese, Metal-binding

    Enzyme and pathway databases

    BRENDA Comprehensive Enzyme Information System

    More...    BRENDAi    		3.4.11.9 2681

    Protein family/group databases

    MEROPS protease database

    More...    MEROPSi    		M24.026

    <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

    <p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
    Recommended name:
    Xaa-Pro aminopeptidase 3Imported (EC:3.4.11.92 Publications)
    Short name:
    X-Pro aminopeptidase 3
    Alternative name(s):
    Aminopeptidase P3
    Short name:
    APP3
    <p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: 'Name', 'Synonyms', 'Ordered locus names' and 'ORF names'.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
    Name:XPNPEP3
    <p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
    <p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the 'taxonomic identifier' or 'taxid'.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
    <p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
    <p>This subsection of the <a href="http://www.uniprot.org/help/names%5Fand%5Ftaxonomy%5Fsection">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
    • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes%5Fmanual">proteome</a> can consist of several components.<br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 22

    Organism-specific databases

    Eukaryotic Pathogen Database Resources

    More...    EuPathDBi    		HostDB:ENSG00000196236.12

    Human Gene Nomenclature Database

    More...    HGNCi    		HGNC:28052 XPNPEP3

    Online Mendelian Inheritance in Man (OMIM)

    More...    MIMi    		613553 gene

    neXtProt; the human protein knowledge platform

    More...    neXtProti    		NX_Q9NQH7

    <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

    Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte & Seán O’Donoghue; Source: COMPARTMENTS

    Keywords - Cellular componenti

    Cytoplasm, Mitochondrion

    <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

    <p>This subsection of the 'Pathology and Biotech' section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

    Nephronophthisis-like nephropathy 1 (NPHPL1)1 Publication
    The disease is caused by mutations affecting the gene represented in this entry.
    Disease descriptionA disorder with features of nephronophthisis, a cystic kidney disease leading to end-stage renal failure. Nephronophthisis is histologically characterized by modifications of the tubules with thickening of the basement membrane, interstitial fibrosis and, in the advanced stages, medullary cysts. Typical clinical manifestation are chronic renal failure, anemia, polyuria, polydipsia, isosthenuria, and growth retardation. Associations with extrarenal symptoms are frequent. In NPHPL1 patients, extrarenal symptoms include hypertension, essential tremor, sensorineural hearing loss and gout. Severely affected individuals can manifest a mitochondrial disorder with isolated complex I deficiency activity in muscle, seizures, mental retardation and hypertrophic dilated cardiomyopathy.
    Related information in OMIM
    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the 'Sequence' section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_063820453G → C in NPHPL1. 1 PublicationCorresponds to variant dbSNP:rs267607179EnsemblClinVar.1

    Mutagenesis

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the <a href="http://www.uniprot.org/manual/pathology%5Fand%5Fbiotech%5Fsection">'Pathology and Biotech'</a> section describes the effect of the experimental mutation of one or more amino acid(s) on the biological properties of the protein.<p><a href='/help/mutagen' target='_top'>More...</a></p>Mutagenesisi18R → A: Prevents cleavage of N-terminal transit peptide; when associated with A-29-30-A; A-39-40-A and A-44. 1 Publication1
    Mutagenesisi29 – 30RR → AA: Prevents cleavage of N-terminal transit peptide; when associated with A-18; A-39-40-A and A-44. 1 Publication2
    Mutagenesisi39 – 40RR → AA: Prevents cleavage of N-terminal transit peptide; when associated with A-18; A-29-30-A and A-44. 1 Publication2
    Mutagenesisi44R → A: Prevents cleavage of N-terminal transit peptide; when associated with A-18; A-29-30-A and A-39-40-A. 1 Publication1
    Mutagenesisi314H → A: Impairs catalytic activity. 1 Publication1
    Mutagenesisi342D → A: Impairs catalytic activity. 1 Publication1
    Mutagenesisi475E → A: Impairs catalytic activity. 1 Publication1

    Keywords - Diseasei

    Disease mutation, Nephronophthisis

    Organism-specific databases

    DisGeNET

    More...    DisGeNETi    		63929

    GeneReviews a resource of expert-authored, peer-reviewed disease descriptions.

    More...    GeneReviewsi    		XPNPEP3

    MalaCards human disease database

    More...    MalaCardsi    		XPNPEP3
    MIMi613159 phenotype

    Open Targets

    More...    OpenTargetsi    		ENSG00000196236

    Orphanet; a database dedicated to information on rare diseases and orphan drugs

    More...    Orphaneti    		93589 Late-onset nephronophthisis

    The Pharmacogenetics and Pharmacogenomics Knowledge Base

    More...    PharmGKBi    		PA147357130

    Miscellaneous databases

    Pharos NIH Druggable Genome Knowledgebase

    More...    Pharosi    		Q9NQH7 Tbio

    Chemistry databases

    ChEMBL database of bioactive drug-like small molecules

    More...    ChEMBLi    		CHEMBL3831223

    Polymorphism and mutation databases

    BioMuta curated single-nucleotide variation and disease association database

    More...    BioMutai    		XPNPEP3

    Domain mapping of disease mutations (DMDM)

    More...    DMDMi    		74761652

    <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

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the 'PTM / Processing' section describes the extent of a transit peptide.<p><a href='/help/transit' target='_top'>More...</a></p>Transit peptidei1 – 31MitochondrionSequence analysis1 PublicationAdd BLAST31
    <p>This subsection of the 'PTM / Processing' section describes the extent of a polypeptide chain in the mature protein following processing or proteolytic cleavage.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000025565432 – 507Xaa-Pro aminopeptidase 3Add BLAST476

    Proteomic databases

    Encyclopedia of Proteome Dynamics

    More...    EPDi    		Q9NQH7

    jPOST - Japan Proteome Standard Repository/Database

    More...    jPOSTi    		Q9NQH7

    MassIVE - Mass Spectrometry Interactive Virtual Environment

    More...    MassIVEi    		Q9NQH7

    MaxQB - The MaxQuant DataBase

    More...    MaxQBi    		Q9NQH7

    PaxDb, a database of protein abundance averages across all three domains of life

    More...    PaxDbi    		Q9NQH7

    PeptideAtlas

    More...    PeptideAtlasi    		Q9NQH7

    PRoteomics IDEntifications database

    More...    PRIDEi    		Q9NQH7

    ProteomicsDB: a multi-organism proteome resource

    More...    ProteomicsDBi    		82154 [Q9NQH7-1]
    82155 [Q9NQH7-2]
    82156 [Q9NQH7-3]
    82157 [Q9NQH7-4]
    82158 [Q9NQH7-5]

    PTM databases

    iPTMnet integrated resource for PTMs in systems biology context

    More...    iPTMneti    		Q9NQH7

    Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

    More...    PhosphoSitePlusi    		Q9NQH7

    <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

    <p>This subsection of the 'Expression' section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified 'at protein level'.<br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

    Isoform 1 and isoform 2 are widely expressed, with isoform 1 being more abundant.1 Publication

    Gene expression databases

    Bgee dataBase for Gene Expression Evolution

    More...    Bgeei    		ENSG00000196236 Expressed in testis and 201 other tissues

    ExpressionAtlas, Differential and Baseline Expression

    More...    ExpressionAtlasi    		Q9NQH7 baseline and differential

    Genevisible search portal to normalized and curated expression data from Genevestigator

    More...    Genevisiblei    		Q9NQH7 HS

    Organism-specific databases

    Human Protein Atlas

    More...    HPAi    		ENSG00000196236 Low tissue specificity

    <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

    <p>This subsection of the <a href="http://www.uniprot.org/help/interaction%5Fsection">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function%5Fsection">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

    Homodimer (PubMed:28476889).

    Isoform 1 interacts with TNFRSF1B/TNFR2 (activated) and TRAF2 (PubMed:25609706).

    2 Publications

    GO - Molecular functioni

    Complete GO annotation on QuickGO ...

    Protein-protein interaction databases

    The Biological General Repository for Interaction Datasets (BioGRID)

    More...    BioGRIDi    		121997, 32 interactors

    CORUM comprehensive resource of mammalian protein complexes

    More...    CORUMi    		Q9NQH7

    Protein interaction database and analysis system

    More...    IntActi    		Q9NQH7, 42 interactors

    Molecular INTeraction database

    More...    MINTi    		Q9NQH7

    STRING: functional protein association networks

    More...    STRINGi    		9606.ENSP00000349658

    Miscellaneous databases

    RNAct, Protein-RNA interaction predictions for model organisms.

    More...    RNActi    		Q9NQH7 protein

    <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

    Secondary structure

    1507
    Legend: HelixTurnBeta strandPDB Structure known for this area
    Show more details

    3D structure databases

    SWISS-MODEL Repository - a database of annotated 3D protein structure models

    More...    SMRi    		Q9NQH7

    Database of comparative protein structure models

    More...    ModBasei    		Search...

    Protein Data Bank in Europe - Knowledge Base

    More...    PDBe-KBi    		Search...

    <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

    Region

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the 'Family and Domains' section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni54 – 79Interaction with TNFRSF1B1 PublicationAdd BLAST26

    <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

    Belongs to the peptidase M24B family.Curated

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    evolutionary genealogy of genes: Non-supervised Orthologous Groups

    More...    eggNOGi    		KOG2414 Eukaryota
    COG0006 LUCA

    Ensembl GeneTree

    More...    GeneTreei    		ENSGT00940000153657

    The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

    More...    HOGENOMi    		CLU_017266_1_1_1

    InParanoid: Eukaryotic Ortholog Groups

    More...    InParanoidi    		Q9NQH7

    KEGG Orthology (KO)

    More...    KOi    		K01262

    Identification of Orthologs from Complete Genome Data

    More...    OMAi    		TGFNEPN

    Database of Orthologous Groups

    More...    OrthoDBi    		352329at2759

    Database for complete collections of gene phylogenies

    More...    PhylomeDBi    		Q9NQH7

    TreeFam database of animal gene trees

    More...    TreeFami    		TF314484

    Family and domain databases

    Gene3D Structural and Functional Annotation of Protein Families

    More...    Gene3Di    		3.40.350.10, 1 hit
    3.90.230.10, 1 hit

    Integrated resource of protein families, domains and functional sites

    More...    InterProi    		View protein in InterPro
    IPR007865 Aminopep_P_N
    IPR029149 Creatin/AminoP/Spt16_NTD
    IPR036005 Creatinase/aminopeptidase-like
    IPR000994 Pept_M24

    Pfam protein domain database

    More...    Pfami    		View protein in Pfam
    PF05195 AMP_N, 1 hit
    PF00557 Peptidase_M24, 1 hit

    Simple Modular Architecture Research Tool; a protein domain database

    More...    SMARTi    		View protein in SMART
    SM01011 AMP_N, 1 hit

    Superfamily database of structural and functional annotation

    More...    SUPFAMi    		SSF53092 SSF53092, 1 hit
    SSF55920 SSF55920, 1 hit

    <p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence%5Flength">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>. The information is filed in different subsections. The current subsections and their content are listed below:<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (5+)i

    <p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

    <p>This subsection of the <a href="http://www.uniprot.org/help/sequences%5Fsection">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

    This entry describes 5 <p>This subsection of the 'Sequence' section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform. This section is only present in reviewed entries, i.e. in UniProtKB/Swiss-Prot.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket

    This entry has 5 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All

    Isoform 1 (identifier: Q9NQH7-1) [UniParc]FASTAAdd to basket
    Also known as: m1 Publication

    This isoform has been chosen as the <div> <p><b>What is the canonical sequence?</b><p><a href='/help/canonical_and_isoforms' target='_top'>More...</a></p>canonicali sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

    « Hide
            10         20         30         40         50
    MPWLLSAPKL VPAVANVRGL SGCMLCSQRR YSLQPVPERR IPNRYLGQPS 
            60         70         80         90        100
    PFTHPHLLRP GEVTPGLSQV EYALRRHKLM SLIQKEAQGQ SGTDQTVVVL 
           110        120        130        140        150
    SNPTYYMSND IPYTFHQDNN FLYLCGFQEP DSILVLQSLP GKQLPSHKAI 
           160        170        180        190        200
    LFVPRRDPSR ELWDGPRSGT DGAIALTGVD EAYTLEEFQH LLPKMKAETN 
           210        220        230        240        250
    MVWYDWMRPS HAQLHSDYMQ PLTEAKAKSK NKVRGVQQLI QRLRLIKSPA 
           260        270        280        290        300
    EIERMQIAGK LTSQAFIETM FTSKAPVEEA FLYAKFEFEC RARGADILAY 
           310        320        330        340        350
    PPVVAGGNRS NTLHYVKNNQ LIKDGEMVLL DGGCESSCYV SDITRTWPVN 
           360        370        380        390        400
    GRFTAPQAEL YEAVLEIQRD CLALCFPGTS LENIYSMMLT LIGQKLKDLG 
           410        420        430        440        450
    IMKNIKENNA FKAARKYCPH HVGHYLGMDV HDTPDMPRSL PLQPGMVITI 
           460        470        480        490        500
    EPGIYIPEDD KDAPEKFRGL GVRIEDDVVV TQDSPLILSA DCPKEMNDIE 

    QICSQAS
    Length:507
    Mass (Da):57,034
    Last modified:October 1, 2000 - v1
    <p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:i82D886736ABD0B5B
    GO
    Isoform 2 (identifier: Q9NQH7-2) [UniParc]FASTAAdd to basket
    Also known as: c1 Publication

    The sequence of this isoform differs from the canonical sequence as follows:
         1-79: Missing.

    Show »
    Length:428
    Mass (Da):48,112
    Checksum:iE95AFE73A5D94D49
    GO
    Isoform 3 (identifier: Q9NQH7-3) [UniParc]FASTAAdd to basket

    The sequence of this isoform differs from the canonical sequence as follows:
         265-278: AFIETMFTSKAPVE → RQGFSVLSRLVSNS
         279-507: Missing.

    Show »
    Length:278
    Mass (Da):31,529
    Checksum:iB4AD5CEB13853823
    GO
    Isoform 4 (identifier: Q9NQH7-4) [UniParc]FASTAAdd to basket

    The sequence of this isoform differs from the canonical sequence as follows:
         1-23: Missing.

    Show »
    Length:484
    Mass (Da):54,672
    Checksum:i6320B2E5975918BB
    GO
    Isoform 5 (identifier: Q9NQH7-5) [UniParc]FASTAAdd to basket

    The sequence of this isoform differs from the canonical sequence as follows:
         265-288: AFIETMFTSKAPVEEAFLYAKFEF → KSVLLARHGGSRLYSHHFGRPRLS
         289-507: Missing.

    Show »
    Length:288
    Mass (Da):32,712
    Checksum:iE245C092422567ED
    GO

    <p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

    There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
    EntryEntry nameProtein names
    		Gene namesLengthAnnotation
    A0A087X0Z2A0A087X0Z2_HUMAN
    Xaa-Pro aminopeptidase 3
    XPNPEP3 LOC63929, hCG_41117
    		55Annotation score:

    Annotation score:1 out of 5

    <p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
    F2Z316F2Z316_HUMAN
    Xaa-Pro aminopeptidase 3
    XPNPEP3
    		69Annotation score:

    Annotation score:1 out of 5

    <p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the 'correct annotation' for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

    Experimental Info

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the 'Sequence' section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti340V → A in CAG33677 (Ref. 1) Curated1
    Sequence conflicti486L → F in AAH01681 (PubMed:15489334).Curated1

    Natural variant

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    Natural variantiVAR_051573450I → L. Corresponds to variant dbSNP:rs17002243Ensembl.1
    Natural variantiVAR_063820453G → C in NPHPL1. 1 PublicationCorresponds to variant dbSNP:rs267607179EnsemblClinVar.1

    Alternative sequence

    Feature keyPosition(s)DescriptionActionsGraphical viewLength
    <p>This subsection of the 'Sequence' section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_0212961 – 79Missing in isoform 2. 1 PublicationAdd BLAST79
    Alternative sequenceiVSP_0401421 – 23Missing in isoform 4. 1 PublicationAdd BLAST23
    Alternative sequenceiVSP_040143265 – 288AFIET…AKFEF → KSVLLARHGGSRLYSHHFGR PRLS in isoform 5. 1 PublicationAdd BLAST24
    Alternative sequenceiVSP_021297265 – 278AFIET…KAPVE → RQGFSVLSRLVSNS in isoform 3. 1 PublicationAdd BLAST14
    Alternative sequenceiVSP_021298279 – 507Missing in isoform 3. 1 PublicationAdd BLAST229
    Alternative sequenceiVSP_040144289 – 507Missing in isoform 5. 1 PublicationAdd BLAST219

    Sequence databases

    Select the link destinations:

    EMBL nucleotide sequence database

    More...    EMBLi

    GenBank nucleotide sequence database

    More...    GenBanki

    DNA Data Bank of Japan; a nucleotide sequence database

    More...    DDBJi
    Links Updated
    		CR457396 mRNA Translation: CAG33677.1
    AL365514 mRNA Translation: CAB97210.1
    CR456442 mRNA Translation: CAG30328.1
    AK301635 mRNA Translation: BAH13526.1
    AK301758 mRNA Translation: BAH13548.1
    AK313770 mRNA Translation: BAG36508.1
    AL834310 mRNA Translation: CAD38980.1
    AL035450 Genomic DNA No translation available.
    Z98048 Genomic DNA No translation available.
    CH471095 Genomic DNA Translation: EAW60399.1
    BC001208 mRNA Translation: AAH01208.1
    BC001681 mRNA Translation: AAH01681.1
    BC004989 mRNA Translation: AAH04989.1

    The Consensus CDS (CCDS) project

    More...    CCDSi    		CCDS14007.1 [Q9NQH7-1]

    NCBI Reference Sequences

    More...    RefSeqi    		NP_071381.1, NM_022098.3 [Q9NQH7-1]

    Genome annotation databases

    Ensembl eukaryotic genome annotation project

    More...    Ensembli    		ENST00000357137; ENSP00000349658; ENSG00000196236 [Q9NQH7-1]

    Database of genes from NCBI RefSeq genomes

    More...    GeneIDi    		63929

    KEGG: Kyoto Encyclopedia of Genes and Genomes

    More...    KEGGi    		hsa:63929

    UCSC genome browser

    More...    UCSCi    		uc003azh.4 human [Q9NQH7-1]

    Keywords - Coding sequence diversityi

    Alternative splicing, Polymorphism

    <p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

    <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

    Select the link destinations:
    EMBLi
    GenBanki
    DDBJi
    Links Updated
    		CR457396 mRNA Translation: CAG33677.1
    AL365514 mRNA Translation: CAB97210.1
    CR456442 mRNA Translation: CAG30328.1
    AK301635 mRNA Translation: BAH13526.1
    AK301758 mRNA Translation: BAH13548.1
    AK313770 mRNA Translation: BAG36508.1
    AL834310 mRNA Translation: CAD38980.1
    AL035450 Genomic DNA No translation available.
    Z98048 Genomic DNA No translation available.
    CH471095 Genomic DNA Translation: EAW60399.1
    BC001208 mRNA Translation: AAH01208.1
    BC001681 mRNA Translation: AAH01681.1
    BC004989 mRNA Translation: AAH04989.1
    CCDSiCCDS14007.1 [Q9NQH7-1]
    RefSeqiNP_071381.1, NM_022098.3 [Q9NQH7-1]

    3D structure databases

    Select the link destinations:

    Protein Data Bank Europe

    More...    PDBei

    Protein Data Bank RCSB

    More...    RCSB PDBi

    Protein Data Bank Japan

    More...    PDBji
    Links Updated
    		PDB entryMethodResolution (Å)ChainPositionsPDBsum
    5X49X-ray1.65A/B54-507[»]
    SMRiQ9NQH7
    ModBaseiSearch...
    PDBe-KBiSearch...

    Protein-protein interaction databases

    BioGRIDi121997, 32 interactors
    CORUMiQ9NQH7
    IntActiQ9NQH7, 42 interactors
    MINTiQ9NQH7
    STRINGi9606.ENSP00000349658

    Chemistry databases

    ChEMBLiCHEMBL3831223

    Protein family/group databases

    MEROPSiM24.026

    PTM databases

    iPTMnetiQ9NQH7
    PhosphoSitePlusiQ9NQH7

    Polymorphism and mutation databases

    BioMutaiXPNPEP3
    DMDMi74761652

    Proteomic databases

    EPDiQ9NQH7
    jPOSTiQ9NQH7
    MassIVEiQ9NQH7
    MaxQBiQ9NQH7
    PaxDbiQ9NQH7
    PeptideAtlasiQ9NQH7
    PRIDEiQ9NQH7
    ProteomicsDBi82154 [Q9NQH7-1]
    82155 [Q9NQH7-2]
    82156 [Q9NQH7-3]
    82157 [Q9NQH7-4]
    82158 [Q9NQH7-5]

    Protocols and materials databases

    Antibodypedia a portal for validated antibodies

    More...    Antibodypediai    		259 140 antibodies

    The DNASU plasmid repository

    More...    DNASUi    		63929

    Genome annotation databases

    EnsembliENST00000357137; ENSP00000349658; ENSG00000196236 [Q9NQH7-1]
    GeneIDi63929
    KEGGihsa:63929
    UCSCiuc003azh.4 human [Q9NQH7-1]

    Organism-specific databases

    Comparative Toxicogenomics Database

    More...    CTDi    		63929
    DisGeNETi63929
    EuPathDBiHostDB:ENSG00000196236.12

    GeneCards: human genes, protein and diseases

    More...    GeneCardsi    		XPNPEP3
    GeneReviewsiXPNPEP3
    HGNCiHGNC:28052 XPNPEP3
    HPAiENSG00000196236 Low tissue specificity
    MalaCardsiXPNPEP3
    MIMi613159 phenotype
    613553 gene
    neXtProtiNX_Q9NQH7
    OpenTargetsiENSG00000196236
    Orphaneti93589 Late-onset nephronophthisis
    PharmGKBiPA147357130

    GenAtlas: human gene database

    More...    GenAtlasi    		Search...

    Phylogenomic databases

    eggNOGiKOG2414 Eukaryota
    COG0006 LUCA
    GeneTreeiENSGT00940000153657
    HOGENOMiCLU_017266_1_1_1
    InParanoidiQ9NQH7
    KOiK01262
    OMAiTGFNEPN
    OrthoDBi352329at2759
    PhylomeDBiQ9NQH7
    TreeFamiTF314484

    Enzyme and pathway databases

    BRENDAi3.4.11.9 2681

    Miscellaneous databases

    BioGRID ORCS database of CRISPR phenotype screens

    More...    BioGRID-ORCSi    		63929 13 hits in 788 CRISPR screens

    ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

    More...    ChiTaRSi    		XPNPEP3 human

    The Gene Wiki collection of pages on human genes and proteins

    More...    GeneWikii    		XPNPEP3

    Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

    More...    GenomeRNAii    		63929
    PharosiQ9NQH7 Tbio

    Protein Ontology

    More...    PROi    		PR:Q9NQH7
    RNActiQ9NQH7 protein

    The Stanford Online Universal Resource for Clones and ESTs

    More...    SOURCEi    		Search...

    Gene expression databases

    BgeeiENSG00000196236 Expressed in testis and 201 other tissues
    ExpressionAtlasiQ9NQH7 baseline and differential
    GenevisibleiQ9NQH7 HS

    Family and domain databases

    Gene3Di3.40.350.10, 1 hit
    3.90.230.10, 1 hit
    InterProiView protein in InterPro
    IPR007865 Aminopep_P_N
    IPR029149 Creatin/AminoP/Spt16_NTD
    IPR036005 Creatinase/aminopeptidase-like
    IPR000994 Pept_M24
    PfamiView protein in Pfam
    PF05195 AMP_N, 1 hit
    PF00557 Peptidase_M24, 1 hit
    SMARTiView protein in SMART
    SM01011 AMP_N, 1 hit
    SUPFAMiSSF53092 SSF53092, 1 hit
    SSF55920 SSF55920, 1 hit

    ProtoNet; Automatic hierarchical classification of proteins

    More...    ProtoNeti    		Search...

    MobiDB: a database of protein disorder and mobility annotations

    More...    MobiDBi    		Search...

    <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 subsection of the 'Entry information' section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiXPP3_HUMAN
    <p>This subsection of the 'Entry information' section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called 'Primary (citable) accession number'.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: Q9NQH7
    Secondary accession number(s): B2R9G1
    , B7Z790, B7Z7B2, Q6I9V9, Q8NDA6, Q9BV27, Q9BVH0
    <p>This subsection of the 'Entry information' section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification ('Last modified'). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical%5Fand%5Fisoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: October 31, 2006
    Last sequence update: October 1, 2000
    Last modified: June 17, 2020
    This is version 170 of the entry and version 1 of the sequence. See complete history.
    <p>This subsection of the 'Entry information' section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program
    DisclaimerAny 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.

    <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

    3D-structure, Reference proteome

    Documents

    1. PDB cross-references
      Index of Protein Data Bank (PDB) cross-references
    2. SIMILARITY comments
      Index of protein domains and families
    3. Human entries with polymorphisms or disease mutations
      List of human entries with polymorphisms or disease mutations
    4. Human polymorphisms and disease mutations
      Index of human polymorphisms and disease mutations
    5. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    6. Human chromosome 22
      Human chromosome 22: entries, gene names and cross-references to MIM
    7. Peptidase families
      Classification of peptidase families and list of entries
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