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Entry version 184 (02 Jun 2021)
Sequence version 2 (18 Oct 2001)
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Protein

Endoplasmic reticulum transmembrane helix translocase

Gene

ATP13A1

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

Endoplasmic reticulum translocase required to remove mitochondrial transmembrane proteins mistargeted to the endoplasmic reticulum (PubMed:32973005).

Acts as a dislocase that mediates the ATP-dependent extraction of mislocalized mitochondrial transmembrane proteins from the endoplasmic reticulum membrane (PubMed:32973005).

Specifically binds mitochondrial tail-anchored transmembrane proteins: has an atypically large substrate-binding pocket that recognizes and binds moderately hydrophobic transmembranes with short hydrophilic lumenal domains (PubMed:32973005).

1 Publication

Caution

Was initially thought to mediate manganese transport (PubMed:24392018). However, it was later shown to specifically bind moderately hydrophobic transmembrane with short hydrophilic lumenal domains that misinsert into the endoplasmic reticulum (PubMed:32973005).2 Publications

<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

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section is used for enzymes and indicates the residues directly involved in catalysis.<p><a href='/help/act_site' target='_top'>More...</a></p>Active sitei5334-aspartylphosphate intermediate1 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 bindingi533MagnesiumBy similarity1
Metal bindingi535MagnesiumBy similarity1
<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 sitei626ATPBy similarity1
Binding sitei684ATPBy similarity1
Binding sitei749ATPBy similarity1
Metal bindingi864MagnesiumBy similarity1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/function%5Fsection">Function</a> section describes a region in the protein which binds nucleotide phosphates. It always involves more than one amino acid and includes all residues involved in nucleotide-binding.<p><a href='/help/np_bind' target='_top'>More...</a></p>Nucleotide bindingi533 – 535ATPBy similarity3
Nucleotide bindingi864 – 868ATPBy similarity5

<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

<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 functionTranslocase
Biological processProtein transport, Transport
LigandATP-binding, Magnesium, Metal-binding, Nucleotide-binding

Enzyme and pathway databases

Pathway Commons web resource for biological pathway data

More...
PathwayCommonsi
Q9HD20

Reactome - a knowledgebase of biological pathways and processes

More...
Reactomei
R-HSA-936837, Ion transport by P-type ATPases

SIGNOR Signaling Network Open Resource

More...
SIGNORi
Q9HD20

Protein family/group databases

Transport Classification Database

More...
TCDBi
3.A.3.10.19, the p-type atpase (p-atpase) superfamily

<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:
Endoplasmic reticulum transmembrane helix translocaseCurated (EC:7.4.2.-1 Publication)
Alternative name(s):
Endoplasmic reticulum P5A-ATPase1 Publication
<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:ATP13A11 PublicationImported
Synonyms:ATP13AImported, KIAA18251 Publication
ORF Names:CGI-1521 Publication
<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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<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 19

Organism-specific databases

Human Gene Nomenclature Database

More...
HGNCi
HGNC:24215, ATP13A1

Online Mendelian Inheritance in Man (OMIM)

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MIMi
619118, gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_Q9HD20

Eukaryotic Pathogen, Vector and Host Database Resources

More...
VEuPathDBi
HostDB:ENSG00000105726.16

<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

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/subcellular%5Flocation%5Fsection">'Subcellular location'</a> section describes the subcellular compartment where each non-membrane region of a membrane-spanning protein is found.<p><a href='/help/topo_dom' target='_top'>More...</a></p>Topological domaini2 – 66CytoplasmicSequence analysisAdd BLAST65
<p>This subsection of the <a href="http://www.uniprot.org/help/subcellular%5Flocation%5Fsection">'Subcellular location'</a> section describes the extent of a membrane-spanning region of the protein. It denotes the presence of both alpha-helical transmembrane regions and the membrane spanning regions of beta-barrel transmembrane proteins.<p><a href='/help/transmem' target='_top'>More...</a></p>Transmembranei67 – 87HelicalSequence analysisAdd BLAST21
Topological domaini88 – 95ExtracellularSequence analysis8
Transmembranei96 – 116HelicalSequence analysisAdd BLAST21
Topological domaini117 – 243CytoplasmicSequence analysisAdd BLAST127
Transmembranei244 – 264HelicalSequence analysisAdd BLAST21
Topological domaini265 – 443ExtracellularSequence analysisAdd BLAST179
Transmembranei444 – 464HelicalSequence analysisAdd BLAST21
Topological domaini465 – 989CytoplasmicSequence analysisAdd BLAST525
Transmembranei990 – 1010HelicalSequence analysisAdd BLAST21
Topological domaini1011ExtracellularSequence analysis1
Transmembranei1012 – 1032HelicalSequence analysisAdd BLAST21
Topological domaini1033 – 1051CytoplasmicSequence analysisAdd BLAST19
Transmembranei1052 – 1072HelicalSequence analysisAdd BLAST21
Topological domaini1073 – 1096ExtracellularSequence analysisAdd BLAST24
Transmembranei1097 – 1117HelicalSequence analysisAdd BLAST21
Topological domaini1118 – 1132CytoplasmicSequence analysisAdd BLAST15
Transmembranei1133 – 1153HelicalSequence analysisAdd BLAST21
Topological domaini1154 – 1166ExtracellularSequence analysisAdd BLAST13
Transmembranei1167 – 1187HelicalSequence analysisAdd BLAST21
Topological domaini1188 – 1204CytoplasmicSequence analysisAdd BLAST17

Keywords - Cellular componenti

Endoplasmic reticulum, Membrane

<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

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>Mutagenesisi533D → A: Loss of ATPase activity. 1 Publication1

Organism-specific databases

DisGeNET

More...
DisGeNETi
57130

Open Targets

More...
OpenTargetsi
ENSG00000105726

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...
PharmGKBi
PA134988892

Miscellaneous databases

Pharos NIH Druggable Genome Knowledgebase

More...
Pharosi
Q9HD20, Tdark

Genetic variation databases

BioMuta curated single-nucleotide variation and disease association database

More...
BioMutai
ATP13A1

Domain mapping of disease mutations (DMDM)

More...
DMDMi
18202961

<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 <a href="http://www.uniprot.org/help/ptm%5Fprocessing%5Fsection">PTM / Processing</a> section indicates that the initiator methionine is cleaved from the mature protein.<p><a href='/help/init_met' target='_top'>More...</a></p>Initiator methionineiRemovedCombined sources
<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_00000464212 – 1204Endoplasmic reticulum transmembrane helix translocaseAdd BLAST1203

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'PTM / Processing' section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei2N-acetylalanineCombined sources1
<p>This subsection of the <a href="http://www.uniprot.org/help/ptm%5Fprocessing%5Fsection">PTM / Processing</a> section specifies the position and type of each covalently attached glycan group (mono-, di-, or polysaccharide).<p><a href='/help/carbohyd' target='_top'>More...</a></p>Glycosylationi287N-linked (GlcNAc...) asparagineSequence analysis1
Glycosylationi420N-linked (GlcNAc...) asparagine1 Publication1
Modified residuei899PhosphoserineCombined sources1
Modified residuei905PhosphoserineCombined sources1

Keywords - PTMi

Acetylation, Glycoprotein, Phosphoprotein

Proteomic databases

Encyclopedia of Proteome Dynamics

More...
EPDi
Q9HD20

jPOST - Japan Proteome Standard Repository/Database

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jPOSTi
Q9HD20

MassIVE - Mass Spectrometry Interactive Virtual Environment

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MassIVEi
Q9HD20

MaxQB - The MaxQuant DataBase

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MaxQBi
Q9HD20

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

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PaxDbi
Q9HD20

PeptideAtlas

More...
PeptideAtlasi
Q9HD20

PRoteomics IDEntifications database

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PRIDEi
Q9HD20

ProteomicsDB: a multi-organism proteome resource

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ProteomicsDBi
81811 [Q9HD20-1]
81812 [Q9HD20-2]
81813 [Q9HD20-3]

PTM databases

CarbonylDB database of protein carbonylation sites

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CarbonylDBi
Q9HD20

GlyGen: Computational and Informatics Resources for Glycoscience

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GlyGeni
Q9HD20, 2 sites

iPTMnet integrated resource for PTMs in systems biology context

More...
iPTMneti
Q9HD20

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

More...
PhosphoSitePlusi
Q9HD20

SwissPalm database of S-palmitoylation events

More...
SwissPalmi
Q9HD20

<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

Bgee dataBase for Gene Expression Evolution

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Bgeei
ENSG00000105726, Expressed in small intestine Peyer's patch and 221 other tissues

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
Q9HD20, baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

More...
Genevisiblei
Q9HD20, HS

Organism-specific databases

Human Protein Atlas

More...
HPAi
ENSG00000105726, 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 binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="https://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated at every <a href="http://www.uniprot.org/help/synchronization">UniProt release</a>.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

Hide details

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGRID)

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BioGRIDi
121393, 84 interactors

Protein interaction database and analysis system

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IntActi
Q9HD20, 48 interactors

Molecular INTeraction database

More...
MINTi
Q9HD20

STRING: functional protein association networks

More...
STRINGi
9606.ENSP00000349877

Miscellaneous databases

RNAct, Protein-RNA interaction predictions for model organisms.

More...
RNActi
Q9HD20, 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

3D structure databases

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

More...
SMRi
Q9HD20

Database of comparative protein structure models

More...
ModBasei
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>Regioni199 – 230A-domain; part 1By similarityAdd BLAST32
Regioni295 – 435A-domain; part 2By similarityAdd BLAST141
Regioni512 – 541P-domain; part 1By similarityAdd BLAST30
Regioni543 – 724N-domainBy similarityAdd BLAST182
Regioni727 – 885P-domain; part 2By similarityAdd BLAST159
Regioni886 – 949Arm-likeBy similarityAdd BLAST64
Regioni889 – 933DisorderedSequence analysisAdd BLAST45
Regioni950 – 965P-domain; part 3By similarityAdd BLAST16

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the 'Family and Domains' section describes the position of regions of compositional bias within the protein and the particular type of amino acids that are over-represented within those regions.<p><a href='/help/compbias' target='_top'>More...</a></p>Compositional biasi900 – 933Polar residuesSequence analysisAdd BLAST34

<p>This subsection of the 'Family and domains' section provides general information on the biological role of a domain. The term 'domain' is intended here in its wide acceptation, it may be a structural domain, a transmembrane region or a functional domain. Several domains are described in this subsection.<p><a href='/help/domain_cc' target='_top'>More...</a></p>Domaini

Contains a large substrate-binding pocket that recognizes alpha-helical transmembranes, which alternately faces the endoplasmic reticulum lumen and cytosol, while remaining accessible to the lipid bilayer through a lateral opening. The translocase alternates between two conformations: inward-open (E1) and outward-open (E2) states. Undergoes a series of conformational changes with ATP-binding, phosphorylation of the Asp active site and subsequent dephosphorylation in a Post-Albers cycle (i.e., E1 -> E1-ATP -> E1P-ADP -> E1P -> E2P -> E2-Pi -> E1). A substrate transmembrane helix with a short, preferentially positively charged lumenal segment binds to the outward-open pocket and the E2P-to-E1 transition flips the transmembrane by a switch from the outward-open to inward-open conformation.By similarity

<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

Keywords - Domaini

Transmembrane, Transmembrane helix

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...
eggNOGi
KOG0209, Eukaryota

Ensembl GeneTree

More...
GeneTreei
ENSGT00550000075064

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

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HOGENOMi
CLU_001828_4_1_1

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
Q9HD20

Identification of Orthologs from Complete Genome Data

More...
OMAi
RFAPKQK

Database for complete collections of gene phylogenies

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PhylomeDBi
Q9HD20

TreeFam database of animal gene trees

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TreeFami
TF300725

Family and domain databases

Gene3D Structural and Functional Annotation of Protein Families

More...
Gene3Di
3.40.1110.10, 1 hit
3.40.50.1000, 1 hit

Integrated resource of protein families, domains and functional sites

More...
InterProi
View protein in InterPro
IPR023299, ATPase_P-typ_cyto_dom_N
IPR018303, ATPase_P-typ_P_site
IPR023298, ATPase_P-typ_TM_dom_sf
IPR008250, ATPase_P-typ_transduc_dom_A_sf
IPR036412, HAD-like_sf
IPR023214, HAD_sf
IPR006544, P-type_TPase_V
IPR001757, P_typ_ATPase
IPR044492, P_typ_ATPase_HD_dom

Structure-Function Linkage Database

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SFLDi
SFLDF00027, p-type_atpase, 1 hit

Superfamily database of structural and functional annotation

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SUPFAMi
SSF56784, SSF56784, 1 hit
SSF81653, SSF81653, 1 hit
SSF81660, SSF81660, 1 hit
SSF81665, SSF81665, 1 hit

TIGRFAMs; a protein family database

More...
TIGRFAMsi
TIGR01494, ATPase_P-type, 2 hits
TIGR01657, P-ATPase-V, 1 hit

PROSITE; a protein domain and family database

More...
PROSITEi
View protein in PROSITE
PS00154, ATPASE_E1_E2, 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 (3+)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 3 <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.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket
Note: Experimental confirmation may be lacking for some isoforms.

This entry has 3 described isoforms and 1 potential isoform that is computationally mapped.Show allAlign All

Isoform A (identifier: Q9HD20-1) [UniParc]FASTAAdd to basket

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
MAAAAAVGNA VPCGARPCGV RPDGQPKPGP QPRALLAAGP ALIANGDELV
60 70 80 90 100
AAVWPYRRLA LLRRLTVLPF AGLLYPAWLG AAAAGCWGWG SSWVQIPEAA
110 120 130 140 150
LLVLATICLA HALTVLSGHW SVHAHCALTC TPEYDPSKAT FVKVVPTPNN
160 170 180 190 200
GSTELVALHR NEGEDGLEVL SFEFQKIKYS YDALEKKQFL PVAFPVGNAF
210 220 230 240 250
SYYQSNRGFQ EDSEIRAAEK KFGSNKAEMV VPDFSELFKE RATAPFFVFQ
260 270 280 290 300
VFCVGLWCLD EYWYYSVFTL SMLVAFEASL VQQQMRNMSE IRKMGNKPHM
310 320 330 340 350
IQVYRSRKWR PIASDEIVPG DIVSIGRSPQ ENLVPCDVLL LRGRCIVDEA
360 370 380 390 400
MLTGESVPQM KEPIEDLSPD RVLDLQADSR LHVIFGGTKV VQHIPPQKAT
410 420 430 440 450
TGLKPVDSGC VAYVLRTGFN TSQGKLLRTI LFGVKRVTAN NLETFIFILF
460 470 480 490 500
LLVFAIAAAA YVWIEGTKDP SRNRYKLFLE CTLILTSVVP PELPIELSLA
510 520 530 540 550
VNTSLIALAK LYMYCTEPFR IPFAGKVEVC CFDKTGTLTS DSLVVRGVAG
560 570 580 590 600
LRDGKEVTPV SSIPVETHRA LASCHSLMQL DDGTLVGDPL EKAMLTAVDW
610 620 630 640 650
TLTKDEKVFP RSIKTQGLKI HQRFHFASAL KRMSVLASYE KLGSTDLCYI
660 670 680 690 700
AAVKGAPETL HSMFSQCPPD YHHIHTEISR EGARVLALGY KELGHLTHQQ
710 720 730 740 750
AREVKREALE CSLKFVGFIV VSCPLKADSK AVIREIQNAS HRVVMITGDN
760 770 780 790 800
PLTACHVAQE LHFIEKAHTL ILQPPSEKGR QCEWRSIDGS IVLPLARGSP
810 820 830 840 850
KALALEYALC LTGDGLAHLQ ATDPQQLLRL IPHVQVFARV APKQKEFVIT
860 870 880 890 900
SLKELGYVTL MCGDGTNDVG ALKHADVGVA LLANAPERVV ERRRRPRDSP
910 920 930 940 950
TLSNSGIRAT SRTAKQRSGL PPSEEQPTSQ RDRLSQVLRD LEDESTPIVK
960 970 980 990 1000
LGDASIAAPF TSKLSSIQCI CHVIKQGRCT LVTTLQMFKI LALNALILAY
1010 1020 1030 1040 1050
SQSVLYLEGV KFSDFQATLQ GLLLAGCFLF ISRSKPLKTL SRERPLPNIF
1060 1070 1080 1090 1100
NLYTILTVML QFFVHFLSLV YLYREAQARS PEKQEQFVDL YKEFEPSLVN
1110 1120 1130 1140 1150
STVYIMAMAM QMATFAINYK GPPFMESLPE NKPLVWSLAV SLLAIIGLLL
1160 1170 1180 1190 1200
GSSPDFNSQF GLVDIPVEFK LVIAQVLLLD FCLALLADRV LQFFLGTPKL

KVPS
Length:1,204
Mass (Da):132,955
Last modified:October 18, 2001 - v2
<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:i9DE335C025FBBA89
GO
Isoform B (identifier: Q9HD20-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-118: Missing.
     119-132: HWSVHAHCALTCTP → MEKWEELNSHQPGE

Show »
Length:1,086
Mass (Da):121,110
Checksum:i0D0D62BF402E68D0
GO
Isoform C (identifier: Q9HD20-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-860: Missing.

Show »
Length:344
Mass (Da):38,207
Checksum:iE988B4A90EC2DE3D
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 is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
H7C3H2H7C3H2_HUMAN
Endoplasmic reticulum transmembrane...
ATP13A1
185Annotation 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>

<p>This subsection of the 'Sequence' section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>Sequence cautioni

The sequence AAH69211 differs from that shown. Reason: Frameshift.Curated

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 conflicti364I → T in BAD18759 (PubMed:14702039).Curated1
Sequence conflicti501V → D in BAD18759 (PubMed:14702039).Curated1
Sequence conflicti1150L → P in BAD18759 (PubMed:14702039).Curated1

Natural variant

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_084651349E → K Found in a patient with intellectual disability; unknown pathological significance. 1 Publication1

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_0004331 – 860Missing in isoform C. 1 PublicationAdd BLAST860
Alternative sequenceiVSP_0004341 – 118Missing in isoform B. 1 PublicationAdd BLAST118
Alternative sequenceiVSP_000435119 – 132HWSVH…LTCTP → MEKWEELNSHQPGE in isoform B. 1 PublicationAdd BLAST14

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
AF288687 mRNA Translation: AAG01173.1
AK026044 mRNA Translation: BAB15334.1
AK056420 mRNA Translation: BAG51704.1
AK095287 mRNA Translation: BAG53019.1
AK172778 mRNA Translation: BAD18759.1
AB058728 mRNA Translation: BAB47454.1
CH471106 Genomic DNA Translation: EAW84849.1
CH471106 Genomic DNA Translation: EAW84850.1
BC009302 mRNA Translation: AAH09302.2
BC069211 mRNA Translation: AAH69211.1 Frameshift.

The Consensus CDS (CCDS) project

More...
CCDSi
CCDS32970.2 [Q9HD20-1]

NCBI Reference Sequences

More...
RefSeqi
NP_065143.2, NM_020410.2 [Q9HD20-1]

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...
Ensembli
ENST00000291503; ENSP00000291503; ENSG00000105726 [Q9HD20-2]
ENST00000357324; ENSP00000349877; ENSG00000105726 [Q9HD20-1]

Database of genes from NCBI RefSeq genomes

More...
GeneIDi
57130

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...
KEGGi
hsa:57130

UCSC genome browser

More...
UCSCi
uc002nng.4, human [Q9HD20-1]

Keywords - Coding sequence diversityi

Alternative splicing

<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
AF288687 mRNA Translation: AAG01173.1
AK026044 mRNA Translation: BAB15334.1
AK056420 mRNA Translation: BAG51704.1
AK095287 mRNA Translation: BAG53019.1
AK172778 mRNA Translation: BAD18759.1
AB058728 mRNA Translation: BAB47454.1
CH471106 Genomic DNA Translation: EAW84849.1
CH471106 Genomic DNA Translation: EAW84850.1
BC009302 mRNA Translation: AAH09302.2
BC069211 mRNA Translation: AAH69211.1 Frameshift.
CCDSiCCDS32970.2 [Q9HD20-1]
RefSeqiNP_065143.2, NM_020410.2 [Q9HD20-1]

3D structure databases

SMRiQ9HD20
ModBaseiSearch...

Protein-protein interaction databases

BioGRIDi121393, 84 interactors
IntActiQ9HD20, 48 interactors
MINTiQ9HD20
STRINGi9606.ENSP00000349877

Protein family/group databases

TCDBi3.A.3.10.19, the p-type atpase (p-atpase) superfamily

PTM databases

CarbonylDBiQ9HD20
GlyGeniQ9HD20, 2 sites
iPTMnetiQ9HD20
PhosphoSitePlusiQ9HD20
SwissPalmiQ9HD20

Genetic variation databases

BioMutaiATP13A1
DMDMi18202961

Proteomic databases

EPDiQ9HD20
jPOSTiQ9HD20
MassIVEiQ9HD20
MaxQBiQ9HD20
PaxDbiQ9HD20
PeptideAtlasiQ9HD20
PRIDEiQ9HD20
ProteomicsDBi81811 [Q9HD20-1]
81812 [Q9HD20-2]
81813 [Q9HD20-3]

Protocols and materials databases

Antibodypedia a portal for validated antibodies

More...
Antibodypediai
28529, 81 antibodies

The DNASU plasmid repository

More...
DNASUi
57130

Genome annotation databases

EnsembliENST00000291503; ENSP00000291503; ENSG00000105726 [Q9HD20-2]
ENST00000357324; ENSP00000349877; ENSG00000105726 [Q9HD20-1]
GeneIDi57130
KEGGihsa:57130
UCSCiuc002nng.4, human [Q9HD20-1]

Organism-specific databases

Comparative Toxicogenomics Database

More...
CTDi
57130
DisGeNETi57130

GeneCards: human genes, protein and diseases

More...
GeneCardsi
ATP13A1
HGNCiHGNC:24215, ATP13A1
HPAiENSG00000105726, Low tissue specificity
MIMi619118, gene
neXtProtiNX_Q9HD20
OpenTargetsiENSG00000105726
PharmGKBiPA134988892
VEuPathDBiHostDB:ENSG00000105726.16

Human Unidentified Gene-Encoded large proteins database

More...
HUGEi
Search...

GenAtlas: human gene database

More...
GenAtlasi
Search...

Phylogenomic databases

eggNOGiKOG0209, Eukaryota
GeneTreeiENSGT00550000075064
HOGENOMiCLU_001828_4_1_1
InParanoidiQ9HD20
OMAiRFAPKQK
PhylomeDBiQ9HD20
TreeFamiTF300725

Enzyme and pathway databases

PathwayCommonsiQ9HD20
ReactomeiR-HSA-936837, Ion transport by P-type ATPases
SIGNORiQ9HD20

Miscellaneous databases

BioGRID ORCS database of CRISPR phenotype screens

More...
BioGRID-ORCSi
57130, 100 hits in 994 CRISPR screens

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

More...
ChiTaRSi
ATP13A1, human

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

More...
GenomeRNAii
57130
PharosiQ9HD20, Tdark

Protein Ontology

More...
PROi
PR:Q9HD20
RNActiQ9HD20, protein

The Stanford Online Universal Resource for Clones and ESTs

More...
SOURCEi
Search...

Gene expression databases

BgeeiENSG00000105726, Expressed in small intestine Peyer's patch and 221 other tissues
ExpressionAtlasiQ9HD20, baseline and differential
GenevisibleiQ9HD20, HS

Family and domain databases

Gene3Di3.40.1110.10, 1 hit
3.40.50.1000, 1 hit
InterProiView protein in InterPro
IPR023299, ATPase_P-typ_cyto_dom_N
IPR018303, ATPase_P-typ_P_site
IPR023298, ATPase_P-typ_TM_dom_sf
IPR008250, ATPase_P-typ_transduc_dom_A_sf
IPR036412, HAD-like_sf
IPR023214, HAD_sf
IPR006544, P-type_TPase_V
IPR001757, P_typ_ATPase
IPR044492, P_typ_ATPase_HD_dom
SFLDiSFLDF00027, p-type_atpase, 1 hit
SUPFAMiSSF56784, SSF56784, 1 hit
SSF81653, SSF81653, 1 hit
SSF81660, SSF81660, 1 hit
SSF81665, SSF81665, 1 hit
TIGRFAMsiTIGR01494, ATPase_P-type, 2 hits
TIGR01657, P-ATPase-V, 1 hit
PROSITEiView protein in PROSITE
PS00154, ATPASE_E1_E2, 1 hit

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 nameiAT131_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: Q9HD20
Secondary accession number(s): B3KPJ2
, B3KTA7, Q6NT90, Q6ZMG7, Q9H6C6
<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 18, 2001
Last sequence update: October 18, 2001
Last modified: June 2, 2021
This is version 184 of the entry and version 2 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

Reference proteome

Documents

  1. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
  2. Human entries with genetic variants
    List of human entries with genetic variants
  3. Human variants curated from literature reports
    Index of human variants curated from literature reports
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families
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