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Entry version 221 (16 Oct 2019)
Sequence version 3 (25 Oct 2002)
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Protein

Ubiquitin-like modifier-activating enzyme 1

Gene

UBA1

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 first step in ubiquitin conjugation to mark cellular proteins for degradation through the ubiquitin-proteasome system (PubMed:1606621, PubMed:1447181). Activates ubiquitin by first adenylating its C-terminal glycine residue with ATP, and thereafter linking this residue to the side chain of a cysteine residue in E1, yielding a ubiquitin-E1 thioester and free AMP (PubMed:1447181). Essential for the formation of radiation-induced foci, timely DNA repair and for response to replication stress. Promotes the recruitment of TP53BP1 and BRCA1 at DNA damage sites (PubMed:22456334).3 Publications

Miscellaneous

There are two active sites within the E1 molecule, allowing it to accommodate two ubiquitin moieties at a time, with a new ubiquitin forming an adenylate intermediate as the previous one is transferred to the thiol site.By similarity

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">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

  • ATP + ubiquitin + [E1 ubiquitin-activating enzyme]-L-cysteine = AMP + diphosphate + S-ubiquitinyl-[E1 ubiquitin-activating enzyme]-L-cysteine.1 Publication EC:6.2.1.45

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section describes the metabolic pathway(s) associated with a protein.<p><a href='/help/pathway' target='_top'>More...</a></p>Pathwayi: protein ubiquitination

This protein is involved in the pathway protein ubiquitination, which is part of Protein modification.2 Publications
View all proteins of this organism that are known to be involved in the pathway protein ubiquitination and in Protein modification.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">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 sitei478ATP; via amide nitrogenBy similarity1
Binding sitei504ATPBy similarity1
Binding sitei515ATPBy similarity1
Binding sitei528ATPBy similarity1
<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">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 sitei632Glycyl thioester intermediatePROSITE-ProRule annotation1

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">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 bindingi576 – 577ATPBy similarity2

<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 functionLigase
Biological processUbl conjugation pathway
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

BioCyc Collection of Pathway/Genome Databases

More...
BioCyci
MetaCyc:HS05465-MONOMER

BRENDA Comprehensive Enzyme Information System

More...
BRENDAi
2.3.2.B5 2681
6.2.1.B9 2681

Reactome - a knowledgebase of biological pathways and processes

More...
Reactomei
R-HSA-8866652 Synthesis of active ubiquitin: roles of E1 and E2 enzymes
R-HSA-983168 Antigen processing: Ubiquitination & Proteasome degradation

SIGNOR Signaling Network Open Resource

More...
SIGNORi
P22314

UniPathway: a resource for the exploration and annotation of metabolic pathways

More...
UniPathwayi
UPA00143

<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_and_taxonomy_section">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:
Ubiquitin-like modifier-activating enzyme 1 (EC:6.2.1.451 Publication)
Alternative name(s):
Protein A1S9
Ubiquitin-activating enzyme E1
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">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:UBA1
Synonyms:A1S9T, UBE1
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">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_and_taxonomy_section">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_and_taxonomy_section">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_and_taxonomy_section">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_manual">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 X

Organism-specific databases

Human Gene Nomenclature Database

More...
HGNCi
HGNC:12469 UBA1

Online Mendelian Inheritance in Man (OMIM)

More...
MIMi
314370 gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_P22314

<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, Nucleus

<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

Spinal muscular atrophy X-linked 2 (SMAX2)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA lethal infantile form of spinal muscular atrophy, a neuromuscular disorder characterized by degeneration of the anterior horn cells of the spinal cord, leading to symmetrical muscle weakness and atrophy. Clinical features include hypotonia, areflexia, and multiple congenital contractures.
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_043501539M → I in SMAX2. 1 PublicationCorresponds to variant dbSNP:rs80356545EnsemblClinVar.1
Natural variantiVAR_043502547S → G in SMAX2. 1 PublicationCorresponds to variant dbSNP:rs80356546EnsemblClinVar.1
Natural variantiVAR_071121557E → V in SMAX2. 1 Publication1

Mutagenesis

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the <a href="http://www.uniprot.org/manual/pathology_and_biotech_section">'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>Mutagenesisi4S → A: Reduces phosphorylation. 1 Publication1
Mutagenesisi8 – 11KKRR → AAAA: Loss of nuclear localization and a 90-95% decrease in the phosphorylation. 1 Publication4

Keywords - Diseasei

Disease mutation, Neurodegeneration

Organism-specific databases

DisGeNET

More...
DisGeNETi
7317

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

More...
GeneReviewsi
UBA1

MalaCards human disease database

More...
MalaCardsi
UBA1
MIMi301830 phenotype

Open Targets

More...
OpenTargetsi
ENSG00000130985

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

More...
Orphaneti
1145 Infantile-onset X-linked spinal muscular atrophy

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...
PharmGKBi
PA37119

Miscellaneous databases

Pharos NIH Druggable Genome Knowledgebase

More...
Pharosi
P22314

Chemistry databases

ChEMBL database of bioactive drug-like small molecules

More...
ChEMBLi
CHEMBL5924

Drug and drug target database

More...
DrugBanki
DB04119 Hexatantalum Dodecabromide
DB04216 Quercetin

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

More...
BioMutai
UBA1

Domain mapping of disease mutations (DMDM)

More...
DMDMi
24418865

<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_processing_section">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.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_00001949342 – 1058Ubiquitin-like modifier-activating enzyme 1Add BLAST1057
Isoform 2 (identifier: P22314-2)
Initiator methionineiRemovedCombined sources

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-acetylserineCombined sources1
Modified residuei4Phosphoserine1 Publication1
Modified residuei13PhosphoserineCombined sources1
Modified residuei21PhosphoserineBy similarity1
Modified residuei24PhosphoserineBy similarity1
Modified residuei46PhosphoserineCombined sources1
Modified residuei55PhosphotyrosineBy similarity1
Modified residuei528N6-succinyllysineBy similarity1
Modified residuei671N6-acetyllysineCombined sources1
Modified residuei800PhosphothreonineCombined sources1
Modified residuei810PhosphoserineCombined sources1
Modified residuei816PhosphoserineBy similarity1
Modified residuei820PhosphoserineCombined sources1
Modified residuei835PhosphoserineCombined sources1
Modified residuei980N6-acetyllysineCombined sources1
Isoform 2 (identifier: P22314-2)
Modified residuei2N-acetylalanineCombined sources1

<p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">PTM/processing</a> section describes post-translational modifications (PTMs). This subsection <strong>complements</strong> the information provided at the sequence level or describes modifications for which <strong>position-specific data is not yet available</strong>.<p><a href='/help/post-translational_modification' target='_top'>More...</a></p>Post-translational modificationi

ISGylated.1 Publication

Keywords - PTMi

Acetylation, Phosphoprotein, Ubl conjugation

Proteomic databases

The CPTAC Assay portal

More...
CPTACi
CPTAC-136
CPTAC-137

Encyclopedia of Proteome Dynamics

More...
EPDi
P22314

jPOST - Japan Proteome Standard Repository/Database

More...
jPOSTi
P22314

MassIVE - Mass Spectrometry Interactive Virtual Environment

More...
MassIVEi
P22314

MaxQB - The MaxQuant DataBase

More...
MaxQBi
P22314

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

More...
PaxDbi
P22314

PeptideAtlas

More...
PeptideAtlasi
P22314

PRoteomics IDEntifications database

More...
PRIDEi
P22314

ProteomicsDB: a multi-organism proteome resource

More...
ProteomicsDBi
53983 [P22314-1]

2D gel databases

REPRODUCTION-2DPAGE

More...
REPRODUCTION-2DPAGEi
IPI00645078

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...
iPTMneti
P22314

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

More...
PhosphoSitePlusi
P22314

SwissPalm database of S-palmitoylation events

More...
SwissPalmi
P22314

<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

Detected in erythrocytes (at protein level). Ubiquitous.1 Publication

Gene expression databases

Bgee dataBase for Gene Expression Evolution

More...
Bgeei
ENSG00000130985 Expressed in 243 organ(s), highest expression level in pituitary gland

ExpressionAtlas, Differential and Baseline Expression

More...
ExpressionAtlasi
P22314 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

More...
Genevisiblei
P22314 HS

Organism-specific databases

Human Protein Atlas

More...
HPAi
CAB019435
CAB073410
HPA000289
HPA001506

<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_section">'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_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Monomer (By similarity).

Interacts with GAN (via BTB domain).

By similarity1 Publication

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction_section%27">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="http://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated on a monthly basis. Each binary interaction is displayed on a separate line.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

More...
BioGridi
113165, 170 interactors

Database of interacting proteins

More...
DIPi
DIP-33686N

Protein interaction database and analysis system

More...
IntActi
P22314, 141 interactors

Molecular INTeraction database

More...
MINTi
P22314

STRING: functional protein association networks

More...
STRINGi
9606.ENSP00000338413

Chemistry databases

BindingDB database of measured binding affinities

More...
BindingDBi
P22314

<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

11058
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

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SMRi
P22314

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

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section indicates the positions and types of repeated sequence motifs or repeated domains within the protein.<p><a href='/help/repeat' target='_top'>More...</a></p>Repeati63 – 1991-1Add BLAST137
Repeati459 – 6111-2Add BLAST153

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>Regioni63 – 6112 approximate repeatsAdd BLAST549

Motif

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section describes a short (usually not more than 20 amino acids) conserved sequence motif of biological significance.<p><a href='/help/motif' target='_top'>More...</a></p>Motifi5 – 11Nuclear localization signal7

<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

The first 11 amino acids are essential for phosphorylation and exclusive nuclear localization.

<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 ubiquitin-activating E1 family.Curated

Keywords - Domaini

Repeat

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...
eggNOGi
KOG2012 Eukaryota
COG0476 LUCA

Ensembl GeneTree

More...
GeneTreei
ENSGT00940000158975

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

More...
HOGENOMi
HOG000167329

InParanoid: Eukaryotic Ortholog Groups

More...
InParanoidi
P22314

KEGG Orthology (KO)

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KOi
K03178

Identification of Orthologs from Complete Genome Data

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OMAi
CIVWARL

Database of Orthologous Groups

More...
OrthoDBi
588360at2759

Database for complete collections of gene phylogenies

More...
PhylomeDBi
P22314

TreeFam database of animal gene trees

More...
TreeFami
TF300586

Family and domain databases

Gene3D Structural and Functional Annotation of Protein Families

More...
Gene3Di
1.10.10.2660, 1 hit
2.40.30.180, 1 hit
3.10.290.60, 1 hit
3.50.50.80, 1 hit

Integrated resource of protein families, domains and functional sites

More...
InterProi
View protein in InterPro
IPR032420 E1_4HB
IPR032418 E1_FCCH
IPR042302 E1_FCCH_sf
IPR000594 ThiF_NAD_FAD-bd
IPR018965 Ub-activating_enz_E1_C
IPR042449 Ub-E1_IAD_1
IPR038252 UBA_E1_C_sf
IPR019572 UBA_E1_Cys
IPR042063 Ubi_acti_E1_SCCH
IPR035985 Ubiquitin-activating_enz
IPR018075 UBQ-activ_enz_E1
IPR018074 UBQ-activ_enz_E1_CS
IPR033127 UBQ-activ_enz_E1_Cys_AS
IPR000011 UBQ/SUMO-activ_enz_E1-like

Pfam protein domain database

More...
Pfami
View protein in Pfam
PF16191 E1_4HB, 1 hit
PF16190 E1_FCCH, 1 hit
PF09358 E1_UFD, 1 hit
PF00899 ThiF, 2 hits
PF10585 UBA_e1_thiolCys, 1 hit

Protein Motif fingerprint database; a protein domain database

More...
PRINTSi
PR01849 UBIQUITINACT

Simple Modular Architecture Research Tool; a protein domain database

More...
SMARTi
View protein in SMART
SM00985 UBA_e1_C, 1 hit

Superfamily database of structural and functional annotation

More...
SUPFAMi
SSF69572 SSF69572, 2 hits

TIGRFAMs; a protein family database

More...
TIGRFAMsi
TIGR01408 Ube1, 1 hit

PROSITE; a protein domain and family database

More...
PROSITEi
View protein in PROSITE
PS00536 UBIQUITIN_ACTIVAT_1, 1 hit
PS00865 UBIQUITIN_ACTIVAT_2, 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_length">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 (2+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">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_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">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 2 <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 initiation. AlignAdd to basket

This entry has 2 described isoforms and 6 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: P22314-1) [UniParc]FASTAAdd to basket
Also known as: E1a

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
MSSSPLSKKR RVSGPDPKPG SNCSPAQSVL SEVPSVPTNG MAKNGSEADI
60 70 80 90 100
DEGLYSRQLY VLGHEAMKRL QTSSVLVSGL RGLGVEIAKN IILGGVKAVT
110 120 130 140 150
LHDQGTAQWA DLSSQFYLRE EDIGKNRAEV SQPRLAELNS YVPVTAYTGP
160 170 180 190 200
LVEDFLSGFQ VVVLTNTPLE DQLRVGEFCH NRGIKLVVAD TRGLFGQLFC
210 220 230 240 250
DFGEEMILTD SNGEQPLSAM VSMVTKDNPG VVTCLDEARH GFESGDFVSF
260 270 280 290 300
SEVQGMVELN GNQPMEIKVL GPYTFSICDT SNFSDYIRGG IVSQVKVPKK
310 320 330 340 350
ISFKSLVASL AEPDFVVTDF AKFSRPAQLH IGFQALHQFC AQHGRPPRPR
360 370 380 390 400
NEEDAAELVA LAQAVNARAL PAVQQNNLDE DLIRKLAYVA AGDLAPINAF
410 420 430 440 450
IGGLAAQEVM KACSGKFMPI MQWLYFDALE CLPEDKEVLT EDKCLQRQNR
460 470 480 490 500
YDGQVAVFGS DLQEKLGKQK YFLVGAGAIG CELLKNFAMI GLGCGEGGEI
510 520 530 540 550
IVTDMDTIEK SNLNRQFLFR PWDVTKLKSD TAAAAVRQMN PHIRVTSHQN
560 570 580 590 600
RVGPDTERIY DDDFFQNLDG VANALDNVDA RMYMDRRCVY YRKPLLESGT
610 620 630 640 650
LGTKGNVQVV IPFLTESYSS SQDPPEKSIP ICTLKNFPNA IEHTLQWARD
660 670 680 690 700
EFEGLFKQPA ENVNQYLTDP KFVERTLRLA GTQPLEVLEA VQRSLVLQRP
710 720 730 740 750
QTWADCVTWA CHHWHTQYSN NIRQLLHNFP PDQLTSSGAP FWSGPKRCPH
760 770 780 790 800
PLTFDVNNPL HLDYVMAAAN LFAQTYGLTG SQDRAAVATF LQSVQVPEFT
810 820 830 840 850
PKSGVKIHVS DQELQSANAS VDDSRLEELK ATLPSPDKLP GFKMYPIDFE
860 870 880 890 900
KDDDSNFHMD FIVAASNLRA ENYDIPSADR HKSKLIAGKI IPAIATTTAA
910 920 930 940 950
VVGLVCLELY KVVQGHRQLD SYKNGFLNLA LPFFGFSEPL AAPRHQYYNQ
960 970 980 990 1000
EWTLWDRFEV QGLQPNGEEM TLKQFLDYFK TEHKLEITML SQGVSMLYSF
1010 1020 1030 1040 1050
FMPAAKLKER LDQPMTEIVS RVSKRKLGRH VRALVLELCC NDESGEDVEV

PYVRYTIR
Length:1,058
Mass (Da):117,849
Last modified:October 25, 2002 - v3
<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:i4B413AAA75FAA562
GO
Isoform 2 (identifier: P22314-2) [UniParc]FASTAAdd to basket
Also known as: E1b

The sequence of this isoform differs from the canonical sequence as follows:
     1-40: Missing.

Show »
Length:1,018
Mass (Da):113,800
Checksum:i9A508347FE29FB19
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 6 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
Q5JRR6Q5JRR6_HUMAN
Ubiquitin-like modifier-activating ...
UBA1
506Annotation 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>
Q5JRS0Q5JRS0_HUMAN
Ubiquitin-like modifier-activating ...
UBA1
284Annotation 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>
Q5JRR9Q5JRR9_HUMAN
Ubiquitin-like modifier-activating ...
UBA1
270Annotation 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>
Q5JRS3Q5JRS3_HUMAN
Ubiquitin-like modifier-activating ...
UBA1
195Annotation 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>
Q5JRS1Q5JRS1_HUMAN
Ubiquitin-like modifier-activating ...
UBA1
173Annotation 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>
Q5JRS2Q5JRS2_HUMAN
Ubiquitin-like modifier-activating ...
UBA1
234Annotation 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 conflicti190D → G in CAA40296 (PubMed:1606621).Curated1
Sequence conflicti434E → Q in CAA40296 (PubMed:1606621).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_043500447R → H. Corresponds to variant dbSNP:rs2070169EnsemblClinVar.1
Natural variantiVAR_043501539M → I in SMAX2. 1 PublicationCorresponds to variant dbSNP:rs80356545EnsemblClinVar.1
Natural variantiVAR_043502547S → G in SMAX2. 1 PublicationCorresponds to variant dbSNP:rs80356546EnsemblClinVar.1
Natural variantiVAR_071121557E → V in SMAX2. 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_0559131 – 40Missing in isoform 2. CuratedAdd BLAST40

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
X56976 mRNA Translation: CAA40296.1
M58028 mRNA Translation: AAA61246.1
AL513366 Genomic DNA No translation available.
CH471164 Genomic DNA Translation: EAW59290.1
BC013041 mRNA Translation: AAH13041.1
X52897 mRNA Translation: CAA37078.1

The Consensus CDS (CCDS) project

More...
CCDSi
CCDS14275.1 [P22314-1]

Protein sequence database of the Protein Information Resource

More...
PIRi
A38564

NCBI Reference Sequences

More...
RefSeqi
NP_003325.2, NM_003334.3 [P22314-1]
NP_695012.1, NM_153280.2 [P22314-1]
XP_016885269.1, XM_017029780.1 [P22314-1]
XP_016885270.1, XM_017029781.1 [P22314-1]

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...
Ensembli
ENST00000335972; ENSP00000338413; ENSG00000130985 [P22314-1]
ENST00000377351; ENSP00000366568; ENSG00000130985 [P22314-1]

Database of genes from NCBI RefSeq genomes

More...
GeneIDi
7317

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...
KEGGi
hsa:7317

UCSC genome browser

More...
UCSCi
uc004dhj.5 human [P22314-1]

Keywords - Coding sequence diversityi

Alternative initiation, 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
X56976 mRNA Translation: CAA40296.1
M58028 mRNA Translation: AAA61246.1
AL513366 Genomic DNA No translation available.
CH471164 Genomic DNA Translation: EAW59290.1
BC013041 mRNA Translation: AAH13041.1
X52897 mRNA Translation: CAA37078.1
CCDSiCCDS14275.1 [P22314-1]
PIRiA38564
RefSeqiNP_003325.2, NM_003334.3 [P22314-1]
NP_695012.1, NM_153280.2 [P22314-1]
XP_016885269.1, XM_017029780.1 [P22314-1]
XP_016885270.1, XM_017029781.1 [P22314-1]

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
4P22X-ray2.75A/B1-439[»]
6DC6X-ray3.14A/C49-1058[»]
SMRiP22314
ModBaseiSearch...
PDBe-KBiSearch...

Protein-protein interaction databases

BioGridi113165, 170 interactors
DIPiDIP-33686N
IntActiP22314, 141 interactors
MINTiP22314
STRINGi9606.ENSP00000338413

Chemistry databases

BindingDBiP22314
ChEMBLiCHEMBL5924
DrugBankiDB04119 Hexatantalum Dodecabromide
DB04216 Quercetin

PTM databases

iPTMnetiP22314
PhosphoSitePlusiP22314
SwissPalmiP22314

Polymorphism and mutation databases

BioMutaiUBA1
DMDMi24418865

2D gel databases

REPRODUCTION-2DPAGEiIPI00645078

Proteomic databases

CPTACiCPTAC-136
CPTAC-137
EPDiP22314
jPOSTiP22314
MassIVEiP22314
MaxQBiP22314
PaxDbiP22314
PeptideAtlasiP22314
PRIDEiP22314
ProteomicsDBi53983 [P22314-1]

Protocols and materials databases

The DNASU plasmid repository

More...
DNASUi
7317

Genome annotation databases

EnsembliENST00000335972; ENSP00000338413; ENSG00000130985 [P22314-1]
ENST00000377351; ENSP00000366568; ENSG00000130985 [P22314-1]
GeneIDi7317
KEGGihsa:7317
UCSCiuc004dhj.5 human [P22314-1]

Organism-specific databases

Comparative Toxicogenomics Database

More...
CTDi
7317
DisGeNETi7317

GeneCards: human genes, protein and diseases

More...
GeneCardsi
UBA1
GeneReviewsiUBA1
HGNCiHGNC:12469 UBA1
HPAiCAB019435
CAB073410
HPA000289
HPA001506
MalaCardsiUBA1
MIMi301830 phenotype
314370 gene
neXtProtiNX_P22314
OpenTargetsiENSG00000130985
Orphaneti1145 Infantile-onset X-linked spinal muscular atrophy
PharmGKBiPA37119

GenAtlas: human gene database

More...
GenAtlasi
Search...

Phylogenomic databases

eggNOGiKOG2012 Eukaryota
COG0476 LUCA
GeneTreeiENSGT00940000158975
HOGENOMiHOG000167329
InParanoidiP22314
KOiK03178
OMAiCIVWARL
OrthoDBi588360at2759
PhylomeDBiP22314
TreeFamiTF300586

Enzyme and pathway databases

UniPathwayiUPA00143
BioCyciMetaCyc:HS05465-MONOMER
BRENDAi2.3.2.B5 2681
6.2.1.B9 2681
ReactomeiR-HSA-8866652 Synthesis of active ubiquitin: roles of E1 and E2 enzymes
R-HSA-983168 Antigen processing: Ubiquitination & Proteasome degradation
SIGNORiP22314

Miscellaneous databases

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

More...
ChiTaRSi
UBA1 human

The Gene Wiki collection of pages on human genes and proteins

More...
GeneWikii
UBA1

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

More...
GenomeRNAii
7317
PharosiP22314

Protein Ontology

More...
PROi
PR:P22314

The Stanford Online Universal Resource for Clones and ESTs

More...
SOURCEi
Search...

Gene expression databases

BgeeiENSG00000130985 Expressed in 243 organ(s), highest expression level in pituitary gland
ExpressionAtlasiP22314 baseline and differential
GenevisibleiP22314 HS

Family and domain databases

Gene3Di1.10.10.2660, 1 hit
2.40.30.180, 1 hit
3.10.290.60, 1 hit
3.50.50.80, 1 hit
InterProiView protein in InterPro
IPR032420 E1_4HB
IPR032418 E1_FCCH
IPR042302 E1_FCCH_sf
IPR000594 ThiF_NAD_FAD-bd
IPR018965 Ub-activating_enz_E1_C
IPR042449 Ub-E1_IAD_1
IPR038252 UBA_E1_C_sf
IPR019572 UBA_E1_Cys
IPR042063 Ubi_acti_E1_SCCH
IPR035985 Ubiquitin-activating_enz
IPR018075 UBQ-activ_enz_E1
IPR018074 UBQ-activ_enz_E1_CS
IPR033127 UBQ-activ_enz_E1_Cys_AS
IPR000011 UBQ/SUMO-activ_enz_E1-like
PfamiView protein in Pfam
PF16191 E1_4HB, 1 hit
PF16190 E1_FCCH, 1 hit
PF09358 E1_UFD, 1 hit
PF00899 ThiF, 2 hits
PF10585 UBA_e1_thiolCys, 1 hit
PRINTSiPR01849 UBIQUITINACT
SMARTiView protein in SMART
SM00985 UBA_e1_C, 1 hit
SUPFAMiSSF69572 SSF69572, 2 hits
TIGRFAMsiTIGR01408 Ube1, 1 hit
PROSITEiView protein in PROSITE
PS00536 UBIQUITIN_ACTIVAT_1, 1 hit
PS00865 UBIQUITIN_ACTIVAT_2, 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 nameiUBA1_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: P22314
Secondary accession number(s): Q5JRR8, Q96E13
<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_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: August 1, 1991
Last sequence update: October 25, 2002
Last modified: October 16, 2019
This is version 221 of the entry and version 3 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, Complete proteome, Direct protein sequencing, 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. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  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 X
    Human chromosome X: entries, gene names and cross-references to MIM
  7. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
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