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UniProtKB - P07237 (PDIA1_HUMAN)

Entry version 259 (23 Feb 2022)
Sequence version 3 (01 Nov 1997)
Previous versions | rss
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Protein

Protein disulfide-isomerase

Gene

P4HB

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

This multifunctional protein catalyzes the formation, breakage and rearrangement of disulfide bonds. At the cell surface, seems to act as a reductase that cleaves disulfide bonds of proteins attached to the cell. May therefore cause structural modifications of exofacial proteins. Inside the cell, seems to form/rearrange disulfide bonds of nascent proteins. At high concentrations, functions as a chaperone that inhibits aggregation of misfolded proteins. At low concentrations, facilitates aggregation (anti-chaperone activity). May be involved with other chaperones in the structural modification of the TG precursor in hormone biogenesis. Also acts a structural subunit of various enzymes such as prolyl 4-hydroxylase and microsomal triacylglycerol transfer protein MTTP. Receptor for LGALS9; the interaction retains P4HB at the cell surface of Th2 T helper cells, increasing disulfide reductase activity at the plasma membrane, altering the plasma membrane redox state and enhancing cell migration (PubMed:21670307).

3 Publications

Miscellaneous

Reduces and may activate fusogenic properties of HIV-1 gp120 surface protein, thereby enabling HIV-1 entry into the cell.

<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

  • Catalyzes the rearrangement of -S-S- bonds in proteins. EC:5.3.4.1

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 sitei53Nucleophile1
<p>This subsection describes interesting single amino acid sites on the sequence that are not defined in any other subsection. This subsection can be displayed in different sections ('Function', 'PTM / Processing', 'Pathology and Biotech') according to its content.<p><a href='/help/site' target='_top'>More...</a></p>Sitei54Contributes to redox potential value1
Sitei55Contributes to redox potential value1
Active sitei56Nucleophile1
Sitei120Lowers pKa of C-terminal Cys of first active site1
Active sitei397NucleophileBy similarity1
Sitei398Contributes to redox potential valueBy similarity1
Sitei399Contributes to redox potential valueBy similarity1
Active sitei400NucleophileBy similarity1
Sitei461Lowers pKa of C-terminal Cys of second active siteBy similarity1

<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 functionChaperone, Isomerase

Enzyme and pathway databases

BRENDA Comprehensive Enzyme Information System

More...BRENDAi		5.3.4.1, 2681

Pathway Commons web resource for biological pathway data

More...PathwayCommonsi		P07237

Reactome - a knowledgebase of biological pathways and processes

More...Reactomei		R-HSA-1650814, Collagen biosynthesis and modifying enzymes
R-HSA-3299685, Detoxification of Reactive Oxygen Species
R-HSA-381426, Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)
R-HSA-5358346, Hedgehog ligand biogenesis
R-HSA-8866423, VLDL assembly
R-HSA-8957275, Post-translational protein phosphorylation
R-HSA-8963888, Chylomicron assembly
R-HSA-8964041, LDL remodeling
R-HSA-9020591, Interleukin-12 signaling
R-HSA-9020933, Interleukin-23 signaling

SignaLink: a signaling pathway resource with multi-layered regulatory networks

More...SignaLinki		P07237

<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:
Protein disulfide-isomerase (EC:5.3.4.1)
Short name:
PDI
Alternative name(s):
Cellular thyroid hormone-binding protein
Prolyl 4-hydroxylase subunit beta
p55
<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:P4HB
Synonyms:ERBA2L, PDI, PDIA1, PO4DB
<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 17

Organism-specific databases

Human Gene Nomenclature Database

More...HGNCi		HGNC:8548, P4HB

Online Mendelian Inheritance in Man (OMIM)

More...MIMi		176790, gene

neXtProt; the human protein knowledge platform

More...neXtProti		NX_P07237

Eukaryotic Pathogen, Vector and Host Database Resources

More...VEuPathDBi		HostDB:ENSG00000185624

<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

Keywords - Cellular componenti

Cell membrane, 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

<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

Cole-Carpenter syndrome 1 (CLCRP1)1 Publication
The disease is caused by variants affecting the gene represented in this entry.
Disease descriptionA form of Cole-Carpenter syndrome, a disorder characterized by features of osteogenesis imperfecta such as bone deformities and severe bone fragility with frequent fractures, in association with craniosynostosis, ocular proptosis, hydrocephalus, growth failure and distinctive facial features. Craniofacial findings include marked frontal bossing, midface hypoplasia, and micrognathia. Despite the craniosynostosis and hydrocephalus, intellectual development is normal. CLCRP1 inheritance is autosomal dominant.
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_073440393Y → C in CLCRP1; impairs ability to act as a disulfide isomerase enzyme. 1 PublicationCorresponds to variant dbSNP:rs786204843EnsemblClinVar.1

Keywords - Diseasei

Craniosynostosis, Disease variant, Osteogenesis imperfecta

Organism-specific databases

DisGeNET

More...DisGeNETi		5034

MalaCards human disease database

More...MalaCardsi		P4HB
MIMi112240, phenotype

Open Targets

More...OpenTargetsi		ENSG00000185624

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

More...Orphaneti		2050, Cole-Carpenter syndrome
216796, Osteogenesis imperfecta type 1

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...PharmGKBi		PA32876

Miscellaneous databases

Pharos NIH Druggable Genome Knowledgebase

More...Pharosi		P07237, Tbio

Chemistry databases

ChEMBL database of bioactive drug-like small molecules

More...ChEMBLi		CHEMBL5422

Drug and drug target database

More...DrugBanki		DB11638, Artenimol
DB09130, Copper
DB03615, Ribostamycin
DB01593, Zinc
DB14487, Zinc acetate
DB14533, Zinc chloride
DB14548, Zinc sulfate, unspecified form

Genetic variation databases

BioMuta curated single-nucleotide variation and disease association database

More...BioMutai		P4HB

Domain mapping of disease mutations (DMDM)

More...DMDMi		2507460

<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 denotes the presence of an N-terminal signal peptide.<p><a href='/help/signal' target='_top'>More...</a></p>Signal peptidei1 – 17Combined sources4 PublicationsAdd BLAST17
<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_000003419518 – 508Protein disulfide-isomeraseAdd BLAST491

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the PTM / Processing":/help/ptm_processing_section section describes the positions of cysteine residues participating in disulfide bonds.<p><a href='/help/disulfid' target='_top'>More...</a></p>Disulfide bondi53 ↔ 56Redox-activePROSITE-ProRule annotation1 Publication
<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 residuei200N6-acetyllysineBy similarity1
Modified residuei222N6-succinyllysineBy similarity1
Modified residuei271N6-succinyllysineBy similarity1
Modified residuei357Phosphoserine; by FAM20C1 Publication1
Disulfide bondi397 ↔ 400Redox-activePROSITE-ProRule annotation

Keywords - PTMi

Acetylation, Disulfide bond, Phosphoprotein

Proteomic databases

Encyclopedia of Proteome Dynamics

More...EPDi		P07237

jPOST - Japan Proteome Standard Repository/Database

More...jPOSTi		P07237

MassIVE - Mass Spectrometry Interactive Virtual Environment

More...MassIVEi		P07237

MaxQB - The MaxQuant DataBase

More...MaxQBi		P07237

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

More...PaxDbi		P07237

PeptideAtlas

More...PeptideAtlasi		P07237

PRoteomics IDEntifications database

More...PRIDEi		P07237

ProteomicsDB: a multi-organism proteome resource

More...ProteomicsDBi		51976

Consortium for Top Down Proteomics

More...TopDownProteomicsi		P07237

2D gel databases

DOSAC-COBS 2D-PAGE database

More...DOSAC-COBS-2DPAGEi		P07237

USC-OGP 2-DE database

More...OGPi		P07237

REPRODUCTION-2DPAGE

More...REPRODUCTION-2DPAGEi		IPI00010796
P07237

Two-dimensional polyacrylamide gel electrophoresis database from the Geneva University Hospital

More...SWISS-2DPAGEi		P07237

PTM databases

GlyGen: Computational and Informatics Resources for Glycoscience

More...GlyGeni		P07237, 1 site, 1 O-linked glycan (1 site)

iPTMnet integrated resource for PTMs in systems biology context

More...iPTMneti		P07237

MetOSite database of methionine sulfoxide sites

More...MetOSitei		P07237

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

More...PhosphoSitePlusi		P07237

SwissPalm database of S-palmitoylation events

More...SwissPalmi		P07237

<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

More...Bgeei		ENSG00000185624, Expressed in stromal cell of endometrium and 245 other tissues

ExpressionAtlas, Differential and Baseline Expression

More...ExpressionAtlasi		P07237, baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

More...Genevisiblei		P07237, HS

Organism-specific databases

Human Protein Atlas

More...HPAi		ENSG00000185624, Tissue enhanced (liver, pancreas)

<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

Heterodimer; heterodimerizes with the protein microsomal triglyceride transfer MTTP (PubMed:23475612, PubMed:26224785, PubMed:16478722). Homodimer. Monomers and homotetramers may also occur.

Interacts with P4HA2, forming a heterotetramer consisting of 2 alpha subunits (P4HA2) and 2 beta (P4HB), where P4HB plays the role of a structural subunit; this tetramer catalyzes the formation of 4-hydroxyproline in collagen (PubMed:7753822). Also constitutes the structural subunit of the microsomal triacylglycerol transfer protein MTTP in mammalian cells. Stabilizes both enzymes and retain them in the ER without contributing to the catalytic activity (By similarity). Binds UBQLN1 (PubMed:12095988).

Interacts with ERO1B (PubMed:11707400). Binds to CD4, and upon HIV-1 binding to the cell membrane, is part of a P4HB/PDI-CD4-CXCR4-gp120 complex.

Interacts with ILDR2 (By similarity).

By similarity6 Publications

<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
P07237
With#Exp.IntAct
ANKEF1 [Q9NU02]3EBI-395883,EBI-8464238
BBS4 [Q96RK4]3EBI-395883,EBI-1805814
BCL2L11 [O43521]3EBI-395883,EBI-526406
CAPN2 [P17655]3EBI-395883,EBI-1028956
CCDC26 [Q8TAB7]3EBI-395883,EBI-10271580
CEP76 [Q8TAP6]3EBI-395883,EBI-742887
CFAP100 - isoform 2 [Q494V2-2]3EBI-395883,EBI-11953200
CHCHD2 [Q9Y6H1]3EBI-395883,EBI-2321769
CNNM3 [Q8NE01]3EBI-395883,EBI-741032
CST2 [P09228]3EBI-395883,EBI-8832659
DDIT4L [Q96D03]3EBI-395883,EBI-742054
ERO1A [Q96HE7]2EBI-395883,EBI-2564539
FAM71C [Q8NEG0]3EBI-395883,EBI-752049
FASTKD1 [Q53R41]3EBI-395883,EBI-3957005
FZD10 [Q9ULW2]3EBI-395883,EBI-8803802
GNB1 [P62873]3EBI-395883,EBI-357130
GRN [P28799]4EBI-395883,EBI-747754
HM13 [Q8TCT9]3EBI-395883,EBI-347472
KLHL36 - isoform 2 [Q8N4N3-2]3EBI-395883,EBI-10973851
KLHL42 [Q9P2K6]3EBI-395883,EBI-739890
KRT6A [P02538]3EBI-395883,EBI-702198
KRTAP12-3 [P60328]3EBI-395883,EBI-11953334
KRTAP13-2 [Q52LG2]3EBI-395883,EBI-11953846
KRTAP4-11 [Q9BYQ6]3EBI-395883,EBI-10302392
KRTAP5-9 [P26371]3EBI-395883,EBI-3958099
KRTAP9-8 [Q9BYQ0]3EBI-395883,EBI-11958364
LCE1B [Q5T7P3]3EBI-395883,EBI-10245913
LCE2D [Q5TA82]3EBI-395883,EBI-10246750
LCE4A [Q5TA78]3EBI-395883,EBI-10246358
LCN2 [P80188]3EBI-395883,EBI-11911016
LRFN4 [Q6PJG9]3EBI-395883,EBI-7910762
NECTIN2 - isoform Alpha [Q92692-2]3EBI-395883,EBI-6979889
NOL4 - isoform 2 [O94818-2]3EBI-395883,EBI-10190763
NTM - isoform 3 [Q9P121-3]3EBI-395883,EBI-12027160
ODF1 [Q14990]3EBI-395883,EBI-10234557
PER1 [O15534]3EBI-395883,EBI-2557276
PLCXD3 [Q63HM9]3EBI-395883,EBI-12105500
PLK4 [O00444]3EBI-395883,EBI-746202
PRDX4 [Q13162]2EBI-395883,EBI-2211957
RBBP4 [Q09028]3EBI-395883,EBI-620823
SH3KBP1 [Q96B97]3EBI-395883,EBI-346595
SKAP1 - isoform 2 [Q86WV1-2]3EBI-395883,EBI-11995314
SLC22A17 [Q8WUG5]3EBI-395883,EBI-11722858
TAP1 [Q03518]4EBI-395883,EBI-747259
TEX35 - isoform 2 [Q5T0J7-2]3EBI-395883,EBI-12833746
TGFB1 [P01137]3EBI-395883,EBI-779636
ZNF69 - isoform 2 [Q9UC07-2]3EBI-395883,EBI-12310821
APH_0248 [Q2GL86] from Anaplasma phagocytophilum (strain HZ).3EBI-395883,EBI-26435798

GO - Molecular functioni

Complete GO annotation on QuickGO ...

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGRID)

More...BioGRIDi		111073, 319 interactors

CORUM comprehensive resource of mammalian protein complexes

More...CORUMi		P07237

Database of interacting proteins

More...DIPi		DIP-32979N

Protein interaction database and analysis system

More...IntActi		P07237, 175 interactors

Molecular INTeraction database

More...MINTi		P07237

STRING: functional protein association networks

More...STRINGi		9606.ENSP00000327801

Chemistry databases

BindingDB database of measured binding affinities

More...BindingDBi		P07237

Miscellaneous databases

RNAct, Protein-RNA interaction predictions for model organisms.

More...RNActi		P07237, 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

1508
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

Biological Magnetic Resonance Data Bank

More...BMRBi		P07237

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

More...SMRi		P07237

Database of comparative protein structure models

More...ModBasei		Search...

Protein Data Bank in Europe - Knowledge Base

More...PDBe-KBi		Search...

Miscellaneous databases

Relative evolutionary importance of amino acids within a protein sequence

More...EvolutionaryTracei		P07237

<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 <a href="http://www.uniprot.org/help/family%5Fand%5Fdomains%5Fsection">Family and Domains</a> section describes the position and type of a domain, which is defined as a specific combination of secondary structures organized into a characteristic three-dimensional structure or fold.<p><a href='/help/domain' target='_top'>More...</a></p>Domaini18 – 134Thioredoxin 1PROSITE-ProRule annotationAdd BLAST117
Domaini349 – 475Thioredoxin 2PROSITE-ProRule annotationAdd BLAST127

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>Regioni471 – 508DisorderedSequence analysisAdd BLAST38

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>Motifi505 – 508Prevents secretion from ER4

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 biasi476 – 508Acidic residuesSequence analysisAdd BLAST33

<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 protein disulfide isomerase family.Curated

Keywords - Domaini

Redox-active center, Repeat, Signal

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...eggNOGi		KOG0190, Eukaryota

Ensembl GeneTree

More...GeneTreei		ENSGT00940000157351

InParanoid: Eukaryotic Ortholog Groups

More...InParanoidi		P07237

Identification of Orthologs from Complete Genome Data

More...OMAi		IEKYVDP

Database of Orthologous Groups

More...OrthoDBi		462118at2759

Database for complete collections of gene phylogenies

More...PhylomeDBi		P07237

TreeFam database of animal gene trees

More...TreeFami		TF106381

Family and domain databases

Database of protein disorder

More...DisProti		DP02637

Integrated resource of protein families, domains and functional sites

More...InterProi		View protein in InterPro
IPR005788, Disulphide_isomerase
IPR005792, Prot_disulphide_isomerase
IPR036249, Thioredoxin-like_sf
IPR017937, Thioredoxin_CS
IPR013766, Thioredoxin_domain

Pfam protein domain database

More...Pfami		View protein in Pfam
PF00085, Thioredoxin, 2 hits

Superfamily database of structural and functional annotation

More...SUPFAMi		SSF52833, SSF52833, 4 hits

TIGRFAMs; a protein family database

More...TIGRFAMsi		TIGR01130, ER_PDI_fam, 1 hit
TIGR01126, pdi_dom, 2 hits

PROSITE; a protein domain and family database

More...PROSITEi		View protein in PROSITE
PS00014, ER_TARGET, 1 hit
PS00194, THIOREDOXIN_1, 2 hits
PS51352, THIOREDOXIN_2, 2 hits

<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>Sequence (1+)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 has 1 described isoform and 31 potential isoforms that are computationally mapped.Show allAlign All

P07237-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MLRRALLCLA VAALVRADAP EEEDHVLVLR KSNFAEALAA HKYLLVEFYA 
        60         70         80         90        100
PWCGHCKALA PEYAKAAGKL KAEGSEIRLA KVDATEESDL AQQYGVRGYP 
       110        120        130        140        150
TIKFFRNGDT ASPKEYTAGR EADDIVNWLK KRTGPAATTL PDGAAAESLV 
       160        170        180        190        200
ESSEVAVIGF FKDVESDSAK QFLQAAEAID DIPFGITSNS DVFSKYQLDK 
       210        220        230        240        250
DGVVLFKKFD EGRNNFEGEV TKENLLDFIK HNQLPLVIEF TEQTAPKIFG 
       260        270        280        290        300
GEIKTHILLF LPKSVSDYDG KLSNFKTAAE SFKGKILFIF IDSDHTDNQR 
       310        320        330        340        350
ILEFFGLKKE ECPAVRLITL EEEMTKYKPE SEELTAERIT EFCHRFLEGK 
       360        370        380        390        400
IKPHLMSQEL PEDWDKQPVK VLVGKNFEDV AFDEKKNVFV EFYAPWCGHC 
       410        420        430        440        450
KQLAPIWDKL GETYKDHENI VIAKMDSTAN EVEAVKVHSF PTLKFFPASA 
       460        470        480        490        500
DRTVIDYNGE RTLDGFKKFL ESGGQDGAGD DDDLEDLEEA EEPDMEEDDD 

QKAVKDEL
Length:508
Mass (Da):57,116
Last modified:November 1, 1997 - 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:i906CE6D9900B8FCE
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 31 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A0A7P0T8J3A0A7P0T8J3_HUMAN
Protein disulfide-isomerase
P4HB
488Annotation score:

Annotation score:2 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>
A0A7P0T8X1A0A7P0T8X1_HUMAN
Protein disulfide-isomerase
P4HB
479Annotation score:

Annotation score:2 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>
A0A7P0T9U6A0A7P0T9U6_HUMAN
Protein disulfide-isomerase
P4HB
486Annotation score:

Annotation score:2 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>
A0A7P0TA35A0A7P0TA35_HUMAN
Protein disulfide-isomerase
P4HB
542Annotation score:

Annotation score:2 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>
A0A7P0TB26A0A7P0TB26_HUMAN
Protein disulfide-isomerase
P4HB
533Annotation score:

Annotation score:2 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>
I3L3P5I3L3P5_HUMAN
Protein disulfide-isomerase
P4HB
472Annotation score:

Annotation score:2 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>
A0A7P0T9K9A0A7P0T9K9_HUMAN
Protein disulfide-isomerase
P4HB
439Annotation score:

Annotation score:2 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>
I3L4M2I3L4M2_HUMAN
Protein disulfide-isomerase
P4HB
537Annotation score:

Annotation score:2 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>
A0A7P0TA71A0A7P0TA71_HUMAN
Protein disulfide-isomerase
P4HB
557Annotation score:

Annotation score:2 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>
A0A7P0TA97A0A7P0TA97_HUMAN
Protein disulfide-isomerase
P4HB
479Annotation score:

Annotation score:2 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>
There are more potential isoformsShow all

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 conflicti10 – 11AV → PW in CAA28775 (PubMed:3034602).Curated2
Sequence conflicti21E → D AA sequence (PubMed:9399589).Curated1
Sequence conflicti24D → V AA sequence (PubMed:9399589).Curated1
Sequence conflicti44 – 45LL → PP in CAA28775 (PubMed:3034602).Curated2
Sequence conflicti49Y → H in CAA28775 (PubMed:3034602).Curated1
Sequence conflicti141P → R in AAA61169 (PubMed:3611107).Curated1
Sequence conflicti360 – 362LPE → RAG in AAA61169 (PubMed:3611107).Curated3
Sequence conflicti372L → P in AAA61169 (PubMed:3611107).Curated1
Sequence conflicti439S → G in CAA28775 (PubMed:3034602).Curated1
Sequence conflicti444K → G in CAA28775 (PubMed:3034602).Curated1
Sequence conflicti460E → Q in CAA30112 (PubMed:3342239).Curated1
Sequence conflicti481D → V in CAA28775 (PubMed:3034602).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_073440393Y → C in CLCRP1; impairs ability to act as a disulfide isomerase enzyme. 1 PublicationCorresponds to variant dbSNP:rs786204843EnsemblClinVar.1

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
X05130 mRNA Translation: CAA28775.1
J02783 mRNA Translation: AAA61169.1
M22806 M22805 Genomic DNA Translation: AAC13652.1
AK315631 mRNA Translation: BAG37999.1
CH471099 Genomic DNA Translation: EAW89690.1
BC010859 mRNA Translation: AAH10859.1
BC029617 mRNA Translation: AAH29617.1
BC071892 mRNA Translation: AAH71892.1
S37207 Genomic DNA Translation: AAB22262.2
X07077 mRNA Translation: CAA30112.1

The Consensus CDS (CCDS) project

More...CCDSi		CCDS11787.1

Protein sequence database of the Protein Information Resource

More...PIRi		A31913, ISHUSS

NCBI Reference Sequences

More...RefSeqi		NP_000909.2, NM_000918.3

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...Ensembli		ENST00000331483; ENSP00000327801; ENSG00000185624

Database of genes from NCBI RefSeq genomes

More...GeneIDi		5034

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...KEGGi		hsa:5034

Matched Annotation from NCBI and EMBL-EBI (MANE) - Phase one

More...MANE-Selecti		ENST00000331483.9; ENSP00000327801.4; NM_000918.4; NP_000909.2

UCSC genome browser

More...UCSCi		uc002kbn.2, human

<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
X05130 mRNA Translation: CAA28775.1
J02783 mRNA Translation: AAA61169.1
M22806 M22805 Genomic DNA Translation: AAC13652.1
AK315631 mRNA Translation: BAG37999.1
CH471099 Genomic DNA Translation: EAW89690.1
BC010859 mRNA Translation: AAH10859.1
BC029617 mRNA Translation: AAH29617.1
BC071892 mRNA Translation: AAH71892.1
S37207 Genomic DNA Translation: AAB22262.2
X07077 mRNA Translation: CAA30112.1
CCDSiCCDS11787.1
PIRiA31913, ISHUSS
RefSeqiNP_000909.2, NM_000918.3

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
1BJXNMR-A136-245[»]
1MEKNMR-A18-137[»]
1X5CNMR-A368-475[»]
2BJXNMR-A136-245[»]
2K18NMR-A135-357[»]
3BJ5X-ray2.20A230-368[»]
3UEMX-ray2.29A137-479[»]
4EKZX-ray2.51A18-479[»]
4EL1X-ray2.88A/B18-479[»]
4JU5X-ray2.28A/B135-367[»]
6I7SX-ray2.50A/B18-508[»]
BMRBiP07237
SMRiP07237
ModBaseiSearch...
PDBe-KBiSearch...

Protein-protein interaction databases

BioGRIDi111073, 319 interactors
CORUMiP07237
DIPiDIP-32979N
IntActiP07237, 175 interactors
MINTiP07237
STRINGi9606.ENSP00000327801

Chemistry databases

BindingDBiP07237
ChEMBLiCHEMBL5422
DrugBankiDB11638, Artenimol
DB09130, Copper
DB03615, Ribostamycin
DB01593, Zinc
DB14487, Zinc acetate
DB14533, Zinc chloride
DB14548, Zinc sulfate, unspecified form

PTM databases

GlyGeniP07237, 1 site, 1 O-linked glycan (1 site)
iPTMnetiP07237
MetOSiteiP07237
PhosphoSitePlusiP07237
SwissPalmiP07237

Genetic variation databases

BioMutaiP4HB
DMDMi2507460

2D gel databases

DOSAC-COBS-2DPAGEiP07237
OGPiP07237
REPRODUCTION-2DPAGEiIPI00010796
P07237
SWISS-2DPAGEiP07237

Proteomic databases

EPDiP07237
jPOSTiP07237
MassIVEiP07237
MaxQBiP07237
PaxDbiP07237
PeptideAtlasiP07237
PRIDEiP07237
ProteomicsDBi51976
TopDownProteomicsiP07237

Protocols and materials databases

Antibodypedia a portal for validated antibodies

More...Antibodypediai		3250, 555 antibodies from 43 providers

The DNASU plasmid repository

More...DNASUi		5034

Genome annotation databases

EnsembliENST00000331483; ENSP00000327801; ENSG00000185624
GeneIDi5034
KEGGihsa:5034
MANE-SelectiENST00000331483.9; ENSP00000327801.4; NM_000918.4; NP_000909.2
UCSCiuc002kbn.2, human

Organism-specific databases

Comparative Toxicogenomics Database

More...CTDi		5034
DisGeNETi5034

GeneCards: human genes, protein and diseases

More...GeneCardsi		P4HB
HGNCiHGNC:8548, P4HB
HPAiENSG00000185624, Tissue enhanced (liver, pancreas)
MalaCardsiP4HB
MIMi112240, phenotype
176790, gene
neXtProtiNX_P07237
OpenTargetsiENSG00000185624
Orphaneti2050, Cole-Carpenter syndrome
216796, Osteogenesis imperfecta type 1
PharmGKBiPA32876
VEuPathDBiHostDB:ENSG00000185624

GenAtlas: human gene database

More...GenAtlasi		Search...

Phylogenomic databases

eggNOGiKOG0190, Eukaryota
GeneTreeiENSGT00940000157351
InParanoidiP07237
OMAiIEKYVDP
OrthoDBi462118at2759
PhylomeDBiP07237
TreeFamiTF106381

Enzyme and pathway databases

BRENDAi5.3.4.1, 2681
PathwayCommonsiP07237
ReactomeiR-HSA-1650814, Collagen biosynthesis and modifying enzymes
R-HSA-3299685, Detoxification of Reactive Oxygen Species
R-HSA-381426, Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)
R-HSA-5358346, Hedgehog ligand biogenesis
R-HSA-8866423, VLDL assembly
R-HSA-8957275, Post-translational protein phosphorylation
R-HSA-8963888, Chylomicron assembly
R-HSA-8964041, LDL remodeling
R-HSA-9020591, Interleukin-12 signaling
R-HSA-9020933, Interleukin-23 signaling
SignaLinkiP07237

Miscellaneous databases

BioGRID ORCS database of CRISPR phenotype screens

More...BioGRID-ORCSi		5034, 23 hits in 1059 CRISPR screens

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

More...ChiTaRSi		P4HB, human
EvolutionaryTraceiP07237

The Gene Wiki collection of pages on human genes and proteins

More...GeneWikii		P4HB

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

More...GenomeRNAii		5034
PharosiP07237, Tbio

Protein Ontology

More...PROi		PR:P07237
RNActiP07237, protein

The Stanford Online Universal Resource for Clones and ESTs

More...SOURCEi		Search...

Gene expression databases

BgeeiENSG00000185624, Expressed in stromal cell of endometrium and 245 other tissues
ExpressionAtlasiP07237, baseline and differential
GenevisibleiP07237, HS

Family and domain databases

DisProtiDP02637
InterProiView protein in InterPro
IPR005788, Disulphide_isomerase
IPR005792, Prot_disulphide_isomerase
IPR036249, Thioredoxin-like_sf
IPR017937, Thioredoxin_CS
IPR013766, Thioredoxin_domain
PfamiView protein in Pfam
PF00085, Thioredoxin, 2 hits
SUPFAMiSSF52833, SSF52833, 4 hits
TIGRFAMsiTIGR01130, ER_PDI_fam, 1 hit
TIGR01126, pdi_dom, 2 hits
PROSITEiView protein in PROSITE
PS00014, ER_TARGET, 1 hit
PS00194, THIOREDOXIN_1, 2 hits
PS51352, THIOREDOXIN_2, 2 hits

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 nameiPDIA1_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: P07237
Secondary accession number(s): B2RDQ2
, P30037, P32079, Q15205, Q6LDE5
<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: April 1, 1988
Last sequence update: November 1, 1997
Last modified: February 23, 2022
This is version 259 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, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 17
    Human chromosome 17: 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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families
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