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Protein

Complement C1r subcomponent

Gene

C1R

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

C1r B chain is a serine protease that combines with C1q and C1s to form C1, the first component of the classical pathway of the complement system.

<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

  • Selective cleavage of Lys(or Arg)-|-Ile bond in complement subcomponent C1s to form the active form of C1s (EC 3.4.21.42). EC:3.4.21.41

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Function’ 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 sitei502Charge relay system1
Active sitei557Charge relay system1
Active sitei654Charge relay system1

<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

  • calcium ion binding Source: CAFA
  • serine-type endopeptidase activity Source: Reactome
  • serine-type peptidase activity Source: ProtInc

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 functionHydrolase, Protease, Serine protease
Biological processComplement pathway, Immunity, Innate immunity

Enzyme and pathway databases

BRENDA Comprehensive Enzyme Information System

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BRENDAi
3.4.21.41 2681

Reactome - a knowledgebase of biological pathways and processes

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Reactomei
R-HSA-166663 Initial triggering of complement
R-HSA-173623 Classical antibody-mediated complement activation
R-HSA-977606 Regulation of Complement cascade

SABIO-RK: Biochemical Reaction Kinetics Database

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SABIO-RKi
P00736

SIGNOR Signaling Network Open Resource

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SIGNORi
P00736

Protein family/group databases

MEROPS protease database

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MEROPSi
S01.192

<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:
Complement C1r subcomponent (EC:3.4.21.41)
Alternative name(s):
Complement component 1 subcomponent r
Cleaved into the following 2 chains:
<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:C1R
<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 12

Organism-specific databases

Eukaryotic Pathogen Database Resources

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EuPathDBi
HostDB:ENSG00000159403.15

Human Gene Nomenclature Database

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HGNCi
HGNC:1246 C1R

Online Mendelian Inheritance in Man (OMIM)

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MIMi
613785 gene

neXtProt; the human protein knowledge platform

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neXtProti
NX_P00736

<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; Source: COMPARTMENTS

Keywords - Cellular componenti

Secreted

<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

Ehlers-Danlos syndrome, periodontal type, 1 (EDSPD1)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA form of Ehlers-Danlos syndrome, a connective tissue disorder characterized by hyperextensible skin, atrophic cutaneous scars due to tissue fragility and joint hyperlaxity. EDSPD1 is characterized by the association of typical features of Ehlers-Danlos syndrome with gingival recession and severe early-onset periodontal disease, leading to premature loss of permanent teeth. EDSPD1 inheritance is autosomal dominant.
See also OMIM:130080
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_07710650V → D in EDSPD1; requires 2 nucleotide substitutions; the mutant is not secreted but retained intracellularly. 1 Publication1
Natural variantiVAR_077107290D → G in EDSPD1; unknown pathological significance. 1 Publication1
Natural variantiVAR_077108297G → D in EDSPD1; unknown pathological significance. 1 Publication1
Natural variantiVAR_077109300L → P in EDSPD1; unknown pathological significance. 1 Publication1
Natural variantiVAR_077110301R → P in EDSPD1; unknown pathological significance. 1 Publication1
Natural variantiVAR_077111302Y → C in EDSPD1. 1 Publication1
Natural variantiVAR_077112306 – 309IIKC → RR in EDSPD1. 1 Publication4
Natural variantiVAR_077113309C → W in EDSPD1; the mutant is not secreted but retained intracellularly. 1 Publication1
Natural variantiVAR_077114338C → R in EDSPD1. 1 Publication1
Natural variantiVAR_077115358C → F in EDSPD1. 1 Publication1
Natural variantiVAR_077116364W → C in EDSPD1; unknown pathological significance. 1 Publication1
Natural variantiVAR_077117371C → W in EDSPD1; the mutant is not secreted but retained intracellularly. 1 Publication1
Natural variantiVAR_077118401 – 405RIQYY → HVI in EDSPD1; unknown pathological significance. 1 Publication5
Natural variantiVAR_077119435W → R in EDSPD1; unknown pathological significance. 1 Publication1

Keywords - Diseasei

Disease mutation, Ehlers-Danlos syndrome

Organism-specific databases

DisGeNET

More...
DisGeNETi
715

MalaCards human disease database

More...
MalaCardsi
C1R
MIMi130080 phenotype
216950 phenotype

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

More...
Orphaneti
75392 Ehlers-Danlos syndrome, periodontitis type
169147 Immunodeficiency due to a classical component pathway complement deficiency
93552 Pediatric systemic lupus erythematosus

The Pharmacogenetics and Pharmacogenomics Knowledge Base

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PharmGKBi
PA25635

Chemistry databases

ChEMBL database of bioactive drug-like small molecules

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ChEMBLi
CHEMBL4611

Drug and drug target database

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DrugBanki
DB00054 Abciximab
DB00051 Adalimumab
DB00092 Alefacept
DB00087 Alemtuzumab
DB00074 Basiliximab
DB00112 Bevacizumab
DB06404 C1 Esterase Inhibitor (Human)
DB09228 C1 Esterase Inhibitor (Recombinant)
DB00002 Cetuximab
DB00111 Daclizumab
DB00095 Efalizumab
DB00005 Etanercept
DB00056 Gemtuzumab ozogamicin
DB00078 Ibritumomab tiuxetan
DB00075 Muromonab
DB00108 Natalizumab
DB00110 Palivizumab
DB00073 Rituximab
DB00081 Tositumomab
DB00072 Trastuzumab

IUPHAR/BPS Guide to PHARMACOLOGY

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GuidetoPHARMACOLOGYi
2334

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

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BioMutai
C1R

Domain mapping of disease mutations (DMDM)

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DMDMi
218511956

<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 – 171 PublicationAdd BLAST17
<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_000002757718 – 705Complement C1r subcomponentAdd BLAST688
ChainiPRO_000002757818 – 463Complement C1r subcomponent heavy chainAdd BLAST446
ChainiPRO_0000027579464 – 705Complement C1r subcomponent light chainAdd BLAST242

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 bondi71 ↔ 89Curated
<p>This subsection of the <a href="http://www.uniprot.org/help/ptm_processing_section">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>Glycosylationi125N-linked (GlcNAc...) asparagine1 Publication1
Disulfide bondi146 ↔ 165
Disulfide bondi161 ↔ 174
<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 residuei167(3R)-3-hydroxyasparagine1 Publication1
Disulfide bondi176 ↔ 189
Disulfide bondi193 ↔ 220Curated
Modified residuei206Phosphoserine; by CK21 Publication1
Glycosylationi221N-linked (GlcNAc...) asparagine1 Publication1
Disulfide bondi250 ↔ 268Curated
Disulfide bondi309 ↔ 358
Disulfide bondi338 ↔ 371
Disulfide bondi376 ↔ 429
Disulfide bondi406 ↔ 447
Disulfide bondi451 ↔ 577Interchain (between heavy and light chains)
Glycosylationi514N-linked (GlcNAc...) asparagine2 Publications1
Glycosylationi581N-linked (GlcNAc...) asparagine1
Disulfide bondi620 ↔ 639
Disulfide bondi650 ↔ 680

<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

The iron and 2-oxoglutarate dependent 3-hydroxylation of aspartate and asparagine is (R) stereospecific within EGF domains.1 Publication

Keywords - PTMi

Disulfide bond, Glycoprotein, Hydroxylation, Phosphoprotein

Proteomic databases

Encyclopedia of Proteome Dynamics

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EPDi
P00736

jPOST - Japan Proteome Standard Repository/Database

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jPOSTi
P00736

PeptideAtlas

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PeptideAtlasi
P00736

PRoteomics IDEntifications database

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PRIDEi
P00736

ProteomicsDB human proteome resource

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ProteomicsDBi
51270

PTM databases

GlyConnect protein glycosylation platform

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GlyConnecti
713

iPTMnet integrated resource for PTMs in systems biology context

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iPTMneti
P00736

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

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PhosphoSitePlusi
P00736

<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
ENSG00000159403 Expressed in 221 organ(s), highest expression level in liver

CleanEx database of gene expression profiles

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CleanExi
HS_C1R

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
P00736 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
P00736 HS

Organism-specific databases

Human Protein Atlas

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HPAi
HPA001251
HPA001551

<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

C1 is a calcium-dependent trimolecular complex of C1q, C1r and C1s in the molar ration of 1:2:2. C1r is a dimer of identical chains, each of which is activated by cleavage into two chains, A and B, connected by disulfide bonds.

<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

WithEntry#Exp.IntActNotes
C1SP098714EBI-3926504,EBI-2810045

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

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BioGridi
107176, 10 interactors

Protein interaction database and analysis system

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IntActi
P00736, 3 interactors

Molecular INTeraction database

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MINTi
P00736

Chemistry databases

BindingDB database of measured binding affinities

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BindingDBi
P00736

<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

1705
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

Select the link destinations:

Protein Data Bank Europe

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PDBei

Protein Data Bank RCSB

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RCSB PDBi

Protein Data Bank Japan

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PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1APQNMR-A140-192[»]
1GPZX-ray2.90A/B307-705[»]
1MD7X-ray3.20A375-702[»]
1MD8X-ray2.80A375-703[»]
2QY0X-ray2.60A/C309-463[»]
B/D464-705[»]
6F1CX-ray4.20A/C18-308[»]
6F1DX-ray1.95A191-307[»]
6F1HX-ray4.50A/C18-308[»]
6F39X-ray5.80A/B22-306[»]

Database of protein disorder

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DisProti
DP00621

Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

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ProteinModelPortali
P00736

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

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SMRi
P00736

Database of comparative protein structure models

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ModBasei
Search...

MobiDB: a database of protein disorder and mobility annotations

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MobiDBi
Search...

Miscellaneous databases

Relative evolutionary importance of amino acids within a protein sequence

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EvolutionaryTracei
P00736

<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_and_domains_section">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 – 141CUB 1PROSITE-ProRule annotationAdd BLAST124
Domaini142 – 190EGF-like; calcium-bindingSequence analysisAdd BLAST49
Domaini193 – 305CUB 2PROSITE-ProRule annotationAdd BLAST113
Domaini307 – 373Sushi 1PROSITE-ProRule annotationAdd BLAST67
Domaini374 – 449Sushi 2PROSITE-ProRule annotationAdd BLAST76
Domaini464 – 702Peptidase S1PROSITE-ProRule annotationAdd BLAST239

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

Belongs to the peptidase S1 family.PROSITE-ProRule annotation

Keywords - Domaini

EGF-like domain, Repeat, Signal, Sushi

Phylogenomic databases

The HOVERGEN Database of Homologous Vertebrate Genes

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HOVERGENi
HBG000559

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
P00736

KEGG Orthology (KO)

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KOi
K01330

Database of Orthologous Groups

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OrthoDBi
558558at2759

Database for complete collections of gene phylogenies

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PhylomeDBi
P00736

TreeFam database of animal gene trees

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TreeFami
TF330373

Family and domain databases

Conserved Domains Database

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CDDi
cd00033 CCP, 2 hits
cd00041 CUB, 2 hits
cd00190 Tryp_SPc, 1 hit

Gene3D Structural and Functional Annotation of Protein Families

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Gene3Di
2.60.120.290, 2 hits

Integrated resource of protein families, domains and functional sites

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InterProi
View protein in InterPro
IPR035707 Complement_C1r
IPR000859 CUB_dom
IPR001881 EGF-like_Ca-bd_dom
IPR013032 EGF-like_CS
IPR000742 EGF-like_dom
IPR000152 EGF-type_Asp/Asn_hydroxyl_site
IPR018097 EGF_Ca-bd_CS
IPR009003 Peptidase_S1_PA
IPR001314 Peptidase_S1A
IPR035914 Sperma_CUB_dom_sf
IPR035976 Sushi/SCR/CCP_sf
IPR000436 Sushi_SCR_CCP_dom
IPR001254 Trypsin_dom
IPR033116 TRYPSIN_SER

The PANTHER Classification System

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PANTHERi
PTHR45206 PTHR45206, 1 hit

Pfam protein domain database

More...
Pfami
View protein in Pfam
PF00431 CUB, 2 hits
PF00084 Sushi, 2 hits
PF00089 Trypsin, 1 hit

Protein Motif fingerprint database; a protein domain database

More...
PRINTSi
PR00722 CHYMOTRYPSIN

Simple Modular Architecture Research Tool; a protein domain database

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SMARTi
View protein in SMART
SM00032 CCP, 2 hits
SM00042 CUB, 2 hits
SM00181 EGF, 1 hit
SM00179 EGF_CA, 1 hit
SM00020 Tryp_SPc, 1 hit

Superfamily database of structural and functional annotation

More...
SUPFAMi
SSF49854 SSF49854, 2 hits
SSF50494 SSF50494, 1 hit
SSF57535 SSF57535, 2 hits

PROSITE; a protein domain and family database

More...
PROSITEi
View protein in PROSITE
PS00010 ASX_HYDROXYL, 1 hit
PS01180 CUB, 2 hits
PS01186 EGF_2, 1 hit
PS01187 EGF_CA, 1 hit
PS50923 SUSHI, 2 hits
PS50240 TRYPSIN_DOM, 1 hit
PS00135 TRYPSIN_SER, 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>.<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_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 has 1 described isoform and 10 potential isoforms that are computationally mapped.Show allAlign All

P00736-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MWLLYLLVPA LFCRAGGSIP IPQKLFGEVT SPLFPKPYPN NFETTTVITV
60 70 80 90 100
PTGYRVKLVF QQFDLEPSEG CFYDYVKISA DKKSLGRFCG QLGSPLGNPP
110 120 130 140 150
GKKEFMSQGN KMLLTFHTDF SNEENGTIMF YKGFLAYYQA VDLDECASRS
160 170 180 190 200
KSGEEDPQPQ CQHLCHNYVG GYFCSCRPGY ELQEDTHSCQ AECSSELYTE
210 220 230 240 250
ASGYISSLEY PRSYPPDLRC NYSIRVERGL TLHLKFLEPF DIDDHQQVHC
260 270 280 290 300
PYDQLQIYAN GKNIGEFCGK QRPPDLDTSS NAVDLLFFTD ESGDSRGWKL
310 320 330 340 350
RYTTEIIKCP QPKTLDEFTI IQNLQPQYQF RDYFIATCKQ GYQLIEGNQV
360 370 380 390 400
LHSFTAVCQD DGTWHRAMPR CKIKDCGQPR NLPNGDFRYT TTMGVNTYKA
410 420 430 440 450
RIQYYCHEPY YKMQTRAGSR ESEQGVYTCT AQGIWKNEQK GEKIPRCLPV
460 470 480 490 500
CGKPVNPVEQ RQRIIGGQKA KMGNFPWQVF TNIHGRGGGA LLGDRWILTA
510 520 530 540 550
AHTLYPKEHE AQSNASLDVF LGHTNVEELM KLGNHPIRRV SVHPDYRQDE
560 570 580 590 600
SYNFEGDIAL LELENSVTLG PNLLPICLPD NDTFYDLGLM GYVSGFGVME
610 620 630 640 650
EKIAHDLRFV RLPVANPQAC ENWLRGKNRM DVFSQNMFCA GHPSLKQDAC
660 670 680 690 700
QGDSGGVFAV RDPNTDRWVA TGIVSWGIGC SRGYGFYTKV LNYVDWIKKE

MEEED
Length:705
Mass (Da):80,119
Last modified:December 16, 2008 - 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:iB45D120201061462
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 10 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
B4DPQ0B4DPQ0_HUMAN
Complement C1r subcomponent
C1R
719Annotation 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>
F5H2D0F5H2D0_HUMAN
Complement C1r subcomponent
C1R
671Annotation 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>
F5H3N3F5H3N3_HUMAN
Complement C1r subcomponent
C1R
156Annotation 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>
A0A3B3ISR2A0A3B3ISR2_HUMAN
Complement C1r subcomponent
C1R
705Annotation 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>
F5H1V0F5H1V0_HUMAN
Complement C1r subcomponent
C1R
144Annotation 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>
F5H6Y3F5H6Y3_HUMAN
Complement C1r subcomponent
C1R
156Annotation 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>
F5H3A3F5H3A3_HUMAN
Complement C1r subcomponent
C1R
93Annotation 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>
F5GWL0F5GWL0_HUMAN
Complement C1r subcomponent
C1R
71Annotation 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>
A0A3B3ITU4A0A3B3ITU4_HUMAN
Complement C1r subcomponent
C1R
351Annotation 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>
F5H1N6F5H1N6_HUMAN
Complement C1r subcomponent
C1R
49Annotation 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 provides information on polymorphic variants. If the variant is associated with a disease state, the description of the latter can be found in the <a href="http://www.uniprot.org/manual/involvement_in_disease">'Involvement in disease'</a> subsection.<p><a href='/help/polymorphism' target='_top'>More...</a></p>Polymorphismi

Complement component C1r deficiency [MIMi:216950] leads to the failure of the classical complement system activation pathway (C1 deficiency). Individuals with C1 deficiency are highly susceptible to infections by microorganisms and have greater risk in developing autoimmune diseases such as systemic lupus erythematosus (SLE).1 Publication

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07710650V → D in EDSPD1; requires 2 nucleotide substitutions; the mutant is not secreted but retained intracellularly. 1 Publication1
Natural variantiVAR_018667131Y → H1 Publication1
Natural variantiVAR_016103152S → L Common polymorphism. 4 PublicationsCorresponds to variant dbSNP:rs1801046Ensembl.1
Natural variantiVAR_018668163H → Y1 Publication1
Natural variantiVAR_018669184E → K Polymorphism; confirmed at protein level. 2 PublicationsCorresponds to variant dbSNP:rs1126605Ensembl.1
Natural variantiVAR_047933186T → R4 PublicationsCorresponds to variant dbSNP:rs4519167Ensembl.1
Natural variantiVAR_018670261G → R Polymorphism; confirmed at protein level. 2 PublicationsCorresponds to variant dbSNP:rs3813728Ensembl.1
Natural variantiVAR_077107290D → G in EDSPD1; unknown pathological significance. 1 Publication1
Natural variantiVAR_077108297G → D in EDSPD1; unknown pathological significance. 1 Publication1
Natural variantiVAR_077109300L → P in EDSPD1; unknown pathological significance. 1 Publication1
Natural variantiVAR_077110301R → P in EDSPD1; unknown pathological significance. 1 Publication1
Natural variantiVAR_077111302Y → C in EDSPD1. 1 Publication1
Natural variantiVAR_077112306 – 309IIKC → RR in EDSPD1. 1 Publication4
Natural variantiVAR_077113309C → W in EDSPD1; the mutant is not secreted but retained intracellularly. 1 Publication1
Natural variantiVAR_077114338C → R in EDSPD1. 1 Publication1
Natural variantiVAR_077115358C → F in EDSPD1. 1 Publication1
Natural variantiVAR_077116364W → C in EDSPD1; unknown pathological significance. 1 Publication1
Natural variantiVAR_077117371C → W in EDSPD1; the mutant is not secreted but retained intracellularly. 1 Publication1
Natural variantiVAR_077118401 – 405RIQYY → HVI in EDSPD1; unknown pathological significance. 1 Publication5
Natural variantiVAR_077119435W → R in EDSPD1; unknown pathological significance. 1 Publication1

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

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EMBLi

GenBank nucleotide sequence database

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GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

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DDBJi
Links Updated
M14058 mRNA Translation: AAA51851.1
X04701 mRNA Translation: CAA28407.1
AB083037 Genomic DNA Translation: BAC19850.2
AC094008 Genomic DNA No translation available.
AC140077 Genomic DNA No translation available.
CR749540 mRNA Translation: CAH18343.1
BC035220 mRNA Translation: AAH35220.1

The Consensus CDS (CCDS) project

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CCDSi
CCDS81658.1

Protein sequence database of the Protein Information Resource

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PIRi
A24170 C1HURB

NCBI Reference Sequences

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RefSeqi
NP_001724.3, NM_001733.4

UniGene gene-oriented nucleotide sequence clusters

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UniGenei
Hs.524224
Hs.731679

Genome annotation databases

Ensembl eukaryotic genome annotation project

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Ensembli
ENST00000542285; ENSP00000438615; ENSG00000159403

Database of genes from NCBI RefSeq genomes

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GeneIDi
715

KEGG: Kyoto Encyclopedia of Genes and Genomes

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KEGGi
hsa:715

UCSC genome browser

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UCSCi
uc031ysf.2 human

Keywords - Coding sequence diversityi

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
M14058 mRNA Translation: AAA51851.1
X04701 mRNA Translation: CAA28407.1
AB083037 Genomic DNA Translation: BAC19850.2
AC094008 Genomic DNA No translation available.
AC140077 Genomic DNA No translation available.
CR749540 mRNA Translation: CAH18343.1
BC035220 mRNA Translation: AAH35220.1
CCDSiCCDS81658.1
PIRiA24170 C1HURB
RefSeqiNP_001724.3, NM_001733.4
UniGeneiHs.524224
Hs.731679

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
1APQNMR-A140-192[»]
1GPZX-ray2.90A/B307-705[»]
1MD7X-ray3.20A375-702[»]
1MD8X-ray2.80A375-703[»]
2QY0X-ray2.60A/C309-463[»]
B/D464-705[»]
6F1CX-ray4.20A/C18-308[»]
6F1DX-ray1.95A191-307[»]
6F1HX-ray4.50A/C18-308[»]
6F39X-ray5.80A/B22-306[»]
DisProtiDP00621
ProteinModelPortaliP00736
SMRiP00736
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi107176, 10 interactors
IntActiP00736, 3 interactors
MINTiP00736

Chemistry databases

BindingDBiP00736
ChEMBLiCHEMBL4611
DrugBankiDB00054 Abciximab
DB00051 Adalimumab
DB00092 Alefacept
DB00087 Alemtuzumab
DB00074 Basiliximab
DB00112 Bevacizumab
DB06404 C1 Esterase Inhibitor (Human)
DB09228 C1 Esterase Inhibitor (Recombinant)
DB00002 Cetuximab
DB00111 Daclizumab
DB00095 Efalizumab
DB00005 Etanercept
DB00056 Gemtuzumab ozogamicin
DB00078 Ibritumomab tiuxetan
DB00075 Muromonab
DB00108 Natalizumab
DB00110 Palivizumab
DB00073 Rituximab
DB00081 Tositumomab
DB00072 Trastuzumab
GuidetoPHARMACOLOGYi2334

Protein family/group databases

MEROPSiS01.192

PTM databases

GlyConnecti713
iPTMnetiP00736
PhosphoSitePlusiP00736

Polymorphism and mutation databases

BioMutaiC1R
DMDMi218511956

Proteomic databases

EPDiP00736
jPOSTiP00736
PeptideAtlasiP00736
PRIDEiP00736
ProteomicsDBi51270

Protocols and materials databases

The DNASU plasmid repository

More...
DNASUi
715
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000542285; ENSP00000438615; ENSG00000159403
GeneIDi715
KEGGihsa:715
UCSCiuc031ysf.2 human

Organism-specific databases

Comparative Toxicogenomics Database

More...
CTDi
715
DisGeNETi715
EuPathDBiHostDB:ENSG00000159403.15

GeneCards: human genes, protein and diseases

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GeneCardsi
C1R
HGNCiHGNC:1246 C1R
HPAiHPA001251
HPA001551
MalaCardsiC1R
MIMi130080 phenotype
216950 phenotype
613785 gene
neXtProtiNX_P00736
Orphaneti75392 Ehlers-Danlos syndrome, periodontitis type
169147 Immunodeficiency due to a classical component pathway complement deficiency
93552 Pediatric systemic lupus erythematosus
PharmGKBiPA25635

GenAtlas: human gene database

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GenAtlasi
Search...

Phylogenomic databases

HOVERGENiHBG000559
InParanoidiP00736
KOiK01330
OrthoDBi558558at2759
PhylomeDBiP00736
TreeFamiTF330373

Enzyme and pathway databases

BRENDAi3.4.21.41 2681
ReactomeiR-HSA-166663 Initial triggering of complement
R-HSA-173623 Classical antibody-mediated complement activation
R-HSA-977606 Regulation of Complement cascade
SABIO-RKiP00736
SIGNORiP00736

Miscellaneous databases

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

More...
ChiTaRSi
C1R human
EvolutionaryTraceiP00736

The Gene Wiki collection of pages on human genes and proteins

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GeneWikii
C1R_(gene)

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

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GenomeRNAii
715

Protein Ontology

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PROi
PR:P00736

The Stanford Online Universal Resource for Clones and ESTs

More...
SOURCEi
Search...

Gene expression databases

BgeeiENSG00000159403 Expressed in 221 organ(s), highest expression level in liver
CleanExiHS_C1R
ExpressionAtlasiP00736 baseline and differential
GenevisibleiP00736 HS

Family and domain databases

CDDicd00033 CCP, 2 hits
cd00041 CUB, 2 hits
cd00190 Tryp_SPc, 1 hit
Gene3Di2.60.120.290, 2 hits
InterProiView protein in InterPro
IPR035707 Complement_C1r
IPR000859 CUB_dom
IPR001881 EGF-like_Ca-bd_dom
IPR013032 EGF-like_CS
IPR000742 EGF-like_dom
IPR000152 EGF-type_Asp/Asn_hydroxyl_site
IPR018097 EGF_Ca-bd_CS
IPR009003 Peptidase_S1_PA
IPR001314 Peptidase_S1A
IPR035914 Sperma_CUB_dom_sf
IPR035976 Sushi/SCR/CCP_sf
IPR000436 Sushi_SCR_CCP_dom
IPR001254 Trypsin_dom
IPR033116 TRYPSIN_SER
PANTHERiPTHR45206 PTHR45206, 1 hit
PfamiView protein in Pfam
PF00431 CUB, 2 hits
PF00084 Sushi, 2 hits
PF00089 Trypsin, 1 hit
PRINTSiPR00722 CHYMOTRYPSIN
SMARTiView protein in SMART
SM00032 CCP, 2 hits
SM00042 CUB, 2 hits
SM00181 EGF, 1 hit
SM00179 EGF_CA, 1 hit
SM00020 Tryp_SPc, 1 hit
SUPFAMiSSF49854 SSF49854, 2 hits
SSF50494 SSF50494, 1 hit
SSF57535 SSF57535, 2 hits
PROSITEiView protein in PROSITE
PS00010 ASX_HYDROXYL, 1 hit
PS01180 CUB, 2 hits
PS01186 EGF_2, 1 hit
PS01187 EGF_CA, 1 hit
PS50923 SUSHI, 2 hits
PS50240 TRYPSIN_DOM, 1 hit
PS00135 TRYPSIN_SER, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

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ProtoNeti
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 nameiC1R_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: P00736
Secondary accession number(s): A6NJQ8, Q68D77, Q8J012
<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: July 21, 1986
Last sequence update: December 16, 2008
Last modified: January 16, 2019
This is version 222 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

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Peptidase families
    Classification of peptidase families and list of entries
  2. SIMILARITY comments
    Index of protein domains and families
  3. Human chromosome 12
    Human chromosome 12: entries, gene names and cross-references to MIM
  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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
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