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Aliases for CPN1 Gene

Aliases for CPN1 Gene

  • Carboxypeptidase N Subunit 1 2 3 5
  • Anaphylatoxin Inactivator 2 3 4
  • Arginine Carboxypeptidase 2 3 4
  • Lysine Carboxypeptidase 2 3 4
  • Carboxypeptidase N, Polypeptide 1 2 3
  • Carboxypeptidase N Small Subunit 3 4
  • Plasma Carboxypeptidase B 3 4
  • Serum Carboxypeptidase N 3 4
  • Carboxypeptidase K 2 3
  • EC 4 63
  • Kininase I 2 3
  • Kininase-1 3 4
  • SCPN 3 4
  • CPN 3 4
  • Carboxypeptidase N, Polypeptide 1, 50kD 2
  • Carboxypeptidase N Polypeptide 1 50 KD 3
  • Carboxypeptidase N Catalytic Subunit 3
  • Carboxypeptidase N Polypeptide 1 4
  • EC 3.4.17 63
  • ACBP 4

External Ids for CPN1 Gene

Previous GeneCards Identifiers for CPN1 Gene

  • GC10M100695
  • GC10M101035
  • GC10M101933
  • GC10M101466
  • GC10M101792
  • GC10M095429

Summaries for CPN1 Gene

Entrez Gene Summary for CPN1 Gene

  • Carboxypeptidase N is a plasma metallo-protease that cleaves basic amino acids from the C terminal of peptides and proteins. The enzyme is important in the regulation of peptides like kinins and anaphylatoxins, and has also been known as kininase-1 and anaphylatoxin inactivator. This enzyme is a tetramer comprised of two identical regulatory subunits and two identical catalytic subunits; this gene encodes the catalytic subunit. Mutations in this gene can be associated with angioedema or chronic urticaria resulting from carboxypeptidase N deficiency. [provided by RefSeq, Jul 2008]

GeneCards Summary for CPN1 Gene

CPN1 (Carboxypeptidase N Subunit 1) is a Protein Coding gene. Diseases associated with CPN1 include Carboxypeptidase N Deficiency and Pyelitis. Among its related pathways are Prostate Cancer and Peptide hormone metabolism. GO annotations related to this gene include metallocarboxypeptidase activity and carboxypeptidase activity. An important paralog of this gene is CPXM2.

UniProtKB/Swiss-Prot for CPN1 Gene

  • Protects the body from potent vasoactive and inflammatory peptides containing C-terminal Arg or Lys (such as kinins or anaphylatoxins) which are released into the circulation.

Gene Wiki entry for CPN1 Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for CPN1 Gene

Genomics for CPN1 Gene

Regulatory Elements for CPN1 Gene

Enhancers for CPN1 Gene
GeneHancer Identifier Score Enhancer Sources TSS distance (kb) Number of Genes Away Size (kb) Transcription Factor Binding Sites within enhancer Other Gene Targets for Enhancer

Enhancers around CPN1 on UCSC Golden Path with GeneCards custom track

Promoters for CPN1 Gene
Ensembl Regulatory Elements (ENSRs) TSS Distance (bp) Size (bp) Binding Sites for Transcription Factors within promoters

ENSRs around CPN1 on UCSC Golden Path with GeneCards custom track

Genomic Location for CPN1 Gene

100,042,193 bp from pter
100,081,901 bp from pter
39,709 bases
Minus strand

Genomic View for CPN1 Gene

Genes around CPN1 on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
CPN1 Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
Genomic Location for CPN1 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for CPN1 Gene

Proteins for CPN1 Gene

  • Protein details for CPN1 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Carboxypeptidase N catalytic chain
    Protein Accession:
    Secondary Accessions:
    • B1AP59

    Protein attributes for CPN1 Gene

    458 amino acids
    Molecular mass:
    52286 Da
    Name=Zn(2+); Xref=ChEBI:CHEBI:29105;
    Quaternary structure:
    • Tetramer of two catalytic chains and two glycosylated inactive chains.

    Three dimensional structures from OCA and Proteopedia for CPN1 Gene

neXtProt entry for CPN1 Gene

Proteomics data for CPN1 Gene at MOPED

Post-translational modifications for CPN1 Gene

  • Glycosylation at Thr 400, Thr 402, and Thr 409
  • Modification sites at PhosphoSitePlus

Other Protein References for CPN1 Gene

ENSEMBL proteins:
REFSEQ proteins:

Antibody Products

  • Abcam antibodies for CPN1

Domains & Families for CPN1 Gene

Gene Families for CPN1 Gene

Protein Domains for CPN1 Gene

Suggested Antigen Peptide Sequences for CPN1 Gene

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the peptidase M14 family.
  • Belongs to the peptidase M14 family.
genes like me logo Genes that share domains with CPN1: view

Function for CPN1 Gene

Molecular function for CPN1 Gene

GENATLAS Biochemistry:
carboxypeptidase N catalytic subunit,50kDa
UniProtKB/Swiss-Prot CatalyticActivity:
Release of a C-terminal basic amino acid, preferentially lysine.
UniProtKB/Swiss-Prot Function:
Protects the body from potent vasoactive and inflammatory peptides containing C-terminal Arg or Lys (such as kinins or anaphylatoxins) which are released into the circulation.

Enzyme Numbers (IUBMB) for CPN1 Gene

genes like me logo Genes that share phenotypes with CPN1: view

Human Phenotype Ontology for CPN1 Gene

HPO Id HPO Name Alternative Ids Definition Synonyms

Animal Models for CPN1 Gene

MGI Knock Outs for CPN1:

Animal Model Products

No data available for Gene Ontology (GO) - Molecular Function , miRNA , Transcription Factor Targets and HOMER Transcription for CPN1 Gene

Localization for CPN1 Gene

Subcellular locations from UniProtKB/Swiss-Prot for CPN1 Gene

Secreted, extracellular space.

Subcellular locations from

Jensen Localization Image for CPN1 Gene COMPARTMENTS Subcellular localization image for CPN1 gene
Compartment Confidence
extracellular 5
endoplasmic reticulum 2
nucleus 2
plasma membrane 2
chloroplast 1
cytoskeleton 1
cytosol 1
lysosome 1
vacuole 1

No data available for Gene Ontology (GO) - Cellular Components for CPN1 Gene

Pathways & Interactions for CPN1 Gene

genes like me logo Genes that share pathways with CPN1: view

Pathways by source for CPN1 Gene

2 BioSystems pathways for CPN1 Gene

Gene Ontology (GO) - Biological Process for CPN1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0016485 protein processing IBA --
genes like me logo Genes that share ontologies with CPN1: view

No data available for SIGNOR curated interactions for CPN1 Gene

Drugs & Compounds for CPN1 Gene

(3) Drugs for CPN1 Gene - From: HMDB and Novoseek

Name Status Disease Links Group Role Mechanism of Action Clinical Trials
Zinc Approved Pharma 2102

(1) Additional Compounds for CPN1 Gene - From: Novoseek

Name Synonyms Role CAS Number PubChem IDs PubMed IDs
genes like me logo Genes that share compounds with CPN1: view

Transcripts for CPN1 Gene

mRNA/cDNA for CPN1 Gene

Unigene Clusters for CPN1 Gene

Carboxypeptidase N, polypeptide 1:
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for CPN1 Gene

No ASD Table

Relevant External Links for CPN1 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for CPN1 Gene

mRNA expression in normal human tissues for CPN1 Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

mRNA differential expression in normal tissues according to GTEx for CPN1 Gene

This gene is overexpressed in Liver (x52.2).

Protein differential expression in normal tissues from HIPED for CPN1 Gene

This gene is overexpressed in Serum (22.2), Plasma (20.2), and Synovial fluid (20.1).

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB, PaxDb, and MOPED for CPN1 Gene

SOURCE GeneReport for Unigene cluster for CPN1 Gene Hs.2246

mRNA Expression by UniProt/SwissProt for CPN1 Gene

Tissue specificity: Synthesized in the liver and secreted in plasma.
genes like me logo Genes that share expression patterns with CPN1: view

Protein tissue co-expression partners for CPN1 Gene

Primer Products

Orthologs for CPN1 Gene

This gene was present in the common ancestor of animals.

Orthologs for CPN1 Gene

Organism Taxonomy Gene Similarity Type Details
(Bos Taurus)
Mammalia CPN1 35
  • 88.11 (n)
  • 87.09 (a)
CPN1 36
  • 86 (a)
(Canis familiaris)
Mammalia CPN1 35
  • 88.06 (n)
  • 86.94 (a)
CPN1 36
  • 85 (a)
(Mus musculus)
Mammalia Cpn1 35
  • 83.98 (n)
  • 84.28 (a)
Cpn1 16
Cpn1 36
  • 84 (a)
(Rattus norvegicus)
Mammalia Cpn1 35
  • 83.98 (n)
  • 83.6 (a)
(Monodelphis domestica)
Mammalia CPN1 36
  • 74 (a)
(Ornithorhynchus anatinus)
Mammalia -- 36
  • 71 (a)
-- 36
  • 67 (a)
-- 36
  • 42 (a)
(Gallus gallus)
Aves CPN1 35
  • 72.04 (n)
  • 73.36 (a)
CPN1 36
  • 71 (a)
(Anolis carolinensis)
Reptilia CPN1 36
  • 66 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia cpn1 35
  • 66.9 (n)
  • 71.19 (a)
African clawed frog
(Xenopus laevis)
Amphibia MGC68490 35
(Danio rerio)
Actinopterygii cpn1 35
  • 66.11 (n)
  • 71.63 (a)
cpn1 36
  • 51 (a)
cpn1 36
  • 66 (a)
rainbow trout
(Oncorhynchus mykiss)
Actinopterygii Omy.9650 35
fruit fly
(Drosophila melanogaster)
Insecta CG4678 37
  • 41 (a)
(Caenorhabditis elegans)
Secernentea F01D4.4 37
  • 45 (a)
F59A3.1 37
  • 44 (a)
T27A8.1 37
  • 39 (a)
Species with no ortholog for CPN1:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • African malaria mosquito (Anopheles gambiae)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • baker's yeast (Saccharomyces cerevisiae)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • bread mold (Neurospora crassa)
  • chimpanzee (Pan troglodytes)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • corn (Zea mays)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • fission yeast (Schizosaccharomyces pombe)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • K. lactis yeast (Kluyveromyces lactis)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rice (Oryza sativa)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea squirt (Ciona intestinalis)
  • sea squirt (Ciona savignyi)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • thale cress (Arabidopsis thaliana)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)

Evolution for CPN1 Gene

Gene Tree for CPN1 (if available)
Gene Tree for CPN1 (if available)

Paralogs for CPN1 Gene

Paralogs for CPN1 Gene

(7) SIMAP similar genes for CPN1 Gene using alignment to 2 proteins:

genes like me logo Genes that share paralogs with CPN1: view

Variants for CPN1 Gene

Sequence variations from dbSNP and Humsavar for CPN1 Gene

SNP ID Clin Chr 10 pos Sequence Context AA Info Type
rs61751507 Carboxypeptidase N deficiency (CPND) 100,069,757(+) TGGGG(C/T)CTCCG reference, missense
rs3750716 -- 100,057,106(+) TCCAG(C/T)GTGAT reference, synonymous-codon
rs3750717 -- 100,065,197(+) TGGAA(A/C/G)AGCTT reference, synonymous-codon
rs3750718 -- 100,075,751(+) TCATC(A/C)TTTCT intron-variant
rs3829161 -- 100,065,403(+) AAGGG(C/G)CAACT intron-variant, reference, missense

Structural Variations from Database of Genomic Variants (DGV) for CPN1 Gene

Variant ID Type Subtype PubMed ID
nsv825541 CNV Gain 20364138

Variation tolerance for CPN1 Gene

Residual Variation Intolerance Score: 14.5% of all genes are more intolerant (likely to be disease-causing)
Gene Damage Index Score: 3.35; 53.71% of all genes are more intolerant (likely to be disease-causing)

Relevant External Links for CPN1 Gene

Human Gene Mutation Database (HGMD)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for CPN1 Gene

Disorders for CPN1 Gene

MalaCards: The human disease database

(9) MalaCards diseases for CPN1 Gene - From: OMIM, ClinVar, Swiss-Prot, DISEASES, and GeneCards

Disorder Aliases PubMed IDs
carboxypeptidase n deficiency
  • cpnd
  • pyelitis nos
lymphogranuloma venereum
  • climatic or tropical bubo
  • angioneurotic edema
- elite association - COSMIC cancer census association via MalaCards
Search CPN1 in MalaCards View complete list of genes associated with diseases


  • Carboxypeptidase N deficiency (CPND) [MIM:212070]: Patients affected present some combination of angioedema or chronic urticaria, as well as hay fever or asthma, and have also slightly depressed serum carboxy peptidase N, suggestive of autosomal recessive inheritance of this disorder. {ECO:0000269 PubMed:12560874}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Relevant External Links for CPN1

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
Atlas of Genetics and Cytogenetics in Oncology and Haematology:
genes like me logo Genes that share disorders with CPN1: view

No data available for Genatlas for CPN1 Gene

Publications for CPN1 Gene

  1. cDNA cloning and complete primary structure of the small, active subunit of human carboxypeptidase N (kininase 1). (PMID: 2912725) Gebhard W. … Eulitz M. (Eur. J. Biochem. 1989) 2 3 4 67
  2. Chromosomal localization of the genes for human carboxypeptidase D (CPD) and the active 50-kilodalton subunit of human carboxypeptidase N (CPN1). (PMID: 9628828) Riley D.A. … Skidgel R.A. (Genomics 1998) 2 3 23
  3. Structure and function of human plasma carboxypeptidase N, the anaphylatoxin inactivator. (PMID: 18039526) Skidgel R.A. … ErdAPs E.G. (Int. Immunopharmacol. 2007) 3 23
  4. Identification of carboxypeptidase N as an enzyme responsible for C-terminal cleavage of stromal cell-derived factor-1alpha in the circulation. (PMID: 15718415) Davis D.A. … Tosato G. (Blood 2005) 3 23
  5. Carboxypeptidase N: a pleiotropic regulator of inflammation. (PMID: 14687935) Matthews K.W. … Wetsel R.A. (Mol. Immunol. 2004) 3 23

Products for CPN1 Gene

Sources for CPN1 Gene