All variants in the CPN1 gene

Information The variants shown are described using the NM_001308.2 transcript reference sequence.

17 entries on 1 page. Showing entries 1 - 17.
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Effect     

Exon     

AscendingDNA change (cDNA)     

RNA change     

Protein     

Classification method     

Clinical classification     

DNA change (genomic) (hg19)     

DNA change (hg38)     

Published as     

ISCN     

DB-ID     

Variant remarks     

Reference     

ClinVar ID     

dbSNP ID     

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Segregation     

Frequency     

Re-site     

VIP     

Methylation     

Owner     
+/+? 1 c.173dup r.(?) p.(His59Thrfs*107) - pathogenic (recessive) g.101841210dup g.100081453dup 385fsInsG - CPN1_000002 - PubMed: Cao 2003 - - Germline - - - - - Johan den Dunnen
-?/. 1i c.223+6T>C r.(=) p.(=) - likely benign g.101841154A>G g.100081397A>G IVS1+6T>C - CPN1_000004 variant found in 13 controls PubMed: Cao 2003 - - Germline - 0.051 in controls - - - Johan den Dunnen
-/. 1i c.223+7T>C r.(=) p.(=) - benign g.101841153A>G g.100081396A>G CPN1(NM_001308.3):c.223+7T>C - CPN1_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. 3 c.533G>A r.(?) p.(Gly178Asp) - pathogenic (recessive) g.101829514C>T g.100069757C>T 746G>A - CPN1_000003 Controversy: introduced in ClinVar as pathogenic by OMIM and as benign by Mendelics, Sao Paulo Brazil PubMed: Cao 2003 ClinVar-SCV000006623.5 rs61751507 Germline - 3.4E-03 - - - Johan den Dunnen
+/. 3 c.533G>A r.(?) p.(Gly178Asp) - pathogenic (recessive) g.101829514C>T g.100069757C>T 746G>A - CPN1_000003 Gly178 is a highly conserved residue in different species and many carboxypeptidases in human. p.(Gly178Asp) is predicted as possibly damaging (PolyPhen) and deleterious (SIFT). CPN deficiency is manifested as urticaria and angioedema when homozygous or compound heterozygous carrier of pathogenic/likely pathogenic CPN1 variant. Early expression of CPN1, CPM and Crry in mouse embryo, before C3, suggest that complement regulation will be functional before complement activation can occur. Full expression of CPN during pregnancy makes bradykinin clearance important. Controversy: introduced in ClinVar as pathogenic by OMIM and as benign by Mendelics, Sao Paulo Brazil Journal: Cao 2003 ClinVar-SCV000027198 rs61751507 Germline - 3.4E-3 - - - Christian Drouet
+/+ 3 c.533G>A r.(?) p.(Gly178Asp) ACMG pathogenic (recessive) g.101829514C>T g.100069757C>T c.[533G>A];[734C>T] - CPN1_000003 Both variants are predicted to be deleterious (SIFT), damaging (ClinPred) and probably damaging (PolyPhen), polymorphic for p.(Gly178Asp) or disease-causing for p.(Thr245Met) according to MutationTaster (Table 3). The observations of both variations meet the ACMG criteria PS3, PS4, PM1, PM2, PP3, PP4, PP5, and BP6 specifically for p.(Gly178Asp), along with a pathogenic (recessive) characterization as evaluated by InterVar. Controversy: introduced in ClinVar as pathogenic by OMIM and as benign by Mendelics, Sao Paulo Brazil Journal: Vincent 2024 ClinVar-SCV000027198 rs61751507 Germline yes 3.4E-03 - - - Christian Drouet
+/+ 3 c.533G>A r.(?) p.(Gly178Asp) ACMG likely pathogenic (recessive) g.101829514C>T g.100069757C>T c.[533G>A];[533G>A] - CPN1_000003 c.533G>A variant has been introduced in ClinVar as pathogenic by OMIM, Baltimore MD, and as benign by Mendelics, Sao Paulo Brazil and by Laboratory for Molecular Medicine, Mass General Brigham Personalized Medicine, MA. Journal: Vincent 2024 ClinVar-SCV000538730.1 rs61751507 Germline yes 3.4-03 - - - Christian Drouet
+?/+? 4 c.582A>G r.(=) p.(Glu194=) ACMG pathogenic (recessive) g.101825122T>C g.100065365T>C c.[533G>A];[582A>G] - CPN1_000011 c.582A>G variant predicted to affect splicing by activation of an exonic cryptic acceptor site (HSF 3.0). The combination of both variants c.[533G>A];[582A>G] occurs at a frequency of 1.09E-07. The observations for c.582A>G meet the ACMG criteria PS3, PS4, PM2, PM3, PP3, and PP4, along with a characterization as pathogenic (recessive) as evaluated by InterVar. Journal: Vincent 2024 - rs190183597 Germline yes 5.8E-05 - - - Christian Drouet
+?/+? 4 c.734C>T r.(?) p.(Thr245Met) ACMG pathogenic (recessive) g.101824970G>A g.100065161G>A 986C>T - CPN1_000010 Thr245 is a conserved residue among species and with CPM. p.(Thr245Met) meets ACMG criteria PS3, PS4, PM1, PM3, PP3, PP4, PP5 Informations from MoBiDic (https://mobidetails.iurc.montp.inserm.fr/MD/vars/CPN1): Predicted as damaging (0.001 SIFT), probably damaging (1.00 Polyphen2), damaging (0.753 ClinPred) and tolerated (2.72 FATHMM) Proband also carrier of a rs3788853 (XPNPEP2 NM_003399.5:c.-2399C>A; NC_000023.10:g.128870791C>A) identified as a risk marker for HAE-FXII and ACEi angioedema Journal: Vincent 2024 - rs371070915 Germline yes 0.00002 - - - Christian Drouet
+?/+? 4 c.734C>T r.(?) p.(Thr245Met) - likely pathogenic (recessive) g.101824970G>A - - - CPN1_000010 - Journal: Vincent 2024 - rs371070915 CLASSIFICATION record yes - - - - MobiDetails
+/+ 4 c.734C>T r.(?) p.(Thr245Met) ACMG pathogenic g.101824970G>A g.100065161G>A - - CPN1_000010 The variant c.734C>T is present in the proband's symptomatic mother but absent in the proband's asymptomatic brother. Additional variants in HAE-nCINH-related genes included PLG:NM_000301:c.476C>T:p.(Pro159Leu) and KNG1:NM_001102416:c.1410T>A:p.(Gly470Gly, with allele frequencies 0.007 and 0.003, respectively, suggesting that the PLG and KNG1 variants are benign. Journal: Hida 2025 - rs371070915 Germline yes 0.00002 - - - Christian Drouet
+?/. 4i c.759+1G>A r.spl? p.? ACMG pathogenic g.101824944C>T g.100065187C>T - - CPN1_000007 - - ClinVar-VCV001031968.1 - Germline - - - - - Christian Drouet
+?/. 4i c.760-1G>A r.spl? p.? - pathogenic g.101823483C>T g.100063726C>T - - CPN1_000008 single record - ClinVar-VCV001031969.1 - Germline/De novo (untested) - - - - - Christian Drouet
+?/. 6 c.931T>C r.(?) p.(Cys311Arg) - likely pathogenic g.101816850A>G g.100057093A>G - - CPN1_000013 Compound heterozygous carrier : [NM_000301.3 c.988A>G, p.(Lys330Glu)](;)[NM_001308.2 c.931T>C; p.(Cys311Arg)]. c.931T>C variant is an unpublished variant, predicted as deleterious. Disruption of Cys271-Cys311 bridge, one of the two intra-chain disulfide bonds, in p.(Cys311Arg) variant product, disorganises the catalytic subunit, with expected alteration of enzymatic activity. Journal: Germenis 2018 - - Germline/De novo (untested) - - - - - Christian Drouet
?/. 8 c.1150_1154dup r.(?) p.(Tyr385*) ACMG pathogenic g.101808591_101808595dup g.100048834_100048838dup - - CPN1_000009 - - - - Germline/De novo (untested) - - - - - Christian Drouet
?/. 8 c.1219G>A r.(?) p.(Glu407Lys) - VUS g.101808526C>T g.100048769C>T - - CPN1_000005 nlC1-INH-HAE association. Variant c.1219G>A in combination with other alleles: BDKRB1, c.844C>T; SERPING1, c.*57C>G; PLAUR, c.2039T>C;p.(Met268Val); MASP1, c.2039T>C;p.(Val680Ala); MPO, c.1571G>A;p.(Arg524His); TLR4, c.842G>A;p.(Cys281Tyr) Journal: Loules 2020 ClinVar-VCV000827594.1 rs1589470177 Germline - - - - - Christian Drouet
-?/. 9 c.1299C>T r.(=) p.(=) - likely benign g.101802262G>A g.1000042505G>A - - CPN1_000012 Polymorphic variant - - rs61733667 Germline - - - - - Christian Drouet
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