Variant #0000763383 (NC_000010.10:g.101829514C>T, NM_001308.2:c.533G>A (CPN1))

Chromosome 10
Allele Parent #2
Affects function (as reported) Affects function
Affects function (by curator) Not classified
Classification method -
Clinical classification pathogenic (recessive)
DNA change (genomic) (Relative to hg19 / GRCh37) g.101829514C>T
DNA change (hg38) g.100069757C>T
Published as 746G>A
ISCN -
DB-ID CPN1_000003 See all 4 reported entries
Variant remarks 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
Reference Journal: Cao 2003
ClinVar ID ClinVar-SCV000027198
dbSNP ID rs61751507
Origin Germline
Segregation -
Frequency 3.4E-3
Re-site -
VIP -
Methylation -
Average frequency (gnomAD v.2.1.1) 0.04233 View details
Owner Christian Drouet
Database submission license Creative Commons Attribution-NonCommercial-ShareAlike 4.0 InternationalCreative Commons License
Created by Johan den Dunnen
Date created 2019-11-30 14:02:39 +01:00 (CET)
Date last edited 2024-02-07 23:06:18 +01:00 (CET)
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Variant on transcripts


Gene     

AscendingTranscript     

Affects function     

Exon     

DNA change (cDNA)     

RNA change     

Protein     
CPN1 NM_001308.2 +/. 3 c.533G>A r.(?) p.(Gly178Asp)


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