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 International |
| 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) |

Variant on transcripts
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