Annotation Detail

Information
Associated Genes
NBN
Associated Variants
NBN p.Lys219AsnfsTer16 (p.K219Nfs*16) ( ENST00000265433.8, ENST00000409330.5, ENST00000517337.2, ENST00000523444.2, ENST00000697292.1, ENST00000697293.1, ENST00000697298.1, ENST00000697299.1, ENST00000697304.1, ENST00000697307.1, ENST00000697308.1, ENST00000697309.1, ENST00000697310.1 )
NBN p.Lys219AsnfsTer16 (p.K219Nfs*16) ( ENST00000265433.8, ENST00000409330.5, ENST00000517337.2, ENST00000523444.2, ENST00000697292.1, ENST00000697293.1, ENST00000697298.1, ENST00000697299.1, ENST00000697304.1, ENST00000697307.1, ENST00000697308.1, ENST00000697309.1, ENST00000697310.1 )
Associated Disease
Microcephaly, normal intelligence and immunodeficiency
Source Database
ClinVar
Description
NM_002485.5(NBN):c.657_661del (p.Lys219fs) AND Microcephaly, normal intelligence and immunodeficiency
ClinVar Allele ID
21979
ClinVar RefSeq Alternation Syntax
NM_002485.5:c.657_661del
ClinVar RefSeq Alternation Syntax
NM_001024688.3:c.411_415del
Clinical Significance Description
Pathogenic
Clinical Significance Last Update
2024-01-29
Clinical Significance Review Status
criteria provided, multiple submitters, no conflicts
URL
https://www.ncbi.nlm.nih.gov/clinvar/RCV000007353
ClinVar Disease
Microcephaly, normal intelligence and immunodeficiency
Observed Origin Sample
germline
Observed Origin Sample
maternal
Observed Origin Sample
unknown
Pubmed
18940477
Pubmed
12505263
Pubmed
12123493
Pubmed
9590180
Pubmed
10398434
Pubmed
16033915
Pubmed
9620777
Pubmed
11279524
Pubmed
17103455
Pubmed
12833396
Pubmed
10852373
Drugs