ClinVar Miner

Submissions for variant NM_000202.8(IDS):c.1144G>C (p.Asp382His)

gnomAD frequency: 0.00008  dbSNP: rs370125505
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Total submissions: 5
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Submitter RCV SCV Clinical significance Condition Last evaluated Review status Method Comment
Ambry Genetics RCV002458216 SCV002613074 uncertain significance Inborn genetic diseases 2015-03-26 criteria provided, single submitter clinical testing The p.D382H variant (also known as c.1144G>C), located in coding exon 8 of the IDS gene, results from a G to C substitution at nucleotide position 1144. The aspartic acid at codon 382 is replaced by histidine, an amino acid with similar properties. This variant has been detected in conjunction with a pathogenic mutation in GRIA3 gene by our laboratory. This variant was previously reported in the SNPDatabase as rs370125505. Based on data from the NHLBI Exome Sequencing Project (ESP), the C allele was absent out of 2443 total male alleles studied. This amino acid position is well conserved in available vertebrate species. In addition, this alteration is predicted to be deleterious by in silico analysis. Since supporting evidence is limited at this time, the clinical significance of this variant remains unclear.
Genome-Nilou Lab RCV000688992 SCV002027033 uncertain significance Mucopolysaccharidosis, MPS-II 2021-09-05 criteria provided, single submitter clinical testing
Daryl Scott Lab, Baylor College of Medicine RCV000688992 SCV001448625 uncertain significance Mucopolysaccharidosis, MPS-II 2020-11-11 criteria provided, single submitter clinical testing
Labcorp Genetics (formerly Invitae), Labcorp RCV000688992 SCV000816625 likely benign Mucopolysaccharidosis, MPS-II 2026-01-31 criteria provided, single submitter clinical testing
Natera, Inc. RCV001830497 SCV002084464 uncertain significance Mucopolysaccharidosis, MPS-III-A 2019-10-28 no assertion criteria provided clinical testing

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