GenTIGSA Gene Database on Rare Genetic Disorders

Variants in ClinVar from 539 of 916 Rare Genetic Disorders of GenTIGS

Mucopolysaccharidosis, MPS-III-A

An  Autosomal recessive  mode(s) within the Metabolic disorders/Lysosomal storage disorders  category

Conflicting classifications of pathogenicity 1
Likely pathogenic 1
Pathogenic 1
Pathogenic/Likely pathogenic 2

Variant name Variant type GRCH38 location Germline classification Molecular consequence dbSNP_ID Submitter
NM_000199.5(SGSH):c.1A>C (p.Met1Leu) Single nucleotide variant Chr17:80220313 Pathogenic Missense variant|initiator_codon_variant|non-coding transcript variant rs1250300189 .Diagnostics Services (NGS), CSIR - Centre For Cellular And Molecular Biology
NM_000199.5(SGSH):c.544C>T (p.Arg182Cys) Single nucleotide variant Chr17:80214291 Pathogenic/Likely pathogenic Missense variant|non-coding transcript variant rs529855742 .Center for Medical Genetics, GenVams Trust
NM_000199.5(SGSH):c.1429G>A (p.Asp477Asn) Single nucleotide variant Chr17:80210532 Likely pathogenic Missense variant|3 prime UTR variant|non-coding transcript variant rs1064795109 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_000199.5(SGSH):c.97G>A (p.Gly33Arg) Single nucleotide variant Chr17:80217184 Conflicting classifications of pathogenicity Missense variant|non-coding transcript variant rs398123246 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_000199.5(SGSH):c.1339G>A (p.Glu447Lys) Single nucleotide variant Chr17:80210622 Pathogenic/Likely pathogenic Missense variant|3 prime UTR variant|non-coding transcript variant rs104894639 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
.Lifecell International Pvt. Ltd

Indian Institutes & Variant Submission Overview
Chromosome-wise Variant Distribution