GenTIGSA Gene Database on Rare Genetic Disorders

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

Von Hippel-Lindau syndrome

An  Autosomal dominant  mode(s) within the Cancer disorders  category

Conflicting classifications of pathogenicity 2
Likely pathogenic 1
Pathogenic 6
Pathogenic/Likely pathogenic 2
Uncertain significance 1

Variant name Variant type GRCH38 location Germline classification Molecular consequence dbSNP_ID Submitter
NM_000551.4(VHL):c.359G>A (p.Arg120Lys) Single nucleotide variant Chr3:10146532 Likely pathogenic Missense variant|non-coding transcript variant|intron variant .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_000551.4(VHL):c.575C>T (p.Pro192Leu) Single nucleotide variant Chr3:10149898 Conflicting classifications of pathogenicity Missense variant|3 prime UTR variant rs902694906 .Laboratory of Molecular and Cytogenetics, Department of Anatomy, All India Institute of Medical Sciences (AIIMS)
NM_000551.4(VHL):c.353T>G (p.Leu118Arg) Single nucleotide variant Chr3:10146526 Pathogenic Missense variant|intron variant rs5030830 .Laboratory of Molecular and Cytogenetics, Department of Anatomy, All India Institute of Medical Sciences (AIIMS)
NM_000551.4(VHL):c.583C>T (p.Gln195Ter) Single nucleotide variant Chr3:10149906 Pathogenic Nonsense|3 prime UTR variant rs5030825 .Laboratory of Molecular and Cytogenetics, Department of Anatomy, All India Institute of Medical Sciences (AIIMS)
NM_000551.4(VHL):c.548C>G (p.Ser183Trp) Single nucleotide variant Chr3:10149871 Uncertain significance Missense variant|3 prime UTR variant rs5030823
NM_000551.4(VHL):c.482G>A (p.Arg161Gln) Single nucleotide variant Chr3:10149805 Pathogenic Missense variant|3 prime UTR variant rs730882035 .Laboratory of Molecular and Cytogenetics, Department of Anatomy, All India Institute of Medical Sciences (AIIMS)
NM_000551.4(VHL):c.499C>T (p.Arg167Trp) Single nucleotide variant Chr3:10149822 Pathogenic/Likely pathogenic Missense variant|3 prime UTR variant rs5030820 .Laboratory of Molecular and Cytogenetics, Department of Anatomy, All India Institute of Medical Sciences (AIIMS)
NM_000551.4(VHL):c.500G>A (p.Arg167Gln) Single nucleotide variant Chr3:10149823 Pathogenic Missense variant|3 prime UTR variant rs5030821 .Laboratory of Molecular and Cytogenetics, Department of Anatomy, All India Institute of Medical Sciences (AIIMS)
.Neuberg Centre For Genomic Medicine, NCGM
NM_003000.3(SDHB):c.268C>T (p.Arg90Ter) Single nucleotide variant Chr1:17033078 Pathogenic Nonsense rs74315366 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
.Laboratory of Molecular and Cytogenetics, Department of Anatomy, All India Institute of Medical Sciences (AIIMS)
NM_000551.4(VHL):c.172C>T (p.Arg58Trp) Single nucleotide variant Chr3:10142019 Conflicting classifications of pathogenicity Missense variant rs757781272 .Laboratory of Molecular and Cytogenetics, Department of Anatomy, All India Institute of Medical Sciences (AIIMS)
NM_000551.4(VHL):c.194C>A (p.Ser65Ter) Single nucleotide variant Chr3:10142041 Pathogenic Nonsense rs5030826 .Laboratory of Molecular and Cytogenetics, Department of Anatomy, All India Institute of Medical Sciences (AIIMS)
NM_000551.4(VHL):c.293A>C (p.Tyr98Ser) Single nucleotide variant Chr3:10142140 Pathogenic/Likely pathogenic Missense variant rs864321643

Indian Institutes & Variant Submission Overview
Chromosome-wise Variant Distribution