GenTIGSA Gene Database on Rare Genetic Disorders

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

Congenital contractural arachnodactyly

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

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

Variant name Variant type GRCH38 location Germline classification Molecular consequence dbSNP_ID Submitter
NM_000138.5(FBN1):c.6004C>T (p.Pro2002Ser) Single nucleotide variant Chr15:48444574 Likely pathogenic Missense variant rs1057519320 .Diagnostics Division, CENTRE FOR DNA FINGERPRINTING AND DIAGNOSTICS
NM_001999.4(FBN2):c.3425G>A (p.Cys1142Tyr) Single nucleotide variant Chr5:128338980 Likely pathogenic Missense variant rs137852828 .Division of Medical Genetics, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi
NM_001999.4(FBN2):c.3466T>C (p.Cys1156Arg) Single nucleotide variant Chr5:128338939 Pathogenic/Likely pathogenic Missense variant rs1750917259 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_001999.4(FBN2):c.2945G>T (p.Cys982Phe) Single nucleotide variant Chr5:128349391 Conflicting classifications of pathogenicity Missense variant rs1057519321 .Diagnostics Division, CENTRE FOR DNA FINGERPRINTING AND DIAGNOSTICS

Indian Institutes & Variant Submission Overview
Chromosome-wise Variant Distribution