GenTIGSA Gene Database on Rare Genetic Disorders

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

Bardet-Biedl syndrome

An  Autosomal recessive  mode(s) within the Eye disorders  category

Conflicting classifications of pathogenicity 4
Likely pathogenic 5
Pathogenic 3
Pathogenic/Likely pathogenic 8
Uncertain significance 3

Variant name Variant type GRCH38 location Germline classification Molecular consequence dbSNP_ID Submitter
NM_024649.5(BBS1):c.442G>A (p.Asp148Asn) Single nucleotide variant Chr11:66515549 Conflicting classifications of pathogenicity Missense variant rs200688985 .Lifecell International Pvt. Ltd
NM_024649.5(BBS1):c.48-2A>C Single nucleotide variant Chr11:66511011 Pathogenic/Likely pathogenic Splice acceptor variant rs764245266 .Department of Medical Genetics, Sanjay Gandhi Post Graduate Institute of Medical Sciences
.Neuberg Centre For Genomic Medicine, NCGM
NM_024649.5(BBS1):c.981del (p.Ala328fs) Deletion Chr11:66523751 Pathogenic/Likely pathogenic Frameshift variant|intron variant rs1057516371 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_024685.4(BBS10):c.180dup (p.Glu61fs) Duplication Chr12:76348178 - 76348179 Pathogenic Frameshift variant rs2540957709 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_024685.4(BBS10):c.569T>C (p.Ile190Thr) Single nucleotide variant Chr12:76347416 Likely pathogenic Missense variant rs2540956687 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_024685.4(BBS10):c.1337_1338del (p.Phe446fs) Deletion Chr12:76346647 - 76346648 Pathogenic/Likely pathogenic Frameshift variant rs1389599028 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_024685.4(BBS10):c.271dup (p.Cys91fs) Duplication Chr12:76347713 - 76347714 Pathogenic Frameshift variant rs549625604 .Department of Medical Genetics, Sanjay Gandhi Post Graduate Institute of Medical Sciences
.Neuberg Centre For Genomic Medicine, NCGM
.SN ONGC Dept of Genetics and Molecular biology Vision Research Foundation
NM_152618.3(BBS12):c.250G>A (p.Gly84Arg) Single nucleotide variant Chr4:122742142 Likely pathogenic Missense variant rs1578489760 .Department of Medical Genetics, Sanjay Gandhi Post Graduate Institute of Medical Sciences
NM_031885.5(BBS2):c.683T>A (p.Val228Asp) Single nucleotide variant Chr16:56506154 Uncertain significance Missense variant|non-coding transcript variant .Genetics laboratory, Department of Obstetrics & Gynae, Institute of Kidney Diseases & Research Centre Dr. H.L. Trivedi Institute Of Transplantation Sciences
NM_031885.5(BBS2):c.505T>C (p.Cys169Arg) Single nucleotide variant Chr16:56510888 Uncertain significance Missense variant|non-coding transcript variant rs2543732277 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_031885.5(BBS2):c.1780C>T (p.Arg594Ter) Single nucleotide variant Chr16:56497760 Pathogenic Nonsense|non-coding transcript variant rs762047808 .Genetics laboratory, Department of Obstetrics & Gynae, Institute of Kidney Diseases & Research Centre Dr. H.L. Trivedi Institute Of Transplantation Sciences
NM_033028.5(BBS4):c.210_213del (p.Ile70fs) Microsatellite Chr15:72712293 - 72712296 Likely pathogenic 5 prime UTR variant|frameshift variant|non-coding transcript variant rs775928735 .Department of Medical Genetics, Sanjay Gandhi Post Graduate Institute of Medical Sciences
NM_025114.4(CEP290):c.4805C>T (p.Thr1602Met) Single nucleotide variant Chr12:88083854 Conflicting classifications of pathogenicity Missense variant rs369451049 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
.Neuberg Centre For Genomic Medicine, NCGM
NM_025114.4(CEP290):c.5745dup (p.Lys1916Ter) Duplication Chr12:88071890 - 88071891 Pathogenic/Likely pathogenic Nonsense rs751361090 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_025114.4(CEP290):c.5668G>T (p.Gly1890Ter) Single nucleotide variant Chr12:88077263 Pathogenic/Likely pathogenic Nonsense rs137852832 .Institute of Medical Genetics and Genomics, Sir Ganga Ram Hospital
.Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
.Neuberg Centre For Genomic Medicine, NCGM
.Suma Genomics
NM_177965.4(CFAP418):c.130C>T (p.Gln44Ter) Single nucleotide variant Chr8:95269060 Likely pathogenic Nonsense rs751922029 .Department of Medical Genetics, Sanjay Gandhi Post Graduate Institute of Medical Sciences
NM_177965.4(CFAP418):c.240del (p.Ser81fs) Deletion Chr8:95263690 Likely pathogenic Frameshift variant rs1587357327 .Department of Medical Genetics, Sanjay Gandhi Post Graduate Institute of Medical Sciences
NM_017777.4(MKS1):c.958G>A (p.Val320Ile) Single nucleotide variant Chr17:58210980 Conflicting classifications of pathogenicity Missense variant rs386834053 .Diagnostics Division, CENTRE FOR DNA FINGERPRINTING AND DIAGNOSTICS
NM_017777.4(MKS1):c.1450_1453dup (p.Thr485fs) Duplication Chr17:58206501 - 58206502 Pathogenic/Likely pathogenic Frameshift variant|intron variant rs386834044 .Pediatrics Genetics, Post Graduate Institute of Medical Education and Research
NM_153704.6(TMEM67):c.514C>T (p.Arg172Ter) Single nucleotide variant Chr8:93765413 Pathogenic/Likely pathogenic Nonsense|non-coding transcript variant rs765468645 .Institute of Medical Genetics and Genomics, Sir Ganga Ram Hospital
NM_153704.6(TMEM67):c.2086C>T (p.Leu696Phe) Single nucleotide variant Chr8:93797456 Conflicting classifications of pathogenicity Missense variant|non-coding transcript variant rs863225238 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_144596.4(TTC8):c.265+5G>A Single nucleotide variant Chr14:88839577 Uncertain significance Intron variant rs2094770219 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_144596.4(TTC8):c.489G>A (p.Thr163=) Single nucleotide variant Chr14:88841196 Pathogenic/Likely pathogenic Synonymous variant|5 prime UTR variant|non-coding transcript variant rs119103286 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India

Indian Institutes & Variant Submission Overview
Chromosome-wise Variant Distribution