GenTIGSA Gene Database on Rare Genetic Disorders

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

H syndrome

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

Benign 1
Conflicting classifications of pathogenicity 5
Likely pathogenic 6
Pathogenic 14
Pathogenic/Likely pathogenic 6
Uncertain significance 2

Variant name Variant type GRCH38 location Germline classification Molecular consequence dbSNP_ID Submitter
NM_152416.3(NDUFAF6):c.[328G>T];[611C>T] CompoundHeterozygote Chr: Pathogenic .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_001378615.1(CC2D2A):c.4555T>G (p.Trp1519Gly) Single nucleotide variant Chr4:15599587 Likely pathogenic Missense variant rs1577406415 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_002667.5(PLN):c.37AGA[1] (p.Arg14del) Microsatellite Chr6:118558957 - 118558959 Pathogenic Inframe_indel|inframe_deletion|intron variant rs397516784 .Lifecell International Pvt. Ltd
NM_001379500.1(COL18A1):c.2368C>T (p.Arg790Ter) Single nucleotide variant Chr21:45494560 Pathogenic Nonsense rs1555870809 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_001379500.1(COL18A1):c.3523_3524del (p.Leu1175fs) Deletion Chr21:45510091 - 45510092 Pathogenic Frameshift variant rs398122391
NM_000249.4(MLH1):c.454-13A>G Single nucleotide variant Chr3:37008801 Likely pathogenic Intron variant rs267607749
NM_000249.4(MLH1):c.306G>T (p.Glu102Asp) Single nucleotide variant Chr3:37001053 Uncertain significance Missense variant|5 prime UTR variant|intron variant rs63751665 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
.Neuberg Centre For Genomic Medicine, NCGM
NM_000249.4(MLH1):c.2038T>G (p.Cys680Gly) Single nucleotide variant Chr3:37048952 Conflicting classifications of pathogenicity Missense variant|intron variant rs63750809 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
.Neuberg Centre For Genomic Medicine, NCGM
NM_000249.4(MLH1):c.2038T>C (p.Cys680Arg) Single nucleotide variant Chr3:37048952 Likely pathogenic Missense variant|intron variant rs63750809
NM_000249.4(MLH1):c.156del (p.Glu53fs) Deletion Chr3:36996656 Pathogenic Frameshift variant|5 prime UTR variant|intron variant rs63750028 .Biotechnology, Institute of Science, Nirma University
.Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
.Neuberg Centre For Genomic Medicine, NCGM
NM_000251.3(MSH2):c.294T>A (p.Tyr98Ter) Single nucleotide variant Chr2:47408483 Pathogenic Nonsense rs763872353 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_000251.3(MSH2):c.274C>G (p.Leu92Val) Single nucleotide variant Chr2:47408463 Conflicting classifications of pathogenicity Missense variant rs587779154 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_000251.3(MSH2):c.1226_1227del (p.Gln409fs) Deletion Chr2:47429891 - 47429892 Pathogenic Frameshift variant rs63750086 .GenePathDx, GenePath diagnostics
NM_000251.3(MSH2):c.1165C>T (p.Arg389Ter) Single nucleotide variant Chr2:47429830 Pathogenic Nonsense rs587779075 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
.Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
.Neuberg Centre For Genomic Medicine, NCGM
NC_012920.1(MT-CYB):m.14766C>T Single nucleotide variant ChrMT:14766 Benign rs193302980 .Department of Zoology Govt. MVM College
.Genomics Division, Defence Institute of Physiology and Allied Sciences
NM_001365902.3(NFIX):c.1112del (p.Phe371fs) Deletion Chr19:13081712 Likely pathogenic Frameshift variant .Department Of Medical Genetics, Apollo Hospitals
NM_001365902.3(NFIX):c.416G>A (p.Gly139Glu) Single nucleotide variant Chr19:13025409 Pathogenic Missense variant rs1555696625 .Department of Medical Genetics, Sanjay Gandhi Post Graduate Institute of Medical Sciences
NM_001365902.3(NFIX):c.1087dup (p.Arg363fs) Duplication Chr19:13081683 - 13081684 Pathogenic Frameshift variant rs886041647 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_001205254.2(OCLN):c.1542del (p.Gly515fs) Deletion Chr5:69553643 Likely pathogenic Frameshift variant rs2531231205 .Lifecell International Pvt. Ltd
NM_000535.7(PMS2):c.1239dup (p.Asp414fs) Duplication Chr7:5987525 - 5987526 Pathogenic Frameshift variant|non-coding transcript variant rs267608159 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_000535.7(PMS2):c.2404C>T (p.Arg802Ter) Single nucleotide variant Chr7:5977629 Pathogenic Nonsense|non-coding transcript variant rs63751466 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_006941.4(SOX10):c.1400A>T (p.Ter467Leu) Single nucleotide variant Chr22:37973496 Likely pathogenic Stop lost|intron variant rs2145760379 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_006941.4(SOX10):c.644G>A (p.Arg215Gln) Single nucleotide variant Chr22:37977920 Conflicting classifications of pathogenicity Missense variant|intron variant rs779933527 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_000540.3(RYR1):c.9847C>T (p.Arg3283Ter) Single nucleotide variant Chr19:38517520 Pathogenic/Likely pathogenic Nonsense rs752199191 .Lifecell International Pvt. Ltd
NM_004168.4(SDHA):c.448G>A (p.Val150Met) Single nucleotide variant Chr5:225554 Uncertain significance Missense variant|intron variant rs542980860 .Laboratory of Molecular and Cytogenetics, Department of Anatomy, All India Institute of Medical Sciences (AIIMS)
NM_018344.6(SLC29A3):c.400C>T (p.Arg134Cys) Single nucleotide variant Chr10:71351578 Pathogenic/Likely pathogenic Missense variant|non-coding transcript variant rs1430557607 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_003172.4(SURF1):c.535dup (p.Arg179fs) Duplication Chr9:133352746 - 133352747 Pathogenic/Likely pathogenic Frameshift variant rs1836465016 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_003172.4(SURF1):c.833+1G>A Single nucleotide variant Chr9:133352060 Pathogenic Splice donor variant rs782609482 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_003172.4(SURF1):c.799_800del (p.Leu267fs) Microsatellite Chr9:133352094 - 133352095 Pathogenic/Likely pathogenic Frameshift variant rs864309500 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
.Neuberg Centre For Genomic Medicine, NCGM
NM_003172.4(SURF1):c.324-11T>G Single nucleotide variant Chr9:133353951 Conflicting classifications of pathogenicity Intron variant rs375398247 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
.Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
.Lifecell International Pvt. Ltd
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_000377.3(WAS):c.1271dup (p.Leu425fs) Duplication ChrX:48688994 - 48688995 Pathogenic Frameshift variant rs1557007312 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_000377.3(WAS):c.881T>C (p.Ile294Thr) Single nucleotide variant ChrX:48688403 Pathogenic/Likely pathogenic Missense variant rs387906717 .Lifecell International Pvt. Ltd

Indian Institutes & Variant Submission Overview
Chromosome-wise Variant Distribution