GenTIGSA Gene Database on Rare Genetic Disorders

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

Hereditary spastic paraplegia

An  Autosomal dominant, Autosomal recessive, X-linked dominant  mode(s) within the Neurodegenerative disorders  category

association 1
Conflicting classifications of pathogenicity 5
Likely pathogenic 5
Pathogenic 10
Pathogenic/Likely pathogenic 6
Uncertain significance 4

Variant name Variant type GRCH38 location Germline classification Molecular consequence dbSNP_ID Submitter
NM_015656.1(KIF26A):c.[1378delG];[4925C>T] CompoundHeterozygote Chr: association .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_002860.4(ALDH18A1):c.1111C>T (p.Arg371Ter) Single nucleotide variant Chr10:95626744 Pathogenic/Likely pathogenic Nonsense rs775201668 .Department of Biochemistry, All India Institute of Medical Sciences, Kalyani
NM_001368809.2(AMPD2):c.970C>T (p.Arg324Trp) Single nucleotide variant Chr1:109627793 Conflicting classifications of pathogenicity Missense variant rs776868175 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_004722.4(AP4M1):c.351+1G>A Single nucleotide variant Chr7:100102961 Likely pathogenic Splice donor variant .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_001128126.3(AP4S1):c.289C>T (p.Arg97Ter) Single nucleotide variant Chr14:31072968 Pathogenic/Likely pathogenic Nonsense rs200440467 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_031448.6(C19orf12):c.118T>G (p.Phe40Val) Single nucleotide variant Chr19:29708296 Conflicting classifications of pathogenicity Missense variant|5 prime UTR variant|intron variant rs2513299932 .Institute of Bioinformatics
.Neuberg Centre For Genomic Medicine, NCGM
NM_031448.6(C19orf12):c.152T>C (p.Leu51Pro) Single nucleotide variant Chr19:29708262 Likely pathogenic Missense variant|5 prime UTR variant|intron variant rs2145639419 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_031448.6(C19orf12):c.161-2del Deletion Chr19:29702979 Conflicting classifications of pathogenicity Splice acceptor variant rs1352744778 .Institute of Bioinformatics
NM_031448.6(C19orf12):c.-2C>T Single nucleotide variant Chr19:29708415 Pathogenic/Likely pathogenic 5 prime UTR variant|intron variant rs397514477 .Institute of Bioinformatics
NM_183075.3(CYP2U1):c.1355A>T (p.Asp452Val) Single nucleotide variant Chr4:107949416 Conflicting classifications of pathogenicity Missense variant rs775157727 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
.Neuberg Centre For Genomic Medicine, NCGM
NM_183075.3(CYP2U1):c.1396C>T (p.Arg466Ter) Single nucleotide variant Chr4:107949457 Pathogenic Nonsense rs766380148 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_001160148.2(DDHD1):c.340T>C (p.Ser114Pro) Single nucleotide variant Chr14:53152759 Uncertain significance Missense variant rs756788863 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_001160148.2(DDHD1):c.53GCG[6] (p.Gly22dup) Microsatellite Chr14:53153031 - 53153032 Uncertain significance Inframe_insertion rs752119831 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_015214.3(DDHD2):c.1396G>T (p.Glu466Ter) Single nucleotide variant Chr8:38251963 Likely pathogenic Nonsense|non-coding transcript variant .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_020435.4(GJC2):c.1141_1151del (p.Ser381fs) Deletion Chr1:228158895 - 228158905 Pathogenic Frameshift variant rs2124967068 .Lifecell International Pvt. Ltd
NM_001244008.2(KIF1A):c.1040A>G (p.Tyr347Cys) Single nucleotide variant Chr2:240773254 Conflicting classifications of pathogenicity Missense variant rs879254004 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_000533.5(PLP1):c.2T>G (p.Met1Arg) Single nucleotide variant ChrX:103776997 Pathogenic Missense variant|initiator_codon_variant rs864622194 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_007055.4(POLR3A):c.1771-7C>G Single nucleotide variant Chr10:78009682 Pathogenic/Likely pathogenic Intron variant rs201314157 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
.Neuberg Centre For Genomic Medicine, NCGM
NM_000533.5(PLP1):c.365A>G (p.Lys122Arg) Single nucleotide variant ChrX:103786638 Likely pathogenic Missense variant|intron variant rs1135401759 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_003105.6(SORL1):c.1211+1G>A Single nucleotide variant Chr11:121514322 Pathogenic Splice donor variant rs2496816791 .Human Genome Lab, NIMHANS, National Institute of Mental Health and Neuro Sciences
NM_014946.4(SPAST):c.631G>A (p.Val211Ile) Single nucleotide variant Chr2:32098840 Uncertain significance Missense variant|intron variant rs143003434 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_025137.4(SPG11):c.2621-1G>A Single nucleotide variant Chr15:44620404 Pathogenic Splice acceptor variant rs2505543230 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_025137.4(SPG11):c.6871dup (p.Cys2291fs) Duplication Chr15:44565981 - 44565982 Likely pathogenic Frameshift variant rs2505173672 .Lifecell International Pvt. Ltd
NM_025137.4(SPG11):c.5528del (p.Phe1843fs) Deletion Chr15:44584152 Pathogenic Frameshift variant rs2505291205 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
NM_025137.4(SPG11):c.6778C>T (p.Gln2260Ter) Single nucleotide variant Chr15:44566282 Pathogenic Nonsense rs2140914403 .Kasturba Medical College, Manipal, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India
NM_025137.4(SPG11):c.442+5G>T Single nucleotide variant Chr15:44660427 Uncertain significance Intron variant rs752482400 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
.Neuberg Centre For Genomic Medicine, NCGM
NM_025137.4(SPG11):c.2716del (p.Gln906fs) Deletion Chr15:44620308 Pathogenic Frameshift variant rs312262747 .Lifecell International Pvt. Ltd
.Neuberg Centre For Genomic Medicine, NCGM
NM_025137.4(SPG11):c.2146C>T (p.Gln716Ter) Single nucleotide variant Chr15:44626429 Pathogenic/Likely pathogenic Nonsense rs312262737 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
.Neuberg Centre For Genomic Medicine, NCGM
NM_025137.4(SPG11):c.733_734del (p.Met245fs) Deletion Chr15:44657230 - 44657231 Pathogenic Frameshift variant rs312262720 .Foundation for Research in Genetics and Endocrinology, FRIGE's Institute of Human Genetics
.Neuberg Centre For Genomic Medicine, NCGM
NM_003119.4(SPG7):c.2228T>C (p.Ile743Thr) Single nucleotide variant Chr16:89556933 Pathogenic/Likely pathogenic 3 prime UTR variant|missense variant rs752623413 .Lifecell International Pvt. Ltd
.Neuberg Centre For Genomic Medicine, NCGM
NM_016525.5(UBAP1):c.426_427del (p.Lys143fs) Deletion Chr9:34241450 - 34241451 Pathogenic Frameshift variant|non-coding transcript variant rs1563920252 .Lifecell International Pvt. Ltd

Indian Institutes & Variant Submission Overview
Chromosome-wise Variant Distribution