Unique variants in the HARS gene

This database is one of the "Eye disease" gene variant databases. NOTE: gene name changed from HARS to HARS1
Information The variants shown are described using the NM_002109.3 transcript reference sequence.

59 entries on 1 page. Showing entries 1 - 59.
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Effect     

Reported     

Exon     

AscendingDNA change (cDNA)     

RNA change     

Protein     

Classification method     

Clinical classification     

DNA change (genomic) (hg19)     

DNA change (hg38)     

Published as     

ISCN     

DB-ID     

Variant remarks     

Reference     

ClinVar ID     

dbSNP ID     

Origin     

Segregation     

Frequency     

Re-site     

VIP     

Methylation     

Owner     
./. 1 - c.-5646396_*16225318dup - - - pathogenic g.123828524_145717285dup - - - SIL1_000024 mosaicism, copy number 3 in 0.38 cells PubMed: DDDS 2015, Journal: DDDS 2015 - - Somatic - - - - - Johan den Dunnen
-?/. 1 - c.-4521A>G r.(?) p.(=) - likely benign g.140075410T>C g.140695825T>C HARS2(NM_012208.3):c.613T>C (p.L205=) - ZMAT2_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.-4472G>A r.(?) p.(=) - likely benign g.140075361C>T g.140695776C>T HARS2(NM_012208.3):c.564C>T (p.P188=) - ZMAT2_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 - c.-4292dup r.(?) p.(=) - pathogenic g.140075182dup g.140695597dup HARS2(NM_012208.3):c.489dupC (p.I164Hfs*7) - ZMAT2_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.-4217C>T r.(?) p.(=) - VUS g.140075106G>A g.140695521G>A - - ZMAT2_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. 2 - c.-2994C>T r.(?) p.(=) - benign g.140073883G>A g.140694298G>A HARS2(NM_012208.4):c.399+18G>A - HARS2_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen, VKGL-NL_Nijmegen
?/. 1 - c.-2966T>C r.(?) p.(=) - VUS g.140073855A>G g.140694270A>G - - ZMAT2_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.-2944C>T r.(?) p.(=) - VUS g.140073833G>A g.140694248G>A HARS2(NM_001363535.1):c.385G>A (p.G129R) - ZMAT2_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.-2901A>G r.(?) p.(=) - likely benign g.140073790T>C g.140694205T>C HARS2(NM_012208.3):c.324T>C (p.Y108=) - HARS2_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.-351G>C r.(?) p.(=) - likely benign g.140071240C>G g.140691655C>G HARS2(NM_012208.3):c.7C>G (p.L3V) - HARS2_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 1 - c.-6G>T r.(?) p.(=) - benign g.140070895C>A g.140691310C>A HARS1(NM_002109.6):c.-6G>T - HARS2_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/., -?/., ?/? 4 1 c.14C>A r.(?), r.14c>a p.(Ala5Glu), p.Ala5Glu - benign, likely benign, VUS g.140070876G>T g.140691291G>T 1 more item - HARS_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record, Unknown ? 2/726 - - - Anthony Antonellis, VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_AMC
-/., ?/. 2 - c.40C>A r.(?) p.(Gln14Lys) - benign, VUS g.140070850G>T g.140691265G>T HARS(NM_002109.6):c.40C>A (p.Q14K), HARS1(NM_002109.6):c.40C>A (p.Q14K) - HARS_000020 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
?/. 1 - c.89T>G r.(?) p.(Leu30Arg) - VUS g.140070801A>C g.140691216A>C HARS1(NM_001258041.3):c.89T>G (p.L30R) - HARS2_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 1 - c.90+4A>C r.spl? p.? - VUS g.140070796T>G g.140691211T>G HARS(NM_002109.6):c.90+4A>C - HARS2_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.179A>C r.(?) p.(Lys60Thr) - VUS g.140070441T>G g.140690856T>G HARS(NM_002109.6):c.179A>C (p.K60T) - HARS_000019 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 1 - c.181-15G>C r.(=) p.(=) - benign g.140062819C>G g.140683234C>G HARS1(NM_002109.6):c.181-15G>C - HARS_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+?/. 1 - c.191dup r.(?) p.(Asp64GlufsTer3) - likely pathogenic g.140062794dup g.140683209dup HARS(NM_002109.6):c.191dupA (p.D64Efs*3) - HARS2_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.194_196del r.(?) p.(Tyr65_Ser66delinsCys) - likely pathogenic g.140062789_140062791del g.140683204_140683206del HARS(NM_002109.6):c.194_196delATA (p.Y65_S66delinsC) - HARS_000017 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.198del r.(?) p.(Arg68GlyfsTer12) - likely pathogenic g.140062787del g.140683202del HARS(NM_002109.6):c.198delT (p.R68Gfs*12) - HARS_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.202C>T r.(?) p.(Arg68Trp) - VUS g.140062783G>A - HARS(NM_001258041.2):c.202C>T (p.R68W) - HARS_000027 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.203G>A r.(?) p.(Arg68Gln) - VUS g.140062782C>T - HARS(NM_001258041.2):c.203G>A (p.R68Q) - HARS2_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
?/. 1 - c.218G>A r.(?) p.(Arg73His) - VUS g.140062767C>T - HARS(NM_001258041.2):c.218G>A (p.R73H) - HARS2_000023 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-/. 1 - c.234C>T r.(?) p.(Asp78=) - benign g.140062751G>A g.140683166G>A HARS1(NM_002109.6):c.234C>T (p.D78=) - HARS_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. 1 - c.246T>G r.(?) p.(Arg82=) - likely benign g.140062739A>C g.140683154A>C HARS1(NM_002109.6):c.246T>G (p.R82=) - HARS2_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 1 - c.262G>A r.(?) p.(Gly88Ser) - pathogenic (recessive) g.140062723C>T g.140683138C>T - - HARS_000024 - PubMed: Tiwari 2016 - - Germline - - - - - LOVD
-?/. 1 - c.297A>G r.(?) p.(Leu99=) - likely benign g.140062688T>C g.140683103T>C HARS(NM_001258041.2):c.297A>G (p.L99=) - HARS2_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.307C>G r.(?) p.(Leu103Val) - VUS g.140059462G>C g.140679877G>C - - HARS_000025 23/1266 control chromosomes PubMed: Xu 2015 - - Germline - 5/314 case chromosomes - - - LOVD
?/. 1 - c.340A>G r.(?) p.(Ile114Val) - VUS g.140059429T>C g.140679844T>C HARS1(NM_002109.6):c.340A>G (p.I114V) - HARS2_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 2 - c.382C>T r.(?) p.(Arg128Cys) - VUS g.140059387G>A g.140679802G>A HARS(NM_002109.6):c.382C>T (p.R128C), HARS1(NM_002109.6):c.382C>T (p.R128C) - HARS_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
+?/. 2 - c.395C>T r.(?) p.(Thr132Ile), p.Thr132Ile ACMG likely pathogenic g.140059374G>A g.140679789G>A - - HARS_000021 ACMG grading: PP3,PM2,PS3,PP5; reported in Safka Brozkova 2015. Brain 138: 2161, 1 more item - - rs143473232 Germline - - - - - Andreas Laner
+/., +/?, +?/., ?/. 8 4 c.410G>A r.(?), r.410g>a p.(Arg137Gln), p.Arg137Gln - likely pathogenic, pathogenic, pathogenic (recessive), VUS g.140058699C>T g.140679114C>T 1 more item - HARS_000005 conflicting interpretations of pathogenicity; 1 heterozygous, no homozygous; Clinindb (India), 1 more item PubMed: Narang 2020, Journal: Narang 2020, PubMed: Tiwari 2016 - rs191391414 CLASSIFICATION record, Germline, Unknown ? 1/2795 individuals, 1/726 - - - Anthony Antonellis, VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_Nijmegen, VKGL-NL_AMC, Mohammed Faruq
?/. 1 - c.437_439dup r.(?) p.(Asn146dup) - VUS g.140058672_140058674dup - HARS(NM_001258041.2):c.437_439dupACA (p.N146dup) - HARS2_000022 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.581T>C r.(?) p.(Ile194Thr) - VUS g.140057542A>G g.140677957A>G - - HARS_000022 - PubMed: Cox 2019 - - Germline - - - - - Timothy Cox
-/. 1 - c.588C>T r.(?) p.(Cys196=) - benign g.140057535G>A g.140677950G>A HARS1(NM_002109.6):c.588C>T (p.C196=) - HARS_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/? 1 5 c.605G>A r.605g>a p.Gly205Asp - VUS g.140057518C>T - - - HARS_000004 1 more item - - - Unknown ? 2/726 - - - Anthony Antonellis
-/., -?/., ?/. 4 - c.614G>A r.(?) p.(Gly205Asp) - benign, likely benign, VUS g.140057509C>T g.140677924C>T HARS(NM_001258041.2):c.554G>A (p.G185D), HARS1(NM_002109.6):c.614G>A (p.G205D) - DND1_000012 8 heterozygous, no homozygous; Clinindb (India), VKGL data sharing initiative Nederland PubMed: Narang 2020, Journal: Narang 2020 - rs147288996 CLASSIFICATION record, Germline - 8/2795 individuals - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen, VKGL-NL_AMC, Mohammed Faruq
-?/., ?/., ?/? 4 7 c.713T>C r.(?), r.713u>c p.(Val238Ala), p.Val238Ala - likely benign, VUS g.140057256A>G g.140677671A>G HARS1(NM_002109.6):c.713T>C (p.V238A) - HARS_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record, Unknown ? 1/726 - - - Anthony Antonellis, VKGL-NL_Groningen, VKGL-NL_Nijmegen, VKGL-NL_AMC
?/. 1 - c.743A>T r.(?) p.(Glu248Val) - VUS g.140056992T>A - HARS(NM_001258041.2):c.683A>T (p.E228V) - DND1_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.800T>C r.(?) p.(Ile267Thr) - VUS g.140056935A>G - HARS1(NM_001258041.3):c.740T>C (p.I247T) - DND1_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. 1 - c.834C>A r.(?) p.(Ser278=) - likely benign g.140056691G>T g.140677106G>T HARS(NM_002109.6):c.834C>A (p.S278=) - DND1_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 1 - c.903C>T r.(?) p.(Asp301=) - benign g.140056622G>A g.140677037G>A HARS1(NM_002109.6):c.903C>T (p.D301=) - DND1_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. 1 - c.951+19G>A r.(=) p.(=) - benign g.140056555C>T g.140676970C>T HARS1(NM_002109.6):c.951+19G>A - DND1_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 1 - c.1056G>A r.(?) p.(Leu352=) - VUS g.140056377C>T g.140676792C>T HARS1(NM_002109.6):c.1056G>A (p.L352=) - HARS_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/., ?/? 2 9 c.1127A>G r.(?), r.1127a>g p.(Lys376Arg), p.Lys376Arg - benign, VUS g.140056306T>C g.140676721T>C HARS1(NM_002109.6):c.1127A>G (p.K376R) - HARS_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record, Unknown ? 1/726 - - - Anthony Antonellis, VKGL-NL_AMC
?/. 1 - c.1156G>T r.(?) p.(Val386Leu) - VUS g.140056277C>A g.140676692C>A HARS(NM_002109.6):c.1156G>T (p.V386L) - DND1_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/., ?/. 3 - c.1194+1G>A r.spl? p.? - pathogenic, VUS g.140056238C>T g.140676653C>T HARS(NM_002109.6):c.1194+1G>A, HARS1(NM_002109.6):c.1194+1G>A - HARS_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen, VKGL-NL_AMC
-/. 2 - c.1196C>T r.(?) p.(Ala399Val) - benign g.140054717G>A - HARS1(NM_001258041.3):c.1136C>T (p.A379V), HARS1(NM_002109.6):c.1196C>T (p.A399V) - HARS_000026 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_AMC
-?/. 1 - c.1201G>A r.(?) p.(Glu401Lys) - likely benign g.140054712C>T g.140675127C>T HARS(NM_002109.6):c.1201G>A (p.E401K) - DND1_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 1 - c.1261C>G r.(?) p.(Leu421Val) - benign g.140054652G>C g.140675067G>C HARS1(NM_001258041.3):c.1201C>G (p.L401V) - DND1_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 1 - c.1309A>G r.(?) p.(Lys437Glu) - VUS g.140054604T>C g.140675019T>C HARS1(NM_002109.6):c.1309A>G (p.K437E) - HARS_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+?/., ?/. 3 - c.1361A>C r.(?) p.(Tyr454Ser) - likely pathogenic, VUS g.140054361T>G g.140674776T>G HARS c.1361A>C, p.Tyr454Ser - HARS_000023 conflicting interpretations of pathogenicity; 1 heterozygous, no homozygous; Clinindb (India), 1 more item PubMed: Narang 2020, Journal: Narang 2020, PubMed: Puffenberger 2012 - rs387906639 Germline yes 1/2795 individuals, population-specific allele frequency: 1.72% (7/406) - - - Mohammed Faruq
-?/., ?/. 3 - c.1445C>T r.(?) p.(Thr482Met) - likely benign, VUS g.140054277G>A g.140674692G>A HARS(NM_002109.6):c.1445C>T (p.T482M) - HARS_000008 0/1266 control chromosomes, 3 heterozygous, no homozygous; Clinindb (India), 1 more item PubMed: Narang 2020, Journal: Narang 2020, PubMed: Xu 2015 - rs147372931 CLASSIFICATION record, Germline - 1/314 case chromosomes, 3/2795 individuals - - - VKGL-NL_Rotterdam, Mohammed Faruq
-?/. 1 - c.1446G>A r.(?) p.(Thr482=) - likely benign g.140054276C>T - HARS(NM_001258041.2):c.1386G>A (p.T462=) - DND1_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 2 - c.1458+7G>A r.(=) p.(=) - benign g.140054257C>T g.140674672C>T HARS1(NM_002109.6):c.1458+7G>A - HARS_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen, VKGL-NL_AMC
?/? 1 12 c.1513C>T r.1513c>u p.Pro505Ser - VUS g.140053859G>A g.140674274G>A - - HARS_000001 - - - - Unknown ? 1/726 - - - Anthony Antonellis
-?/. 1 - c.*2756G>A r.(=) p.(=) - likely benign g.140051086C>T g.140671501C>T DND1(NM_194249.2):c.854G>A (p.(Trp285Ter)) - DND1_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.*2935G>A r.(=) p.(=) - likely benign g.140050907C>T g.140671322C>T DND1(NM_194249.2):c.1033G>A (p.(Ala345Thr)) - DND1_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.*2948C>G r.(=) p.(=) - likely benign g.140050894G>C g.140671309G>C DND1(NM_194249.2):c.1046C>G (p.(Thr349Ser)) - DND1_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
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aliases include HRS, USH3B


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