Unique variants in the KLHL7 gene

This database is one of the "Eye disease" gene variant databases.
Information The variants shown are described using the NM_001031710.2 transcript reference sequence.

63 entries on 1 page. Showing entries 1 - 63.
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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     
?/. 3 1 c.-235G>T r.(=) p.(=) - VUS g.23145411G>T - c.-235G/T - KLHL7_000035 - PubMed: Friedman 2009 - - Germline - - - - - LOVD
?/. 1 1 c.-118G>A r.(=) p.(=) - VUS g.23145528G>A - c.-118G/A - KLHL7_000036 - PubMed: Friedman 2009 - - Germline - - - - - LOVD
+/. 1 - c.? r.? p.? - pathogenic (dominant) g.? - Ala153Val - EZH2_000001 - PubMed: Daiger 2014 - - Germline - - - - - LOVD
-?/. 1 - c.21G>A r.(?) p.(Glu7=) - likely benign g.23145666G>A g.23106047G>A KLHL7(NM_001031710.2):c.21G>A (p.E7=) - KLHL7_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 4 1 c.53A>G r.? p.? - VUS g.23145698A>G - c.53A/G - KLHL7_000037 - PubMed: Friedman 2009 - rs17147682 Germline - - - - - LOVD
+?/. 1 1 c.112C>T r.(?) p.(Arg38Trp) - likely pathogenic (recessive) g.23145757C>T - c.112C>T - KLHL7_000030 - PubMed: Colombo-2020 - - Germline - - - - - LOVD
?/. 1 - c.120+3A>G r.spl? p.? - VUS g.23145768A>G - KLHL7(NM_001031710.3):c.120+3A>G - KLHL7_000032 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. 1 - c.126G>A r.(?) p.(Thr42=) - likely benign g.23163401G>A g.23123782G>A KLHL7(NM_001031710.2):c.126G>A (p.T42=) - KLHL7_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 2 c.181_183del r.? p.? - likely pathogenic g.23163456_23163458delTGT - c.181_183delTGT - KLHL7_000038 - PubMed: Kanthi-2019 - - Germline yes - - - - LOVD
+/. 2 3 c.247del r.(?) p.(Phe83Leufs*3) - pathogenic g.23164330del - c.247del; p.(Phe83Leufs*3) - KLHL7_000039 - PubMed: Cheraghi-2020 - - Germline - - - - - LOVD
-/. 1 - c.317+126C>G r.(=) p.(=) - benign g.23164526C>G g.23124907C>G KLHL7(NM_018846.5):c.173+126C>G - KLHL7_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-/., ?/. 2 4 c.352C>T r.(=), r.(?) p.(=) - benign, VUS g.23164701C>T - c.352C/T - KLHL7_000040 VKGL data sharing initiative Nederland PubMed: Friedman 2009 - rs15775 CLASSIFICATION record, Germline - - - - - VKGL-NL_Nijmegen
+/., +?/. 4 4 c.422T>C r.(?) p.(Val141Ala) - likely pathogenic, pathogenic (dominant) g.23164771T>C g.23125152T>C 7:23164771T>C ENST00000339077.5:c.422T>C (Val141Ala), c.422T>C, 1 more item - KLHL7_000021 heterozygous, VKGL data sharing initiative Nederland PubMed: Carss 2017, PubMed: Panneman 2023, PubMed: Turro 2020 - - CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown ? - - - - VKGL-NL_Nijmegen, Daan Panneman
+/., +?/. 10 4 c.433A>G r.(?) p.(Asn145Asp) ACMG likely pathogenic, pathogenic, pathogenic (dominant), VUS g.23164782A>G g.23125163A>G 7:23164782A>G ENST00000339077.5:c.433A>G (Asn145Asp), c.433A>G:p.(Asn145Asp), 3 more items - KLHL7_000022 heterozygous, solved, heterozygous, VKGL data sharing initiative Nederland PubMed: Carss 2017, PubMed: Jauregui 2020, PubMed: Midgley 2024, PubMed: Oh 2019, PubMed: Turro 2020, 1 more item - rs1182983579 CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown ? - - - - Johan den Dunnen, VKGL-NL_Nijmegen, Jinu Han
+/., +?/. 2 4 c.433A>T r.(?) p.(Asn145Tyr) - likely pathogenic, pathogenic g.23164782A>T g.23125163A>T c.433A>T:p.(Asn145Tyr)*, KLHL7 c.433A>T, p.N145Y - KLHL7_000027 heterozygous PubMed: Jauregui 2020, PubMed: Oh 2019 - - De novo, Unknown ? - - - - LOVD
+?/. 1 - c.440T>A r.(?) p.(Leu147His) - likely pathogenic g.23164789T>A - - - KLHL7_000054 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 4 5 c.449G>A r.(?) p.(Ser150Asn) ACMG likely pathogenic, VUS g.23180394G>A - c.449G/A, p.S150N - KLHL7_000020 0/102 Scandinavian controls, 0/183 North American controls and 0/185 UK controls PubMed: Friedman 2009, PubMed: Holtan 2020 - - Germline yes 2/899 cases - - - Global Variome, with Curator vacancy, Jinu Han
+?/. 2 5 c.457G>A r.(?) p.(Ala153Thr) - likely pathogenic g.23180402G>A - c.457G/A, p.A153T, c.457G>A - KLHL7_000026 - PubMed: Friedman 2009, PubMed: Sullivan-2013 - - Germline, Unknown - - - - - LOVD
+/., +?/. 16 5 c.458C>T r.(?) p.(Ala153Val) ACMG likely pathogenic, likely pathogenic (dominant), pathogenic, pathogenic (dominant) g.23180403C>T g.23140784C>T c.458C/T, p.A153V, c.458C>T, c.458C>T:p.(Ala153Val), NM_001031710 - KLHL7_000019 1 heterozygous, no homozygous; Clinindb (India), ACMG PP3, PM2, PM5_SUPPORTING, PM1, PP2, PP5_STRONG PubMed: Bowne 2011, PubMed: Colombo-2020, PubMed: Daiger 2014, PubMed: Friedman 2009, PubMed: Oh 2019, 7 more items 1009 rs137853113 Germline, Unknown yes 1/2420 IRD families, 1/2795 individuals - - - Global Variome, with Curator vacancy, Johan den Dunnen, Mohammed Faruq, Daan Panneman
-/. 1 - c.459G>A r.(?) p.(Ala153=) - benign g.23180404G>A g.23140785G>A KLHL7(NM_018846.5):c.315G>A (p.A105=) - KLHL7_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/., +?/., ?/. 4 5 c.472T>C r.(?) p.(Cys158Arg) - likely pathogenic, pathogenic, VUS g.23180417T>C g.23140798T>C c.472T>C:p.(Cys158Arg)*, KLHL7 c.472T>C, p.C158R, KLHL7 c.472T>C, p.Cys158Arg - KLHL7_000028 heterozygous PubMed: Jauregui 2020, PubMed: Oh 2019, PubMed: Zampaglione 2020 - - Germline, Unknown ? - - - - LOVD
+?/. 2 5 c.474+1G>A r.spl? p.? - likely pathogenic g.23180420G>A - NM_018846.4:c.474+1G>A - KLHL7_000041 - PubMed: Bruel 2017 - - Germline yes - - - - LOVD
+?/. 1 - c.506T>A r.(?) p.(Ile169Asn) - likely pathogenic g.23180451T>A g.23140832T>A - - KLHL7_000023 - PubMed: Stone 2017 - - Germline - - - - - LOVD
-?/., ?/. 3 5 c.513G>A r.(=), r.(?) p.(=), p.(Gln171=) - likely benign, VUS g.23180458G>A g.23140839G>A c.513G/A, KLHL7(NM_018846.4):c.369G>A (p.Q123=), KLHL7(NM_018846.5):c.369G>A (p.Q123=) - KLHL7_000015 VKGL data sharing initiative Nederland PubMed: Friedman 2009 - - CLASSIFICATION record, Germline - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
+?/. 1 - c.514C>G r.(?) p.(His172Asp) - likely pathogenic g.23180459C>G g.23140840C>G - - KLHL7_000024 - PubMed: Stone 2017 - - Germline - - - - - LOVD
+?/. 1 - c.598_601del r.(?) p.(Thr200*) - likely pathogenic g.23180543_23180546del g.23140924_23140927del KLHL7, variant 1: c.598_601del/p.T200* - KLHL7_000029 solved, heterozygous PubMed: Weisschuh 2020 - - Unknown ? - - - - LOVD
+?/. 1 5 c.614A>G r.(?) p.(Asp205Gly) - likely pathogenic (recessive) g.23180559A>G - c.614A>G - KLHL7_000031 - PubMed: Colombo-2020 - - Germline yes - - - - LOVD
+?/. 1 - c.618+1G>A r.spl? p.? - likely pathogenic (recessive) g.23180564G>A g.23140945G>A NM_018846.4:c.474+1G>A - KLHL7_000055 - PubMed: Nambot 2018 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.618+19C>T r.(=) p.(=) - likely benign g.23180582C>T - KLHL7(NM_001031710.3):c.618+19C>T - KLHL7_000056 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 1 - c.665G>A r.(?) p.(Arg222His) - VUS g.23183516G>A - KLHL7(NM_001031710.2):c.665G>A (p.(Arg222His)) - KLHL7_000059 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.738G>A r.(?) p.(Thr246=) - likely benign g.23183589G>A g.23143970G>A KLHL7(NM_018846.4):c.594G>A (p.T198=) - KLHL7_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 6 c.763G>A r.(?) p.(Asp255Asn) - VUS g.23183614G>A - c.763G/A - KLHL7_000042 - PubMed: Friedman 2009 - - Germline - - - - - LOVD
./. 1 - c.793+4006_*9718117del r.? p.? - pathogenic g.23187650_32932034del - - - ADCYAP1R1_000001 decreased gene dosage PubMed: DDDS 2015, Journal: DDDS 2015 - - De novo - - - - - Johan den Dunnen
?/. 1 - c.797G>A r.(?) p.(Gly266Glu) ACMG VUS g.23191689G>A g.23152070G>A - - KLHL7_000057 ACMG PM2, PP2 PubMed: Weisschuh 2024 - - Germline - - - - - Johan den Dunnen
?/. 1 7 c.816G>A r.(=) p.(=) - VUS g.23191708G>A - c.816G/A - KLHL7_000043 - PubMed: Friedman 2009 - - Germline - - - - - LOVD
+?/. 1 7 c.935_936+19del r.? p.? - likely pathogenic g.23191827_23191847del - c.935_936+19 del - KLHL7_000044 - PubMed: Bruel 2017 - - Germline yes - - - - LOVD
-?/. 1 - c.936+5C>A r.spl? p.? - likely benign g.23191833C>A g.23152214C>A KLHL7(NM_001031710.2):c.936+5C>A (p.?) - KLHL7_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 - c.969del r.(?) p.(Phe323LeufsTer15) ACMG likely pathogenic (dominant) g.23205349del g.23165730del - - KLHL7_000058 ACMG PM2, PVS1 PubMed: Weisschuh 2024 - - Germline/De novo (untested) - - - - - Johan den Dunnen
?/. 1 - c.1006A>C r.(?) p.(Asn336His) - VUS g.23205386A>C - - - KLHL7_000033 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. 1 - c.1007A>T r.(?) p.(Asn336Ile) - likely benign g.23205387A>T - KLHL7(NM_001031710.2):c.1007A>T (p.(Asn336Ile)) - KLHL7_000060 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 2 8 c.1022del r.(?) p.(Leu341Trpfs*9) - pathogenic g.23205402del g.23165783del - - KLHL7_000001 - PubMed: Angius 2016, Journal: Angius 2016 - - Germline - - - - - Jamie Zeegers
+?/. 2 8 c.1051C>T r.(?) p.(Arg351*) - likely pathogenic g.23205431C>T - NM_018846.4:p.Arg351* - KLHL7_000045 - PubMed: Bruel 2017, PubMed: Heng 2019 - - Germline yes - - - - LOVD
+?/. 1 8 c.1115G>A r.(?) p.(Arg372Gln) - likely pathogenic g.23205495G>A g.23165876G>A - - KLHL7_000002 - PubMed: Angius 2016, Journal: Angius 2016 - - Germline - - - - - Jamie Zeegers
+?/. 1 8 c.1147A>T r.(?) p.(Lys383*) - likely pathogenic g.23205527A>T - NM_018846.4:p.Lys383* - KLHL7_000046 - PubMed: Bruel 2017 - - Germline yes - - - - LOVD
?/. 1 8i c.1177+77C>T r.(=) p.(=) - VUS g.23205634C>T - c.1177+77C/T - KLHL7_000047 - PubMed: Friedman 2009 - rs858312 Germline - - - - - LOVD
+?/. 1 9 c.1258C>T r.(?) p.(Arg420Cys) - likely pathogenic g.23207535C>T g.23167916C>T - - KLHL7_000003 - PubMed: Angius 2016, Journal: Angius 2016 - - Germline - - - - - Jamie Zeegers
+?/. 1 9 c.1261T>A r.(?) p.(Cys421Ser) - likely pathogenic g.23207538T>A g.23167919T>A - - KLHL7_000004 - PubMed: Angius 2016, Journal: Angius 2016 - - Germline - - - - - Jamie Zeegers
-/. 1 9 c.1267C>T r.(?) p.(His423Tyr) - benign g.23207544C>T - c.1267C/T - KLHL7_000048 - PubMed: Friedman 2009 - - Germline no - - - - LOVD
?/. 1 - c.1272G>T r.(?) p.(Gly424=) - VUS g.23207549G>T - - - KLHL7_000034 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 9 c.1353T>C r.(=) p.(=) - VUS g.23207630T>C - c.1353T/C - KLHL7_000049 - PubMed: Friedman 2009 - - Germline - - - - - LOVD
-?/. 1 - c.1374A>G r.(?) p.(Thr458=) - likely benign g.23207651A>G g.23168032A>G KLHL7(NM_018846.4):c.1230A>G (p.T410=) - KLHL7_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.1378A>G r.(?) p.(Thr460Ala) - VUS g.23207655A>G g.23168036A>G - - KLHL7_000013 - PubMed: Koyanagi 2019, Journal: Koyanagi 2019 - rs761755398 Germline - 6/1204 cases with retinitis pigmentosa - - - Yoshito Koyanagi
?/. 1 9i c.1380-12T>G r.(=) p.(=) - VUS g.23212555T>G - c.1380-12T/G - KLHL7_000050 - PubMed: Friedman 2009 - - Germline - - - - - LOVD
+?/. 1 - c.1380-1G>C r.spl p.? - likely pathogenic g.23212566G>C g.23172947G>C IVS9-1 G>C - KLHL7_000025 - PubMed: Stone 2017 - - Germline - - - - - LOVD
?/. 1 10 c.1414A>G r.(?) p.(Lys472Glu) - VUS g.23212601A>G - c.1414A/G - KLHL7_000051 - PubMed: Friedman 2009 - - Germline - - - - - LOVD
?/. 1 - c.1438A>G r.(?) p.(Lys480Glu) - VUS g.23212625A>G g.23173006A>G KLHL7(NM_018846.5):c.1294A>G (p.K432E) - KLHL7_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/., ?/. 2 10 c.1440A>G r.(=), r.(?) p.(=), p.(Lys480=) - likely benign, VUS g.23212627A>G g.23173008A>G c.1440A/G, KLHL7(NM_018846.4):c.1296A>G (p.K432=) - KLHL7_000017 VKGL data sharing initiative Nederland PubMed: Friedman 2009 - - CLASSIFICATION record, Germline - - - - - VKGL-NL_Rotterdam
?/. 1 - c.1447A>G r.(?) p.(Ile483Val) - VUS g.23212634A>G g.23173015A>G KLHL7(NM_018846.5):c.1303A>G (p.I435V) - KLHL7_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 1 - c.1531G>A r.(?) p.(Val511Ile) - VUS g.23213687G>A g.23174068G>A KLHL7(NM_018846.4):c.1387G>A (p.V463I) - KLHL7_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 11 c.1578T>C r.(=) p.(=) - VUS g.23213734T>C - c.1578T/C - KLHL7_000052 - PubMed: Friedman 2009 - - Germline - - - - - LOVD
-?/. 1 - c.1584T>C r.(?) p.(Ser528=) - likely benign g.23213740T>C g.23174121T>C KLHL7(NM_018846.4):c.1440T>C (p.S480=) - KLHL7_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 11 c.*7A>G r.(=) p.(=) - VUS g.23213924A>G - c.*7A/G - KLHL7_000053 - PubMed: Friedman 2009 - - Germline - - - - - LOVD
?/. 3 - c.*23_*35delinsTG r.? p.? - VUS g.? - c.*23_35delinsTG - EZH2_000001 - PubMed: Friedman 2009 - - Germline - - - - - LOVD
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