Unique variants in the RS1 gene

This database is one of the "Eye disease" gene variant databases.
Information The variants shown are described using the NM_000330.3 transcript reference sequence.

563 entries on 6 pages. Showing entries 1 - 100.
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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 3i_6_ c.(184+1_185-1)_(*2316_?)del r.? p.? - pathogenic (recessive) g.(?_18657808)_(18665453_18674772)del g.(?_18639688)_(18647333_18656652)del ex4-6del - RS1_000485 - PubMed: Gao 2021 - - Germline - - - - - Johan den Dunnen
+?/. 2 5i_6_ c.(522+1_523-1)_(*2316_?)del r.spl p.(?) - likely pathogenic g.(?_18657808)_(18660277_18662549)del g.(?_18639688)_(18642157_18644429)del RS1, variant 1 :Deletion exon 6 - RS1_000011 solved, hemizygous PubMed: Weisschuh 2020 - - Unknown ? - - - - LOVD
+/. 1 _1_1i c.-2577_52+260del r.0? p.0? - pathogenic (recessive) g.18689879_18692767del g.18671759_18674647del [-5303_-3784del; -2579_52+258del] - RS1_000198 fragment cloned PubMed: Huopaniemi 2000 - - Germline yes - - - - Johan den Dunnen
+?/. 1 _1_1i c.-1758_52+2664del r.0? p.0? - likely pathogenic g.18687473_18691946del g.18669353_18673826del c.(-35)-1723_c.51+2664del4472 - RS1_000324 error in annotation, exon 1 is ending on nucleotide 52 PubMed: D'Souza - - Unknown ? - - - - LOVD
+/. 1 _1_1i c.-1492_53-421del r.0? p.0? ACMG pathogenic g.18676206_18691680del g.18658086_18673560del del ex1 - RS1_000422 - PubMed: Chen 2020 - - Germline/De novo (untested) ? 1/90 cases RS - - - LOVD
?/. 1 1 c.(?_-473)_(17+1_?)del r.(?) p.? - VUS g.18690171_18690661del - exon 1 deletion - RS1_000278 Check also: Bowles 2011 PubMed: Avela 2019 - - Germline - - - - - LOVD
+?/. 5 _1_1i c.(?_-35)_(52+1_53-1)del r.0? p.0? - likely pathogenic g.(18675786_18690136)_(18690223_?)del g.(18657666_18672016)_(18672103_?)del Exon 1 deletion - RS1_000010 - PubMed: Sergeev 2013 - - Unknown ? - - - - LOVD
+?/. 3 _1_1i c.(?_-35)_(52+1_53-1)del r.0? p.0? - likely pathogenic g.(18675786_18690136)_(18690223_?)del g.(18657666_18672016)_(18672103_?)del exon 1 deletion - RS1_000010 - PubMed: Pennesi 2018 - - Unknown ? - - - - LOVD
?/. 2 _1_2i c.(?_-35)_(78+1_79-1)del r.0? p.0? - VUS g.(18674879_18675759)_(18690223_?)del g.(18656759_18657639)_(18672103_?)del RS1 nucleotide 1, protein 1:exon1del, p.? - RS1_000307 hemizygous, ACMG unclassified - no access to supplementary table 2 PubMed: Hull 2020 - - Germline ? - - - - LOVD
+?/. 1 _1_3i c.(?_-35)_(184+1_185-1)del r.0? p.0? - likely pathogenic (maternal) g.(18665453_18674772)_(18690223_?)del g.(18647333_18656652)_(18672103_?)del E1-3del - RS1_000123 - PubMed: Liu-2020 - - Germline - - - - - LOVD
+/. 8 _1_1i c.(?_-35)_(52+1_53-1)del r.0? p.0?, p.? ACMG pathogenic (recessive) g.(18675786_18690136)_(18690223_?)del g.(18657666_18672016)_(18672103_?)del (?_1-1)_(52+1_53-1)del, del ex1, (1-?_52+?)del, del ex1-4, (1-?_326+?)del, del ex4-5, (185-?_522+?)del, 1 more item - RS1_000010 ACMG PVS1, PS4, PM2, PP4 PubMed: Bender 2022, PubMed: Georgiou 2022 - - Germline - - - - - Johan den Dunnen
+/. 1 _1_3i c.(?_-35)_(184+1_185-1)del r.0? p.0? - pathogenic (recessive) g.(18665453_18674772)_(18779695_18797127)del g.(18647333_18656652)_(18761577_18779009)del ex1-3del - RS1_000123 - PubMed: Gao 2021 - - Germline - - - - - Johan den Dunnen
+/. 1 _1_4i c.(?_-35)_(326+1_327-1)del r.0? p.? ACMG pathogenic (recessive) g.(18665453_18674772)_(18690223_?)del g.(18647333_18656652)_(18672103_?)del - - RS1_000123 ACMG PVS1, PM2, PP4 PubMed: Bender 2022 - - Germline - - - - - Johan den Dunnen
+?/. 2 _1_6_ c.(?_-35)_(*2316_?)del r.0 p.0 - likely pathogenic g.(?_18657808)_(18690223_?)del g.(?_18639688)_(18672103_?)del 170bp deletion Xp22.13 - RS1_000000, RS1_000023 large deletion chrX (p22.2-p22.13) including OFD1 and RS1 PubMed: Bravo-Gil 2017, PubMed: Holtan 2020 - - Germline - 1/899 cases - - - Global Variome, with Curator vacancy, Nereida Bravo Gil
+/. 8 _1_1i c.(?_-35)_(52+1_53-1)del r.0? p.0? - pathogenic g.(18675786_18690136)_(18690223_?)del g.(18657666_18672016)_(18672103_?)del del ex1 - RS1_000010 deletion upto 1 kb 5' ATG PubMed: Hiriyanna 1999, PubMed: Eksandh 2000, PubMed: Eksandh 2005, PubMed: Shinoda 2000, 2 more items - - Germline - - - - - Johan den Dunnen, Dorothy Trump
+/. 2 _1_3i c.(?_-35)_(184+1_185-1)del r.0? p.0? - pathogenic, pathogenic (recessive) g.(18665453_18674772)_(18690223_?)del, g.(18665453_18674772)_(18779695_18797127)del g.(18647333_18656652)_(18672103_?)del, g.(18647333_18656652)_(18761577_18779009)del - - RS1_000123 136 kb deletion from PPEF intron 9 to RS1 intron 3 also involving CDKL5 (STK9) PubMed: Huopaniemi, PubMed: Yu 2001 - - Germline yes - - - - Johan den Dunnen
?/. 1 _1 - r.? p.? - VUS g.18693972_18695491del g.18675852_18677371del [-5303_-3784del;-2579_52+258del] - RS1_000308 fragment cloned PubMed: Huopaniemi 2000 - - Germline - - - - - Johan den Dunnen
+?/. 3 _1_1i c.(?_-35)_(52+1_53-1)del r.0? p.0? - likely pathogenic g.(18675786_18690136)_(18690223_?)del g.(18657666_18672016)_(18672103_?)del del ex1, RS1 exon1 deletion - RS1_000010 - PubMed: Avela 2019, PubMed: Chen 2014, PubMed: Fahim 2017 - - Germline, Germline/De novo (untested) yes - - - - LOVD
+/. 1 _1_6_ c.= r.(=) p.(=) - NA g.= - - - RS1_000000 expression construct in pcDNA3.1, COS-7 cell expression - - - In vitro (cloned) - - - - - Johan den Dunnen
+/. 3 _1_1i c.(?_-35)_(52+1_53-1)del r.0? p.0? - pathogenic g.(18675786_18690136)_(18690223_?)del g.(18657666_18672016)_(18672103_?)del - - RS1_000010 deletion upto 1 kb 5' ATG PubMed: Chan 2004, PubMed: Renner 2008, PubMed: Tsang 2007 - - Germline - - - - - Johan den Dunnen
+/. 2 1 c.1A>G r.(?) p.(Met1?) - pathogenic, pathogenic (recessive) g.18690188T>C g.18672068T>C - - RS1_000093 - PubMed: Mashima 1999, PubMed: Shinoda 2000 - - Germline, Germline/De novo (untested) - - -MboII - - Johan den Dunnen
+/., +?/. 8 1 c.1A>T r.(?), r.0? p.(Met1?) ACMG likely pathogenic, pathogenic, pathogenic (recessive) g.18690188T>A g.18672068T>A c.1A>T, p.Met1Lle, RS1 c.1A>T, p.Met1Leu, [35T>A;52+5G>C] - RS1_000160 ACMG PVS1, PS4, PM2, PS3>M, PP3, PP4, not in 100 control chromosomes, 1 more item PubMed: Bender 2022, PubMed: Kim 2006, PubMed: Pennesi 2018, PubMed: Stone 2017, 1 more item - - Germline, Unknown ? - -MboII - - Johan den Dunnen
+?/. 1 - c.2T>A r.(?) p.? - likely pathogenic g.18690187A>T - - - RS1_000429 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/., +?/. 2 1 c.2T>C r.(?) p.(Met1?) - likely pathogenic, pathogenic g.18690187A>G g.18672067A>G - - RS1_000419 VKGL data sharing initiative Nederland PubMed: RS-consortium 1998, group 5 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Nijmegen
+/., +?/. 6 - c.3G>A r.(?) p.(Met1?) ACMG likely pathogenic, pathogenic (recessive) g.18690186C>T g.18672066C>T c.3G> A (p.M1) in the RS1 gene - RS1_000408 ACMG PS1, PP4, PP5, PM2, PM3 PubMed: D'Anna Mardero 2024, PubMed: Xiao 2016 - - Germline, Germline/De novo (untested) yes - - - - Johan den Dunnen
+/., +?/. 2 1 c.3G>T r.(?) p.(Met1?) - likely pathogenic, pathogenic (recessive) g.18690186C>A g.18672066C>A (Met1Ile) - RS1_000289 VKGL data sharing initiative Nederland PubMed: Hahn 2022 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Nijmegen
+/. 2 _1_1i c.(-63_3)_(52+3913_52+4861)del r.0? p.0? - pathogenic (recessive) g.(18685276_18686224)_(18690186_18690251)del g.(18667156_18668104)_(18672066_18672131)del - arr[GRCh37] Xp22.13(18685276x1,18686224_18690186x0,18690251x1) RS1_000480 - PubMed: Fortunato 2023 - - Germline - - - - - Johan den Dunnen
+/., -?/. 2 1 c.7C>G r.(?) p.(Arg3Gly) - likely benign, pathogenic (recessive) g.18690182G>C g.18672062G>C RS1(NM_000330.3):c.7C>G (p.(Arg3Gly)) - RS1_000219 VKGL data sharing initiative Nederland PubMed: Chowdury 2024 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Leiden
+?/. 1 - c.14del r.(?) p.(Ile5LysfsTer121) - likely pathogenic (recessive) g.18690175del g.18672055del 14delT - RS1_000511 - PubMed: Gao 2021 - - Germline - - - - - Johan den Dunnen
+/. 4 1 c.20del r.(?) p.(Gly7AlafsTer119) - pathogenic (recessive) g.18690170del g.18672050del - - RS1_000482 ACMG PVS1, PM2, PP3, PP4 PubMed: Georgiou 2022, PubMed: Kousal 2021 - - Germline - - - - - Johan den Dunnen
+/. 1 1 c.26del r.(?) p.(Leu9Cysfs*117) - pathogenic g.18690167del g.18672047del - - RS1_000162 - PubMed: Li 2007, PubMed: Ma 2008 - - Germline - - - - - Johan den Dunnen
+?/. 1 1 c.26T>A r.(?) p.(Leu9*) - likely pathogenic g.18690163A>T g.18672043A>T c.26A>T, p.(Leu9*) - RS1_000296 error in annotation: c.26A>T instead of T>A, hemizygous PubMed: Wang 2019 - - Germline ? - - - - LOVD
+/., +?/. 7 1 c.33_36del r.(?) p.(Leu11Phefs*114), p.(Leu11PhefsTer114) - likely pathogenic, pathogenic, pathogenic (recessive) g.18690155_18690158del g.18672035_18672038del RS1, variant 1: c.33_36del/p.L11Ffs*114 - RS1_000012 solved, hemizygous PubMed: Kousal 2021, PubMed: RS-consortium 1998, group 3, PubMed: RS-consortium 1998, group 5, 1 more item ClinVar-000098944.7 - Germline, Unknown ? - - - - Johan den Dunnen
+?/. 1 - c.34_35insGATA r.(?) p.(Leu12ArgfsTer7) ACMG likely pathogenic (recessive) g.18690154_18690155insTATC g.18672034_18672035insTATC - - RS1_000481 ACMG PVS1, PP4, PM2 PubMed: D'Anna Mardero 2024 - - Germline/De novo (untested) - - - - - Johan den Dunnen
+/., +?/., ?/. 15 1 c.35T>A -, r.(?) p.(Leu12His), p.Leu12His ACMG likely pathogenic, likely pathogenic (recessive), NA, pathogenic, pathogenic (recessive), VUS g.18690154A>T g.18672034A>T Leu12His, RS1 c.35T>A, p.(Leu12His), RS1 c.35T>A, p.Leu12His, [35T>A;52+5G>C] - RS1_000016 ACMG PM1, PM2, PP2, PP3, PP4, PP5, ACMG PS4, PM2, PS3>M, PP3, PP4, 2 more items PubMed: Bender 2022, PubMed: Fahim 2017, PubMed: Georgiou 2022, PubMed: RS-consortium 1998, group 3, 2 more items - rs62645879 Germline, In vitro (cloned), Unknown ? - - - - Johan den Dunnen, Dorothy Trump
+?/. 1 1 c.35T>C r.(?) p.(Leu12Pro) - likely pathogenic g.18690154A>G g.18672034A>G RS1 c.35T>C, p.Leu12Pro - RS1_000407 - PubMed: Kondo 2019 - - Unknown ? - - - - LOVD
+?/., -?/., ?/. 3 1 c.37C>T -, r.(?) p.(Leu13Phe), p.Leu13Phe - likely pathogenic, NA, VUS g.18690152G>A g.18672032G>A Leu13Phe, RS1 c.37C>T, p.Leu13Phe, RS1(NM_000330.3):c.37C>T (p.L13F) - RS1_000258 in vitro analysis COS-7 cells shows readily secreted RS1 in cellular fraction, 1 more item PubMed: Vijayasarathy 2010 - - CLASSIFICATION record, In vitro (cloned), Unknown ? - - - - Johan den Dunnen, VKGL-NL_Rotterdam
+/., +?/. 4 1 c.38T>C -, r.(?) p.(Leu13Pro), p.Leu13Pro - likely pathogenic, NA, pathogenic g.18690151A>G g.18672031A>G Leu13Pro, RS1 c.38T>C, p.Leu13Pro - RS1_000109 1 more item PubMed: Hiriyanna 1999, PubMed: Kondo 2019, PubMed: Vijayasarathy 2010 - rs104894935 Germline, In vitro (cloned), Unknown ? - - - - Johan den Dunnen
-?/. 1 1 c.44G>A r.(?) p.(Gly15Asp) - likely benign g.18690145C>T g.18672025C>T RS1(NM_000330.3):c.44G>A (p.(Gly15Asp)) - RS1_000244 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/., +?/. 2 1_1i c.48_52+2dup r.spl, r.spl? p.(?), p.0? ACMG likely pathogenic, pathogenic g.18690135_18690141dup g.18672015_18672021dup c.52+2_3insTGAAGGT, RS1 c.52+2_3 ins tgaaggt - RS1_000404 error in annotation, variant automapped to c.48_52+2dup, 1 more item PubMed: Chen 2014, PubMed: Chen 2020 - - Germline, Unknown ?, yes 1/90 - - - LOVD
+?/. 1 1 c.49G>T r.(?) p.(Glu17Ter) - likely pathogenic g.18690140C>A g.18672020C>A RS1 c.49G>T, p.Glu17* - RS1_000406 - PubMed: Kondo 2019 - - Unknown ? - - - - LOVD
+/., +?/. 4 1 c.52G>A r.(?), r.spl p.(Ala18Thr), p.0?, p.? - likely pathogenic (recessive), pathogenic (recessive) g.18690137C>T g.18672017C>T RS1 c.673C>T, p.Arg213Trp - RS1_000322 in vitro analysis mini-gene splicing assay shows splicing is affected PubMed: Hahn 2022, PubMed: Vijayasarathy 2010, PubMed: Wei 2024 - - Germline yes - - - - Johan den Dunnen
+/. 8 1i c.52+1G>A r.(?), r.spl p.? ACMG pathogenic, pathogenic (recessive) g.18690136C>T g.18672016C>T - - RS1_000013 ACMG PVS1, PS4, PM2, PP4 PubMed: Bender 2022, PubMed: Mashima 1999, PubMed: RS-consortium 1998, group 2, PubMed: Shinoda 2000, 1 more item - - Germline, Germline/De novo (untested) - - +MboII - - Johan den Dunnen
+/. 2 1 c.52+1G>C r.(?), r.spl p.? ACMG pathogenic, pathogenic (recessive) g.18690136C>G g.18672016C>G RS1 c.52+1g>c - RS1_000405 number of alleles does not match number of patients (91 vs 90) PubMed: Chen 2020, PubMed: Jimenez 2022 - - Germline, Unknown ? 1/90 - - - Johan den Dunnen
+/., ?/. 6 1, 1i c.52+1G>T r.(?), r.spl p.? ACMG pathogenic, pathogenic (recessive), VUS g.18690136C>A g.18672016C>A RS1 c.52+1g>t - RS1_000014 ACMG PVS1, PM2, PP3, PP4, ACMG PVS1, PM2, PP4, 1 more item PubMed: Bender 2022, PubMed: Chen 2020, PubMed: Georgiou 2022, PubMed: Lamey 2010, 1 more item - rs281865336 Germline, Unknown ? 1/90 - - - Johan den Dunnen
+/. 1 1i c.52+2dup r.spl? p.? - pathogenic g.18690135dup g.18672015dup - - RS1_000015 - PubMed: RS-consortium 1998, group 3 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.52+2T>A r.spl p.? - pathogenic (recessive) g.18690135A>T g.18672015A>T - - RS1_000510 - PubMed: Gao 2021 - - Germline - - - - - Johan den Dunnen
+/., +?/. 10 1, 1i c.52+2T>C r.spl p.(?), p.? - likely pathogenic, pathogenic, pathogenic (recessive) g.18690135A>G g.18672015A>G c.52+2T>C, IVS1+2 T to C, RS1 c.52+2T>C, RS1 c.52+2T>C, p.?, Splice site IVS1+2T>C - RS1_000128 ACMG PVS1, PM2, PP3, PP4 PubMed: Fahim 2017, PubMed: Georgiou 2022, PubMed: Khan 2001, PubMed: Li 2007, PubMed: Ma 2008, 5 more items - rs281865334 Germline, Unknown ? - - - - Johan den Dunnen, Dorothy Trump
+?/., ?/. 4 1i c.52+3A>G r.spl? p.(?), p.? - likely pathogenic, VUS g.18690134T>C g.18672014T>C Allele 1 c.52+3A>G, Allele 2 not applicable - RS1_000243 hemizygous, VKGL data sharing initiative Nederland PubMed: Khan 2019 - - CLASSIFICATION record, Germline/De novo (untested) ? - - - - VKGL-NL_Nijmegen
?/. 6 1i c.52+5G>C r.(?), r.spl? p.(=), p.? ACMG VUS g.18690132C>G, g.52515354A>G g.18672012C>G, g.51941218A>G - - ATP7B_000001, RS1_000017 ACMG PS4, PM2, PP4, PP3, BP2 PubMed: Bender 2022, PubMed: RS-consortium 1998, group 3 - - Germline - - - - - Gerard C.P. Schaafsma, Johan den Dunnen
+/. 1 1i_2i c.53-859_78+276del r.(53_78del) p.(Ala18Glyfs*2) - pathogenic (recessive) g.18675485_18676645del g.18657365_18658525del (A18Pfs*108) - RS1_000484 - PubMed: Jimenez 2022, PubMed: Wen 2023 - - Germline - - - - - Johan den Dunnen
+?/. 1 1i_2i c.53-713_78+266del r.(?) p.(Ala18Glyfs*2) - likely pathogenic g.18675494_18676498del g.18657374_18658378del c.53-713_78+266del - RS1_000301 - PubMed: Maggi_2021 - - Germline yes - - - - LOVD
+/., +?/. 4 1i c.53-34A>G r.(53_78del), r.(?), r.spl p.(=), p.(Ala18fs), p.? ACMG likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.18675819T>C g.18657699T>C IVS1-34A>G - RS1_000179 ACMG PM1, PM2, PP4, ACMG PP1>S, PS4, PS3-PM, PM2, PP3, PP4 PubMed: Bender 2022, PubMed: Georgiou 2022, PubMed: Gerth 2008 - - Germline - - - - - Johan den Dunnen
+/. 2 - c.53-1G>A r.spl p.? - pathogenic (recessive) g.18675786C>T g.18657666C>T - - RS1_000479 - PubMed: Wang 2022, PubMed: Wei 2024 - - Germline yes - - - - Johan den Dunnen
+/., +?/., ?/. 33 1i_2i c.(52+1_53-1)_(78+1_79-1)del r.(53_78del), r.(?), r.? p.(?), p.(Ala18Glyfs*2), p.(Ala18GlyfsTer2), p.? ACMG likely pathogenic, pathogenic, pathogenic (recessive), VUS g.(18674879_18675759)_(18675786_18690136)del g.(18656759_18657639)_(18657666_18672016)del arr[hg19] Xp22.13 (18674960 x1, 18675273-18681809 x0, 18681810 x1) mat; c.53-?_78+?del, del ex2, 5 more items - RS1_000018 ACMG PVS1, PS4, PM2, PP4, no amplification exon 2 , 1 more item PubMed: Abalem 2018, PubMed: Bender 2022, PubMed: Georgiou 2022, PubMed: Hewitt 2005, PubMed: Wei 2024, 6 more items - - Germline, Unknown ?, yes - - - - Johan den Dunnen
+/., +?/. 6 1i_3i c.(52+1_53-1)_(184+1_185-1)del r.(53_184del), r.(?), r.? p.(?), p.(Ala18Pro61del), p.? ACMG likely pathogenic, pathogenic (recessive), VUS g.(18665453_18674772)_(18675786_18690136)del c.(52+1_53-1)_(184+1_185-1)del, g.(18647333_18656652)_(18657666_18672016)del del ex2-3, RS1 Deletion of exons 2 and 3, RS1 deletion of exons 2, 3, RS1 exon2-3 del - RS1_000424 deletion of exons 2, 3 PubMed: Fahim 2017, PubMed: Georgiou 2022, PubMed: Kondo 2019, PubMed: Ores 2018, PubMed: Wei 2024, 1 more item - - Germline, Unknown ? - - - - Johan den Dunnen
+?/. 2 _1_4i c.(?_-35)_(326+1_327-1)del r.0? p.0? - likely pathogenic g.(18662746_18665310)_(18690223_?)del g.(18644626_18647190)_(18672103_?)del RS1 deletion of exons 1, 2, 3, 4 - RS1_000427 deletion of exons 1, 2, 3, 4 PubMed: Ores 2018 - - Unknown ? - - - - LOVD
+?/. 1 1i_5i c.(52+1_53-1)_(522+1_523-1)del r.? p.? - likely pathogenic g.(18660277_18662549)_(18675786_18690136)del g.(18642157_18644429)_(18657666_18672016)del RS1 deletion of exons 2, 3, 4, 5 - RS1_000426 deletion of exons 2, 3, 4, 5 PubMed: Ores 2018 - - Unknown ? - - - - LOVD
+?/. 1 2_3 c.(53_184)delN[?] r.? p.? - likely pathogenic g.(18674775_18675787)delN[?] g.(18656655_18657667)delN[?] deletion in ex2 and/or ex3 - RS1_000000 - PubMed: Khan 2001 - - Germline/De novo (untested) ? - - - - LOVD
+/. 2 2 c.61G>T r.(?) p.(Gly21*) - pathogenic g.18675777C>A g.18657657C>A - - RS1_000137 - PubMed: Hewitt 2005 - - Germline - - +DraIII - - Johan den Dunnen
+/. 2 2 c.68C>A r.(?) p.(Ser23*), p.(Ser23Ter) ACMG pathogenic, pathogenic (recessive) g.18675770G>T g.18657650G>T - - RS1_000019 ACMG PVS1, PM2, PP4 PubMed: Bender 2022, PubMed: RS-consortium 1998, group 4, 5 - - Germline - - - - - Johan den Dunnen
+/. 1 2 c.76G>T r.(?) p.(Glu26*) - pathogenic g.18675762C>A g.18657642C>A - - RS1_000020 - PubMed: RS-consortium 1998, group 4 - - Germline - - +MaeI - - Johan den Dunnen
+/., +?/. 4 2 c.78G>C r.(?) p.(Glu26Asp) - likely pathogenic (recessive), pathogenic g.18675760C>G g.18657640C>G - - RS1_000142 ACMG PVS1, PM2, PP2, PP3, PP4 PubMed: Georgiou 2022, PubMed: Pimenides 2005 - - Germline - - +MaeI - - Johan den Dunnen
+/., +?/. 2 2i c.78+1G>A r.spl p.(?), p.? ACMG likely pathogenic, pathogenic g.18675759C>T g.18657639C>T RS1 c.78+1G>A, p.(?) - RS1_000152 hemizygous PubMed: Inoue 2002, PubMed: Kim 2021 - - Germline, Unknown ? - - - - Johan den Dunnen
+/. 1 2i c.78+1G>C r.spl p.? - pathogenic g.18675759C>G g.18657639C>G - - RS1_000155 - PubMed: Lesch 2008, PubMed: Lesch 2008 - - Germline - - +AluI - - Viktoria Szabo
+/., +?/. 6 2i c.78+1G>T r.(?), r.spl p.(?), p.? - likely pathogenic, pathogenic, pathogenic (recessive) g.18675759C>A g.18657639C>A c.78+1G>T, RS1 c.78+1G>T , p.? - RS1_000169 not in 108 control chromosomes PubMed: Kim 2009;Kim, ASHG2007 P1124, PubMed: Lee 2025, PubMed: Sudha 2017, PubMed: Sudha 2018 - - Germline, Unknown ?, yes - - - - Johan den Dunnen
+?/. 1 2i c.78+2T>C r.spl p.(?) - likely pathogenic g.18675758A>G g.18657638A>G RS1 c.78+2T>C, undetermined splicing defect - RS1_000403 - PubMed: Kondo 2019 - - Unknown ? - - - - LOVD
+/., ?/. 2 2i c.78+5G>A r.spl? p.? ACMG pathogenic, VUS g.18675755C>T g.18657635C>T - - RS1_000170 ACMG PM2, PP4, not in 108 control chromosomes PubMed: Bender 2022, PubMed: Kim 2009;Kim, ASHG2007 P1124 - - Germline - - - - - Johan den Dunnen, Viktoria Szabo
+/., +?/. 3 2 c.78+5G>T r.(?), r.spl, r.spl? p.(?), p.? ACMG likely pathogenic, pathogenic (recessive) g.18675755C>A g.18657635C>A c.78+5G>T, RS1 c.78+5G>T - RS1_000402 number of alleles does not match number of patients (91 vs 90) PubMed: Chen 2014, PubMed: Chen 2020, PubMed: Gao 2021 - - Germline, Unknown ?, yes 1/90 - - - Johan den Dunnen
+/. 1 2i c.78+5_78+7delinsTTT r.spl p.? - pathogenic (recessive) g.18675753_18675755delinsAAA g.18657633_18657635delinsAAA - - RS1_000478 - PubMed: Hahn 2022 - - Germline - - - - - Johan den Dunnen
?/. 1 2i c.78+380T>C r.(=) p.(=) - VUS g.18675380A>G g.18657260A>G - - RS1_000209 - - - - Germline - - - - - Yu Sun
+/. 3 2i c.79-2A>G r.spl p.? - pathogenic g.18674880T>C g.18656760T>C - - RS1_000001 - PubMed: RS-consortium 1998, group W, PubMed: RS-consortium 1998; group 6, PubMed: Pimenides 2005, 1 more item - - Germline - - -PstI - - Johan den Dunnen, Dorothy Trump
+/., +?/. 12 2i_3i c.(78+1_79-1)_(184+1_185-1)del r.(79_184del), r.(?), r.spl p.(?), p.(Asp27Asnfs*64), p.(Asp27fs) - likely pathogenic, pathogenic, pathogenic (recessive) g.(18665453_18674772)_(18674879_18675759)del g.(18647333_18656652)_(18656759_18657639)del del ex3, RS1 exon3 del, RS1, variant 1 :Deletion exon 3 - RS1_000021 no amplification exon 3 , solved, hemizygous PubMed: Hahn 2022, PubMed: Hewitt 2005, PubMed: Kondo 2019, PubMed: RS-consortium 1998, group 1, 3 more items - - Germline, Unknown ?, yes - - - - Johan den Dunnen
-?/., ?/. 2 3 c.87C>T r.(?) p.(Gly29=) - likely benign, VUS g.18674870G>A - RS1(NM_000330.3):c.87C>T (p.G29=) - RS1_000270 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
+/. 1 - c.96del r.(?) p.(Trp33GlyfsTer93) - pathogenic (recessive) g.18674864del g.18656744del 96delC - RS1_000477 - PubMed: Wei 2024 - - Germline - - - - - Johan den Dunnen
+/., +?/. 5 3 c.96dup r.(?) p.(Trp33Leufs*53), p.(Trp33LeufsTer53) ACMG likely pathogenic, pathogenic, pathogenic (recessive) g.18674864dup g.18656744dup 92_96insC, RS1 c.(92_97)insC, (p.W33fs), RS1 c.96dup, p.(Trp33Leufs*53) - RS1_000211 ACMG PVS1, PS4, PM2, PP4, error in annotation, variant automapped to c.96dup Kucinskas, ASHG2017, P1110, PubMed: Bender 2022, PubMed: Ores 2018, PubMed: Strupaite 2018 - - Germline, Unknown ?, yes - - - - Johan den Dunnen
+?/. 1 - c.96dupC r.(?) p.(Trp33Leufs*53) - likely pathogenic g.18674864dup g.18656744dup c.96dupC, p.Trp33Leu*53 - RS1_000211 - PubMed: Pennesi 2018 - - Unknown ? - - - - LOVD
+/., +?/. 11 3 c.97del r.(?) p.(Trp33Glyfs*93), p.(Trp33GlyfsTer93) ACMG likely pathogenic, pathogenic (recessive) g.18674860del g.18656740del 97delT, c.97delT, RS1 c.97del, p.(Trp33Glyfs*93) - RS1_000261 ACMG PVS1, PM2, PP4, no protein change given PubMed: Bender 2022, PubMed: Gao 2021, PubMed: Holtan 2020, PubMed: Lai 2015, PubMed: Lee 2025, 1 more item - - Germline, Unknown ?, yes 1/899 cases - - - Global Variome, with Curator vacancy, Johan den Dunnen
+/., +?/. 3 3 c.98G>A r.(?) p.(Trp33*), p.(Trp33Ter) ACMG likely pathogenic, pathogenic g.18674859C>T g.18656739C>T c.98G>A, p.W33X, RS1 c.98G>A, p.Trp33*, RS1 c.98G>A, p.W33X - RS1_000401 number of alleles does not match number of patients (91 vs 90) PubMed: Chen 2014, PubMed: Chen 2020, PubMed: Kondo 2019 - - Germline, Unknown ?, yes 1/90 - - - LOVD
+/., +?/. 3 3 c.99G>A r.(?) p.(Trp33*), p.(Trp33Ter) ACMG likely pathogenic, pathogenic (recessive) g.18674858C>T g.18656738C>T c.99G>A, p.Trp33* - RS1_000400 ACMG PVS1, PS4, PM2, PP4 PubMed: Bender 2022, PubMed: Pennesi 2018 - - Germline, Unknown ? - - - - Johan den Dunnen
+/., +?/. 4 3 c.103C>T r.(?) p.(Gln35*), p.(Gln35Ter) ACMG likely pathogenic, pathogenic, pathogenic (recessive) g.18674854G>A g.18656734G>A Substitution 103C>T, p. Gln35X - RS1_000138 ACMG PVS1, PM2, PP3, ACMG PVS1, PM2, PP4 PubMed: Bender 2022, PubMed: Georgiou 2022, PubMed: Hewitt 2005, PubMed: Sergeev 2013 - - Germline, Unknown ? - -KpnI - - Johan den Dunnen
+/. 1 3 c.108del r.(?) p.(Ala37Hisfs*89) - pathogenic g.18674853del g.18656733del c.108del, p.(Ala37Hisfs*89) - RS1_000295 error in annotation: c.108del instead of, hemizygous PubMed: Wang 2019 - - Germline ? - - - - LOVD
+/., +?/. 15 3 c.120C>A r.(?) p.(Cys40*), p.(Cys40Ter) ACMG likely pathogenic, pathogenic, pathogenic (recessive) g.18674837G>T g.18656717G>T RS1 c.120C>A, p.(Cys40X) - RS1_000002 ACMG PVS1, PM2, PP4, BP4, ACMG PVS1, PS4, PM2, PP4 PubMed: Bender 2022, PubMed: Eksandh 2000, PubMed: Fahim 2017, PubMed: Georgiou 2022, 8 more items - rs62645885 Germline, Unknown ? - - - - Johan den Dunnen, Dorothy Trump
+?/. 1 - c.127C>T r.(?) p.(Gln43Ter) - likely pathogenic g.18674830G>A - - - RS1_000432 - - - - CLASSIFICATION record - - - - - MobiDetails
+/. 1 3 c.130_140del r.(?) p.(Gly44CysfsTer38) - pathogenic (recessive) g.18674821_18674831del g.18656701_18656711del - - RS1_000476 - PubMed: Lee 2025 - - Germline - - - - - Johan den Dunnen
+/. 1 3 c.140dup r.(?) p.(Asn47Lysfs*39) - pathogenic g.18674818dup g.18656698dup c.140dup, p.(Asn47Lysfs*39) - RS1_000294 error in annotation: c.140dup instead of, hemizygous PubMed: Wang 2019 - - Germline yes - - - - LOVD
+/. 2 3 c.149G>A r.(?) p.(Trp50*), p.(Trp50Ter) - pathogenic, pathogenic (recessive) g.18674808C>T g.18656688C>T - - RS1_000135 - PubMed: Eksandh 2005, PubMed: Fortunato 2023 - - Germline - - +AciI - - Johan den Dunnen
+/., +?/. 2 3 c.150G>A r.(?) p.(Trp50*), p.(Trp50Ter) - likely pathogenic, pathogenic (recessive) g.18674807C>T g.18656687C>T c.150G>A - RS1_000300 - PubMed: Lee 2025, PubMed: Maggi_2021 - - Germline - - - - - Johan den Dunnen
+/. 1 3 c.160_163dup r.(?) p.(Thr55Serfs*32) - pathogenic g.18674794_18674797dup g.18656674_18656677dup - - RS1_000122 - PubMed: Hiraoka 2000 - - Germline - - - - - Johan den Dunnen
+/. 2 3 c.163del r.(?) p.(Thr55Profs*71), p.(Thr55ProfsTer71) ACMG pathogenic, pathogenic (recessive) g.18674794del g.18656674del - - RS1_000101 ACMG PVS1, PM2, PP4 PubMed: Bender 2022, PubMed: Gehrig 1999 - - Germline - - -BstXI +SduI - - Johan den Dunnen
+/. 1 3 c.173_184+21del r.spl p.? - pathogenic g.18674759_18674791del g.18656639_18656671del - - RS1_000107 - PubMed: Shinoda 2000 - - Germline - - - - - Johan den Dunnen
+/. 5 3 c.175T>A r.(?) p.(Cys59Ser) - NA, pathogenic g.18674782A>T g.18656662A>T - - RS1_000022 expression construct in pcDNA3.1, COS-7 cell expression PubMed: Gehrig 1999, PubMed: Renner 2008, PubMed: RS-consortium 1998, group 2, PubMed: Wang 2002, 1 more item - - De novo, Germline, In vitro (cloned) - - - - - Johan den Dunnen
+?/. 6 3 c.175T>G r.(?) p.(Cys59Gly) - likely pathogenic g.18674782A>C g.18656662A>C c.175A>C, p.(Cys59Gly), RS1 c.175T>G, p.Cys59Gly - RS1_000293 hemizygous PubMed: Kondo 2019, PubMed: Wang 2019 - - Germline yes - - - - LOVD
+/., +?/. 5 3 c.176G>A r.(?) p.(Cys59Tyr) ACMG likely pathogenic (recessive), pathogenic g.18674781C>T g.18656661C>T RS1 176G>A, Cys59Tyr, RS1 c.176G>A , p. C59Y - RS1_000320 ACMG PM1, PM2, PM5, PP1, PP2, PP3, PP4, PP5, 1 more item PubMed: Bender 2022, PubMed: Chen 2020, PubMed: Yi 2012 - - Germline, Unknown ? 1/90 - - - Johan den Dunnen
+/., +?/. 3 3 c.176G>C r.(?) p.(Cys59Ser) ACMG likely pathogenic, likely pathogenic (recessive) g.18674781C>G g.18656661C>G RS1 c.176G>C (p.Cys59Ser) - RS1_000321 ACMG PS3, PM2, PP3, PP4, PP PubMed: Bender 2022, PubMed: Skorczyk 2012 - - Germline, Unknown ? - - - - Johan den Dunnen
+/. 1 3_3i c.183_184+12del r.spl p.? - pathogenic g.18674763_18674776del g.18656643_18656656del 181-184+10del - RS1_000423 - PubMed: RS-consortium 1998, group 5 - - Germline - - - - - Johan den Dunnen
+/., +?/. 3 3i, 3_3i c.184+1G>C r.spl p.(?), p.? ACMG likely pathogenic, pathogenic, pathogenic (recessive) g.18674772C>G g.18656652C>G IVS3+1G to C - RS1_000399 ACMG PVS1, PM2, PP4 PubMed: Bender 2022, PubMed: Khan 2001, PubMed: RS-consortium 1998, group 3 - - Germline, Unknown ? - - - - Johan den Dunnen
+/., +?/. 3 3i c.184+2T>G r.spl p.(?), p.? - likely pathogenic, pathogenic (recessive) g.18674771A>C g.18656651A>C RS1 c.184+2T>G, p.?, Splice site 184+2T>G - RS1_000398 ACMG PVS1, PM2, PP3, PP4, PP5 PubMed: Fahim 2017, PubMed: Georgiou 2022, PubMed: Sergeev 2013 - rs1555959367 Germline, Unknown ? - - - - Johan den Dunnen
-/., ?/. 4 3i c.184+35T>C r.(=) p.(=) - benign, VUS g.18674738A>G g.18656618A>G 184+35C>T - RS1_000157 - PubMed: Kim 2009;Kim, ASHG2007 P1124, PubMed: Kjellstrom 2010 - rs6633109 Germline - 0.07 - - - Johan den Dunnen
-/. 2 3i c.184+129T>G r.(=) p.(=) - benign g.18674644A>C g.18656524A>C 184+129G>T - RS1_000171 - PubMed: Kim 2009;Kim, ASHG2007 P1124 - - Germline - - - - - Johan den Dunnen
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