Unique variants in the ABCA4 gene


The expert classification of the variants can be found here.

To other Inherited Retinal Disease gene variant database:
Information The variants shown are described using the NM_000350.2 transcript reference sequence.

2868 entries on 29 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 17i_50 c.(2653+1_2654-1)_(*1_?)del r.? p.? ACMG VUS g.(?_94458792)_(94514514_94517188)del g.(?_93993236)_(94048958_94051632)del - - ABCA4_002890 ACMG PVS1_S, PM2_sup; severity category not assesed Journal: Cornelis 2023 - - SUMMARY record - - - - - Stéphanie Cornelis
?/. 1 22i_50_ c.(3328+1_3523-1)_*400{0} r.? p.? - VUS g.(?_94458393)_(94505684_94508316)del g.(?_93992837)_(94040128_94042760)del - - ABCA4_002595 - PubMed: Cornelis 2024, Journal: Cornelis 2024 - - Unknown - - - - - Frans Cremers
+?/. 1 48i_50_ c.(6729+1_6730-1)_(*1_?)del r.? p.? ACMG likely pathogenic g.(?_94458792)_(94461752_94463416)del g.(?_93993236)_(93996196_93997860)del - - NPHS2_000000 ACMG PVS1_S, PM2_sup, PM3_sup; severity category severe Journal: Cornelis 2023 - - SUMMARY record - - - - - Stéphanie Cornelis
?/. 1 49i_50_ c.(6816+1_6817-1)_(*1_?)del r.? p.? ACMG VUS g.(?_94458792)_(94458799_94461664)del g.(?_93993236)_(93993243_93996108)del - - ABCA4_002889 ACMG PVS1_S, PM2_sup; severity category not assesed Journal: Cornelis 2023 - - SUMMARY record - - - - - Stéphanie Cornelis
?/. 1 44 c.*10C>A r.(?) p.? ACMG VUS g.94458783G>T g.93993227G>T - - ABCA4_000874 ACMG PM3, PP3_m; severity category mild/moderate Journal: Cornelis 2023 - - SUMMARY record - - - - - Stéphanie Cornelis
?/. 1 5 c.*55G>A r.(?) p.? ACMG VUS g.94458738C>T g.93993182C>T - - ABCA4_001553 ACMG PM2_sup, PM3_sup, PP3_m; severity category benign Journal: Cornelis 2023 - - SUMMARY record - - - - - Stéphanie Cornelis
?/. 1 _1_1i c.-104_(66+1_67-1){0} r.0? p.0? - VUS g.(94578623_94586535)_(94586602_?)del g.(94113067_94120979)_(94121046_?)del - - ABCA4_002758 - PubMed: Cornelis 2024, Journal: Cornelis 2024 - - Germline yes - - - - Frans Cremers
+?/. 1 1_40i c.-104_5714+250{0} r.0? p.0? ACMG likely pathogenic g.94476106_94586955del g.94010550_94121399del c.-354_5714+250del - ABCA4_002291 ACMG PVS1, PM2_sup; severity category not assesed Journal: Cornelis 2023 - - SUMMARY record - - - - - Stéphanie Cornelis
+?/. 1 24_50 c.3525_*400{0} r.? p.? ACMG likely pathogenic g.94402743_94505682del g.93937187_94040126del c.3524_*56050del - ABCA4_002888 ACMG PVS1, PM2_sup; severity category not assesed Journal: Cornelis 2023 - - SUMMARY record - - - - - Stéphanie Cornelis
+/., +?/. 2 39i, 39i_50_ c.5585-164_*400{0} r.(?), r.? p.(?), p.? ACMG likely pathogenic, pathogenic (recessive) g.94457537_94476649del, g.94457539_94476651del g.93991981_94011093del, g.93991983_94011095del c.5585-164_*1256del, [5585-166_*1254del] - ABCA4_001552 ACMG PVS1, PM2_sup; severity category not assesed, no segregation analysis done Journal: Cornelis 2023, PubMed: Bauwens 2019 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
./. 1 - c.-4263834_*647310del r.? p.? - pathogenic g.93811483_98850435del - - - DPYD_000017 decreased gene dosage PubMed: DDDS 2015, Journal: DDDS 2015 - - De novo - - - - - Johan den Dunnen
-?/., ?/. 2 1 c.-92C>T r.(?) p.(?) ACMG likely benign, VUS g.94586693G>A g.94121137G>A c.-92C>T, Heterozygous - ABCA4_002258 ACMG PM2_sup, BP4_m; severity category not assesed Journal: Cornelis 2023, PubMed: Goetz 2020 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
-?/., ?/. 2 1 c.-14G>A r.(?) p.(?) ACMG likely benign, VUS g.94586615C>T g.94121059C>T c.2588G>C p.Gly863Ala - ABCA4_002257 ACMG PM2_sup, BP4_m; severity category not assesed Journal: Cornelis 2023, PubMed: Georgiou 2019 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
+?/. 1 _1_24 c.(?_-1)_3525del r.0? p.0? ACMG likely pathogenic g.94505681_(94586602_?)del g.94040125_(94121046_?)del - - ABCA4_002897 ACMG PVS1, PM2_sup; severity category not assesed Journal: Cornelis 2023 - - SUMMARY record - - - - - Stéphanie Cornelis
+?/. 1 _1_50_ c.-104_*400{0} r.0 p.0 - likely pathogenic g.(?_94458792)_(94586602_?)del g.(?_93993236)_(94121046_?)del c.(?_1)_(6822_?)del - ABCA4_000000 deletion encompassing region flanked by extragenic markers D1S435 and D1S2793 PubMed: Valverde 2006 - - Germline - - - - - Stéphanie Cornelis
+/. 1 14i_17i c.(2160+1_2161-1)_(2653+1_2654-1) r.(?) p.(?) - pathogenic (recessive) g.(94514514_94517188)_(94522379_94526092)del g.(94048958_94051632)_(94056823_94060536)del del ex15-17 - ABCA4_002591 - PubMed: Goetz 2020 - - Unknown - - - - - Stéphanie Cornelis
+/. 2 17i_50_ c.(2653+1_2654-1)_*400{0} r.? p.(Gly885Valfs*71), p.? ACMG pathogenic, pathogenic (recessive) g.(?_94458393)_(94514514_94517188)del g.(?_93992837)_(94048958_94051632)del c.(2653+1_2654-1)_(*1_?)del - ABCA4_002592 - PubMed: Khan 2020, PubMed: Tracewska 2019 - - Germline, Unknown yes 0 (in-house database, ~5000 samples) - - - Anna Tracewska
+?/. 1 23i_50_ c.(3522+1_3523-1)_*400{0} r.? p.? - likely pathogenic g.(?_94402743)_(94505684_94506764)del g.(?_93937187)_(94040128_94041208)del chr1:g.94402743_94505682del - ABCA4_002761 heterozygous PubMed: Turro 2020 - - Germline/De novo (untested) ? - - - - LOVD
+/. 1 49i_50_ c.(6817-1_6818+1)_*400{0} r.? p.? - pathogenic (recessive) g.(?_94458393)_(94458796_94458799)del g.(?_93992837)_93993240_93993243)del c.(6816+1_6817-1)_(*1_?)del# - ABCA4_002590 - PubMed: Jespersgaard 2019 - - Unknown - - - - - Stéphanie Cornelis
+?/. 1 - c.0? r.0? p.(?) - likely pathogenic g.94476106_94586955del g.94010550_94121399del ABCA4 chr1:94476106_94586955del - ABCA4_002291 range 110244-110849 bp in various techniques, heterozygous PubMed: Zampaglione 2020 - - Unknown ? - - - - LOVD
+/., +?/., -?/., ?/. 15 13, 23, 23i_50_, 36, 38, ? c.? r.(?), r.? p.(?), p.(W439*), p.? ACMG likely benign, likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), 1 more item g.94452244_94471761del, g.94528142C>T, g.94586187_94587411del, g.? - 11-basepair deletion in exon 38, 3896G>T intronic, 5226delC, c.1928G>A, c.3385C>T, D654N, 7 more items - ABCA4_000000, NPHS2_000000 ACMG PM2, PVS1, no variant 2nd chromosome PubMed: Birch 2001, PubMed: Carss 2017, PubMed: Cideciyan 2009, PubMed: Downs 2007, PubMed: Oh 2004, 8 more items - rs114572202 Germline, Unknown ? - - - - Johan den Dunnen, Julia Lopez, Stéphanie Cornelis
+/., +?/. 41 1 c.1A>G r.(1a>g), r.(?), r.? p.(Met1?), p.(Met1Val), p.0?, p.? ACMG likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.94586601T>C g.94121045T>C 1A>G, c.1A>G, c.1A>G (p.?), c.1A>G p.(M1?), c.1A>G p.(Met1?), c.1A>G p.Met1? het, 16 more items - ABCA4_000264 compound heterozygous, Homozygous, no segregation analysis done, no variant 2nd chromosome, 2 more items Bianco 2023, submitted, Prevention Genetics, Sharon, submitted, PubMed: Burke 2010, PubMed: Cai 2018, 23 more items - - CLASSIFICATION record, Germline, Germline/De novo (untested), SUMMARY record, Unknown -, ?, yes 1/2420 IRD families - - - Global Variome, with Curator vacancy, Dror Sharon, Frans Cremers, Stéphanie Cornelis, VKGL-NL_Nijmegen, Rebekkah Hitti-Malin, Lorenzo Bianco, Zelia Corradi
+/., ?/. 2 1 c.1_3delinsGTC r.(?) p.(Met1?), p.0? ACMG pathogenic, VUS g.94586599_94586601delinsGAC g.94121043_94121045delinsGAC ATG>GTC - ABCA4_000262 - PubMed: Briggs 2001, PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023 - - Germline, SUMMARY record - - - - - Stéphanie Cornelis
+?/. 1 _1_50_ c.-104_*400{0} r.0 p.0 ACMG likely pathogenic (recessive) g.(?_94458792)_(94586602_?)del g.(?_93993236)_(94121046_?)del del gene, c.(?_-1)_(*1_?)del - ABCA4_000862 deletion entire ABCA4 gene PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023 - - SUMMARY record - - - - - Stéphanie Cornelis
+/., +?/. 5 1 c.2T>C r.(2u>c), r.(?) p.(=,?), p.(Met1?), p.0? ACMG likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.94586600A>G g.94121044A>G c.2T<C, Codon 1 ATG-ACG Met-Thr, M1T - ABCA4_000263 case unsolved, no variant 2nd chromosome PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, PubMed: Maia-Lopes 2008, 3 more items - - Germline, SUMMARY record, Unknown - 2, 121346, 0, 0.00001648 - - - Johan den Dunnen, Stéphanie Cornelis
+/., +?/. 3 1 c.3G>A r.(3g>a), r.(?) p.(Met1?), p.(Met1Ile), p.0? ACMG likely pathogenic, likely pathogenic (recessive), pathogenic (recessive) g.94586599C>T g.94121043C>T c.3G>A - ABCA4_000261 no segregation analysis done, 2 more items PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, PubMed: Riveiro-Alvarez 2013, 1 more item - - Germline, SUMMARY record, Unknown ? 3, 121340, 0, 0.00002472 - - - Stéphanie Cornelis
+/., ?/. 2 1 c.3G>T r.(?) p.(?), p.(Met1?) ACMG pathogenic (recessive), VUS g.94586599C>A g.94121043C>A c.3G>T, Heterozygous - ABCA4_002256 ACMG PVS1_M, PM2_sup, PM5; severity category uncertain Journal: Cornelis 2023, PubMed: Goetz 2020 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
+/., +?/. 3 1 c.16C>T r.(?) p.(Gln6*), p.(Gln6Ter) ACMG likely pathogenic, pathogenic, pathogenic (recessive) g.94586586G>A g.94121030G>A c.16C>T, p.Gln6Ter - ABCA4_002255 ACMG PVS1, PM2_sup, PP3_m; severity category severe Journal: Cornelis 2023, PubMed: Fujinami 2019, PubMed: Hitti-Malin 2024, Journal: Hitti-Malin 2024 - - Germline, SUMMARY record, Unknown - - - - - Stéphanie Cornelis, Rebekkah Hitti-Malin
+?/. 1 - c.18_19del r.(?) p.(Gln6HisfsTer47) ACMG likely pathogenic (recessive) g.94586584_94586585del g.94121028_94121029del - - ABCA4_002370 - - - - Germline - - - - - Nancy Xilotl de Jesús
+?/., ?/. 2 1 c.20T>A r.(?) p.(Ile7Lys) ACMG likely pathogenic (recessive), VUS g.94586582A>T g.94121026A>T c.20T>A, p.Ile7Lys Heterozygous - ABCA4_002254 ACMG PM2_sup; severity category uncertain Journal: Cornelis 2023, PubMed: Goetz 2020 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
+/. 5 1 c.21dup r.(?) p.(Gln8Hisfs*33), p.(Gln8ThrfsTer46) ACMG pathogenic, pathogenic (recessive) g.94586581dup g.94121025dup c.20insA p.Q8fs, c.21_22insA,p.Gln8ThrfsTer46, p.[Gln8fs];c.[5461-10T>C] - ABCA4_002253 ACMG PVS1, PS4; severity category severe Journal: Cornelis 2023, PubMed: Cornelis 2024, Journal: Cornelis 2024, PubMed: Fakin 2016, 2 more items - - Germline/De novo (untested), SUMMARY record, Unknown yes - - - - Stéphanie Cornelis
+/., +?/. 5 1 c.22C>T r.(22c>u), r.(?) p.(Gln8*), p.(Gln8Ter) ACMG likely pathogenic, pathogenic, pathogenic (recessive) g.94586580G>A g.94121024G>A c.22C>T, c.22C>T p.(Gln8*) - ABCA4_000260 not statistically tested, classification unknown PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, PubMed: Dan 2019, 2 more items - - Germline, SUMMARY record, Unknown ?, yes - - - - Stéphanie Cornelis, Handong Dan, Lu Tian
+/. 3 1 c.29dup r.(?) p.(Leu10Phefs*44), p.(Leu10PhefsTer44) ACMG pathogenic, pathogenic (recessive) g.94586576dup g.94121020dup c.29_30insT p.Leu10PhefsTer44 - ABCA4_002252 ACMG PVS1, PM2_sup, PP3_m; severity category not assesed, no variant 2nd chromosome Journal: Cornelis 2023, PubMed: Cornelis 2024, Journal: Cornelis 2024, PubMed: Tanna 2019 - - Germline/De novo (untested), SUMMARY record, Unknown - - - - - Stéphanie Cornelis
+/., +?/., ?/. 15 1 c.32T>C r.(32u>c), r.(?) p.(Leu11Pro) ACMG likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS g.94586570A>G g.94121014A>G 32T>C, c.32T>C, c.32T>C p. Leu11Pro, c.32T>C p.(Leu11Pro), c.32T>C p.Leu11Pro het, 4 more items - ABCA4_000154 - Prevention Genetics, PubMed: Cideciyan 2009, PubMed: Cornelis 2024, Journal: Cornelis 2024, 9 more items - - Germline, SUMMARY record, Unknown -, yes 1, 121404, 0, 0.000008237, ExAC 1, 121404, 0, 0.000008237 - - - Frans Cremers, Stéphanie Cornelis
-?/., ?/. 2 1 c.33C>T r.(?) p.(Leu11=) ACMG likely benign, VUS g.94586569G>A g.94121013G>A c.33C>T p.Leu11Leu Het - ABCA4_002251 ACMG BP4_m, BP7; severity category not assesed, no variant 2nd chromosome Prevention Genetics, Journal: Cornelis 2023 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
+/. 2 1 c.36G>A r.(?) p.(Trp12*), p.(Trp12Ter) ACMG pathogenic, pathogenic (recessive) g.94586566C>T g.94121010C>T c.36G>A p.W12* - ABCA4_002250 ACMG PVS1, PM2_sup, PM3_sup, PP3_m; severity category severe Journal: Cornelis 2023, PubMed: Smaragda 2018 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
+?/., ?/. 2 1 c.37A>G r.(?) p.(Lys13Glu), p.(Lys13Gly) ACMG likely pathogenic (recessive), VUS g.94586565T>C g.94121009T>C c.37A>G, p.Lys13Glu Heterozygous - ABCA4_002249 ACMG PM2_sup, PP3_m; severity category not assesed Journal: Cornelis 2023, PubMed: Goetz 2020 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
?/. 3 1 c.38_46del r.(38_46del), r.(?) p.(Lys13_Trp15del) ACMG VUS g.94586561_94586569del g.94121005_94121013del Deletion Lys13-Trp15 - ABCA4_000258 - PubMed: Briggs 2001, PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023 - - Germline, SUMMARY record - - - - - Stéphanie Cornelis
+/., +?/., ?/. 5 1 c.42C>A r.(?) p.(Asn14Lys) ACMG likely pathogenic, likely pathogenic (recessive), pathogenic (recessive), VUS g.94586560G>T g.94121004G>T c.42C>A, p.N14K - ABCA4_001330 ACMG PS4, PM3, PP3; severity category mild/moderate, no segregation analysis done Journal: Cornelis 2023, PubMed: Hu 2019, PubMed: Jiang 2016, PubMed: Tian 2024 - - Germline, SUMMARY record, Unknown - - - - - Stéphanie Cornelis, Fangyuan Hu, Lu Tian
+/. 4 1 c.42_48delinsAG r.(?) p.(Asn14Lysfs*38), p.(Asn14LysfsTer38) ACMG pathogenic, pathogenic (recessive) g.94586554_94586560delinsCT g.94120998_94121004delinsCT c.42_48delinsAG p.Asn14Lysfs*38 het, c.42_48delinsAG, p.Asn14Lysfs*38 Heterozygous - ABCA4_002248 ACMG PVS1, PS4, PP3_m; severity category severe Prevention Genetics, Journal: Cornelis 2023, PubMed: Cornelis 2024, Journal: Cornelis 2024, 1 more item - - Germline/De novo (untested), SUMMARY record, Unknown - - - - - Stéphanie Cornelis
+/. 2 1 c.43_47del r.(43_47del), r.(?) p.(Trp15Profs*37), p.(Trp15ProfsTer37) ACMG pathogenic, pathogenic (recessive) g.94586557_94586561del g.94121001_94121005del c.43_48del6insC - ABCA4_000257 - PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, PubMed: Zhao 2015 - - Germline, SUMMARY record ? - - - - Stéphanie Cornelis
+/., +?/. 21 1 c.45G>A r.(45g>a), r.(?) p.(Trp15*), p.(Trp15Ter) ACMG likely pathogenic, pathogenic, pathogenic (recessive) g.94586557C>T g.94121001C>T c.45G>A, c.45G>A (p.Trp15*), c.45G>A p.(W15*), c.45G>A, p.Trp15Stop Heterozygous, p.Trp15*, 5 more items - ABCA4_000259 ACMG PM2, PVS1, PP5, no segregation analysis done, no variant 2nd chromosome PubMed: Birtel 2018, PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, 12 more items - - Germline, SUMMARY record, Unknown -, ?, yes - - - - Johan den Dunnen, Stéphanie Cornelis
+/. 2 1 c.46_47del r.(?) p.(Thr16Profs*37), p.(Thr16ProfsTer37) ACMG pathogenic, pathogenic (recessive) g.94586555_94586556del g.94120999_94121000del c.46_47del - ABCA4_002513 ACMG PVS1, PM2_sup, PP3_m; severity category not assesed Journal: Cornelis 2023, PubMed: Khan 2020 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
+/., +?/., ?/. 45 1 c.52C>T r.(52c>u), r.(?) p.(Arg18Trp) ACMG likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS g.94586550G>A g.94120994G>A 52C>T, Arg18Trp, C > T transition at nucleotide 52, c.52C> T, p.R18W, c.52C>T, c.52C>T, 14 more items - ABCA4_000037 ACMG PP3, PM2, PM5, PP2, PP5_STRONG, combination of variants not reported, no variant 2nd chromosome, 1 more item PubMed: Birtel 2018, PubMed: Braun 2013, PubMed: Bravo-Gil 2017, PubMed: Chacón-Camacho 2013, 24 more items 1048166 - Germline, Germline/De novo (untested), SUMMARY record, Unknown -, ?, yes 3, 121392, 0, 0.00002471, ExAC 3, 121392, 0, 0.00002471 - - - Johan den Dunnen, Frans Cremers, Stéphanie Cornelis, Nereida Bravo Gil, Zelia Corradi, Oscar Francisco Chacón Camacho
+/., +?/. 11 1 c.53G>A r.(?) p.(Arg18Gln) ACMG likely pathogenic, likely pathogenic (recessive), pathogenic (recessive) g.94586549C>T g.94120993C>T c.4773+1 G>Ac.53 G>A, c.53G>A, c.53G>A p.(Arg18Gln), c.53G>A p.Arg18Gln, 1 more item - ABCA4_001028 ACMG PS4, PM3_sup, PM5, PP3; severity category severe Journal: Cornelis 2023, PubMed: Cornelis 2024, Journal: Cornelis 2024, PubMed: Georgiou 2019, 5 more items - rs868543294 Germline, Germline/De novo (untested), SUMMARY record, Unknown yes - - - - Frans Cremers, Stéphanie Cornelis, Marco Nassisi, Lu Tian
+/., +?/., ?/. 17 1 c.53G>C r.(?) p.(Arg18Pro) ACMG likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS g.94586549C>G g.94120993C>G ABCA4 c.53G>C, p.R18P, c.53G>C, c.53G>C p.(Arg18Pro), p.R18P - ABCA4_001329 ACMG PS4, PM3_S, PM5, PP3_m; severity category moderate, 1 more item Zixi Sun 2020, submitted, Journal: Cornelis 2023, PubMed: Corradi 2023, Journal: Corradi 2023, 6 more items - - Germline, Germline/De novo (untested), SUMMARY record, Unknown ?, yes - - - - Stéphanie Cornelis, Fangyuan Hu, Zixi Sun, Lu Tian, Zelia Corradi
+/., +?/. 11 1 c.61C>T r.(61c>u), r.(?) p.(Gln21*), p.(Gln21Ter) ACMG likely pathogenic, pathogenic, pathogenic (recessive) g.94586541G>A g.94120985G>A c.61C>T, c.61C>T p.(Gln21*), c.61C>T; p.Gln21Ter, Het NM_000350.2: c.61C>T, ;Q21X - ABCA4_000256 VKGL data sharing initiative Nederland PubMed: Abed 2018, PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, 6 more items - - CLASSIFICATION record, Germline, SUMMARY record, Unknown -, yes 0 (in-house database, ~5000 samples), 1, 121388, 0, 0.000008238 - - - Stéphanie Cornelis, VKGL-NL_Nijmegen, Anna Tracewska, Zelia Corradi
+/., +?/., ?/. 9 1 c.66G>A r.(?), r.spl, r.spl? p.(=), p.(Lys22=), p.(Lys22=,?), p.[(Lys21=, ?)] ACMG likely pathogenic, pathogenic, pathogenic (recessive), VUS g.94586536C>T g.94120980C>T c.66G>A (p.?), c.66G>A, p.Lys22Lys Heterozygous, c.[66G4A;859-9T4C], c.[66G>A;859-9T>C], 2 more items - ABCA4_000255, ABCA4_000871 ACMG PS4, PM3_S, PP3_m; severity category mild/moderate, no variant 2nd chromosome Journal: Cornelis 2023, PubMed: Chen 2019, PubMed: Duncker 2015, PubMed: Gliem 2020, PubMed: Lee 2017, 4 more items - - Germline, SUMMARY record, Unknown -, yes 0 (in-house database, ~5000 samples) - - - Jana Zernant, Stéphanie Cornelis, Anna Tracewska
+?/., ?/. 5 1 c.66G>T r.(?), r.spl? p.(Lys22Asn), p.(Lys22Asn,?) ACMG likely pathogenic, likely pathogenic (recessive), VUS g.94586536C>A g.94120980C>A c.66G>T p.Lys22Asn, p.Lys22Ans - ABCA4_002247 ACMG PM2_sup, PP3; severity category not assesed Journal: Cornelis 2023, PubMed: Hitti-Malin 2024, Journal: Hitti-Malin 2024, PubMed: Salles 2017, 2 more items - - Germline, SUMMARY record, Unknown yes - - - - Stéphanie Cornelis, Rebekkah Hitti-Malin
?/. 2 1i c.66+3A>C r.spl? p.(?), p.? ACMG VUS g.94586533T>G g.94120977T>G c.66+3A>C - ABCA4_000254 1 more item PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, PubMed: Ritter 2013 - - Germline, SUMMARY record - 1, 121380, 0, 0.000008239 - - - Stéphanie Cornelis
+?/., ?/. 2 1i c.66+520_67-389dup r.(?) p.(?), p.? ACMG likely pathogenic (recessive), VUS g.94579013_94586018dup g.94113457_94120462dup [66+520_67-389dup ] - ABCA4_002246 ACMG PM2_sup; severity category uncertain, no segregation analysis done Journal: Cornelis 2023, PubMed: Bauwens 2019 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
-?/., ?/. 2 1i c.66+2044G>A r.spl? p.(?), p.? ACMG likely benign, VUS g.94584492C>T g.94118936C>T m36: c.66+2044G>A; p.? - ABCA4_002281 ACMG BP4_m; severity category not assesed Journal: Cornelis 2023, PubMed: Gonzalez del Pozo 2018 - - Germline, SUMMARY record yes - - - - Stéphanie Cornelis
+?/., ?/. 6 1i c.67-2023T>G r.(66_67ins67-2266_67-2024,=), r.[(66_67ins67-2266_67-2024,=)], r.[66_67ins67-2266_67-2024,=] p.(IIe23IIefs*30,=), p.[(IIe23IIefs*30,=)], p.[IIe23IIefs*30,=], p.[IIe23IIefsTer30,=] ACMG likely pathogenic (recessive), VUS g.94580645A>C g.94115089A>C c.67-2023T>G - ABCA4_002512 ACMG PS3_sup, PM2_sup, PM3, PP3; severity category moderate Khan 2020, Journal: Cornelis 2023, PubMed: Cornelis 2024, Journal: Cornelis 2024 - - Germline/De novo (untested), SUMMARY record, Unknown - - - - - Frans Cremers, Stéphanie Cornelis
+/., +?/. 2 1i, 1i_2i c.67-1845_160+345del r.(?) p.(?), p.? ACMG likely pathogenic, pathogenic (recessive) g.94578188_94580471del g.94112632_94114915del c.67-1845_c.160+345del (p.?) - ABCA4_002236 ACMG PVS1, PM2_sup; severity category severe, no segregation analysis done Journal: Cornelis 2023, PubMed: Zernant 2017 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
+/., ?/. 2 1i_6i c.67-975_769-4582dup r.(?) p.(?), p.? ACMG pathogenic (recessive), VUS g.94553580_94579598dup g.94088024_94114042dup [67-975_769-4582dup{insA}] - ABCA4_002166 ACMG PM2_sup, PM3, PM4; severity category severe Journal: Cornelis 2023, PubMed: Bauwens 2019 - - SUMMARY record, Unknown yes - - - - Stéphanie Cornelis
+/., +?/. 3 1i_2i c.67-140_161-355del r.(67_160del) p.(Ile23Alafs*24), p.(Ile23AlafsTer24) ACMG likely pathogenic, pathogenic, pathogenic (recessive) g.94577492_94578764del g.94111936_94113208del - - ABCA4_002315 ACMG PVS1, PM2_sup; severity category not assesed Journal: Cornelis 2023, PubMed: Tian 2022, PubMed: Tian 2022, PubMed: Tian 2024 - - Germline, SUMMARY record - - - - - Stéphanie Cornelis, Lu Tian
+/., +?/., ?/. 3 1i c.67-16T>A r.spl, r.spl? p.(?), p.(Ile23AlafsTer24), p.? ACMG likely pathogenic (recessive), pathogenic (recessive), VUS g.94578638A>T g.94113082A>T c.67-16T>A - ABCA4_002245 ACMG PM2_sup, PM3, PP3; severity category uncertain, no segregation analysis done Journal: Cornelis 2023, PubMed: Jiang 2016, PubMed: Tian 2024 - - Germline, SUMMARY record, Unknown yes - - - - Stéphanie Cornelis, Lu Tian
+/., +?/. 9 1i c.67-2A>G r.spl p.? ACMG likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.94578624T>C g.94113068T>C c.67-2A>G p.(?), c.67-2A>G(;)769-784C>T - ABCA4_001055 ACMG PM2, PVS1, PP5, ACMG PVS1, PM2_sup; severity category severe, no segregation analysis done, 1 more item Journal: Cornelis 2023, PubMed: Cornelis 2024, Journal: Cornelis 2024, PubMed: Khan 2019, 3 more items 92871 - Germline, Germline/De novo (untested), SUMMARY record, Unknown no - - - - Johan den Dunnen, Stéphanie Cornelis, Rebekkah Hitti-Malin
+/. 8 1i c.67-1del r.spl p.? ACMG pathogenic, pathogenic (recessive) g.94578623del g.94113067del 67-1delG, c.[5714+5G>A];[67-1delG] - ABCA4_001093 67-1delG, ACMG PVS1, PM2_sup, PP3_m; severity category severe Journal: Cornelis 2023, PubMed: Green 2020, PubMed: Green 2020, Journal: Green 2020 - - Germline, SUMMARY record yes - - - - Terry-Lynn Young, Stéphanie Cornelis, Darren O'Rielly
+/. 1 - c.67-1dup r.spl? p.? ACMG pathogenic (recessive) g.94578623dup g.94113067dup - - ABCA4_002879 combination of variants not reported - - - Germline - - - - - Oscar Francisco Chacón Camacho
+/., +?/. 7 1i c.67-1G>C r.spl p.? ACMG likely pathogenic, likely pathogenic (recessive), pathogenic (recessive) g.94578623C>G g.94113067C>G c.67-1G>C IVS1-1 G>C, c.67-1G>C p.(I23Afs*24), c.67-1G>C, Heterozygous, IVS1-1G>C, 1 more item - ABCA4_001481 ACMG PVS1, PM2_sup; severity category severe, no variant 2nd chromosome Journal: Cornelis 2023, PubMed: Cornelis 2024, Journal: Cornelis 2024, PubMed: Fujinami 2019, 3 more items - - Germline, SUMMARY record, Unknown yes - - - - Frans Cremers, Stéphanie Cornelis
+?/., ?/. 2 2 c.69T>G r.spl? p.(Ile23Met) ACMG likely pathogenic (recessive), VUS g.94578620A>C g.94113064A>C c.69T>G, p.Ile23Met Heterozygous - ABCA4_002244 ACMG PM2_sup; severity category not assesed, no variant 2nd chromosome Journal: Cornelis 2023, PubMed: Goetz 2020 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
+/., +?/+?, +?/., ?/. 15 2 c.70C>T r.(70c>u), r.(?) p.(Arg24Cys), p.[IIe23IIefs*30,=] ACMG likely pathogenic, likely pathogenic (recessive), pathogenic (recessive), VUS g.94578619G>A g.94113063G>A 70C>T, c.70 C>T, c.70C>T Arg24Cys CGC>TGC, c.70C>T p.(Arg24Cys), c.70C>T p.Arg24Cys, 5 more items - ABCA4_000253 no segregation analysis done, no variant 2nd chromosome, VKGL data sharing initiative Nederland Zixi Sun 2020, submitted, PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, 10 more items - - CLASSIFICATION record, Germline, Germline/De novo (untested), SUMMARY record, Unknown ? 5, 120400, 0, 0.00004153, ExAC 5, 120400, 0, 0.00004153 - - - Johan den Dunnen, Stéphanie Cornelis, VKGL-NL_Nijmegen, Zixi Sun
+/., +?/., -?/., ?/. 55 1, 2 c.71G>A r.(71g>a), r.(?) p.(Arg24Cys), p.(Arg24His), p.(Met1Ile) ACMG likely benign, likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), 1 more item g.94578618C>T g.94113062C>T ABCA4 c.71G>A(;)1804C>T, V2: c.71G>A, (p.Arg24His), ABCA4 c.71G>A, p.R24H, c.71 G>A, 15 more items - ABCA4_000252 alleles in cis or trans; heterozygous, case unsolved, heterozygous, no segregation analysis done, 5 more items Prevention Genetics, PubMed: Bauwens 2014, PubMed: Bauwens 2019, PubMed: Chacón-Camacho 2013, 28 more items - - CLASSIFICATION record, Germline, Germline/De novo (untested), SUMMARY record, Unknown -, ?, yes 2/899 cases, 23, 120444, 0, 0.000191, ExAC 23, 120444, 0, 0.000191, frequency 0.016, 1 more item - - - Global Variome, with Curator vacancy, Johan den Dunnen, Frans Cremers, Stéphanie Cornelis, VKGL-NL_Rotterdam, VKGL-NL_Nijmegen, Lu Tian, Zelia Corradi
+?/. 1 - c.83A>C r.(?) p.(Glu28Ala) - likely pathogenic (recessive) g.94578606T>G g.94113050T>G [83A>C;1610G>A] - ABCA4_002454 - PubMed: Tian 2024 - - Germline yes - - - - Lu Tian
+?/., ?/. 2 2 c.85C>T r.(?) p.(Leu29Phe) ACMG likely pathogenic (recessive), VUS g.94578604G>A g.94113048G>A c.85C>T, p.Leu29Phe Heterozygous - ABCA4_002243 ACMG PM2_sup; severity category not assesed, no variant 2nd chromosome Journal: Cornelis 2023, PubMed: Goetz 2020 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
+?/., ?/. 4 2 c.86T>G r.(?) p.(Arg24His), p.(Leu29Arg) ACMG likely pathogenic (recessive), VUS g.94578603A>C g.94113047A>C c.86T>G p.(L29R) - ABCA4_002242 ACMG PM2_sup, PP3_m; severity category not assesed, no variant 2nd chromosome Journal: Cornelis 2023, PubMed: Cornelis 2024, Journal: Cornelis 2024, PubMed: Schulz 2017 - - SUMMARY record, Unknown - - - - - Frans Cremers, Stéphanie Cornelis
-/. 1 - c.87C>T r.(?) p.(Leu29=) - benign g.94578602G>A g.94113046G>A ABCA4(NM_000350.3):c.87C>T (p.L29=) - ABCA4_001246 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 4 - c.88G>A r.(?) p.(Val30Met) - VUS g.94578601C>T g.94113045C>T - - ABCA4_002757 - PubMed: Cornelis 2024, Journal: Cornelis 2024 - - Germline, Unknown yes - - - - Frans Cremers
+?/. 9 2 c.91T>C r.(91u>c), r.(?) p.(Trp31Arg) ACMG likely pathogenic, likely pathogenic (recessive) g.94578598A>G g.94113042A>G c.91T>C, c.91T>C (p.Trp31Arg), c.91T>C, p.Trp31Arg Heterozygous, p.(W31*), p.(W31R), p.W31* - ABCA4_000251 no segregation analysis done PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, PubMed: Goetz 2020, 5 more items - - Germline, SUMMARY record, Unknown ? - - - - Jana Zernant, Stéphanie Cornelis
+/. 3 2 c.92G>A r.(?) p.(Trp31*), p.(Trp31Ter) ACMG pathogenic, pathogenic (recessive) g.94578597C>T g.94113041C>T c.92G>A, c.92G>A, p.Trp31Stop Heterozygous - ABCA4_000250 ACMG PVS1, PS4, PM3_sup, PP3_m; severity category severe Journal: Cornelis 2023, PubMed: Goetz 2020, PubMed: Khan 2020 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
+/., +?/. 21 2 c.93G>A r.(93g>a), r.(?) p.(Gln8Hisfs*33), p.(Trp31*), p.(Trp31Ter) ACMG likely pathogenic, pathogenic (recessive) g.94578596C>T, g.94578597C>T g.94113040C>T c.93G>A p.W31X, c.93G>A (p.Trp31*), c.93G>A p.(Trp31*), c.93G>A, p.Tyr31*, p.(W31*) - ABCA4_000250 - PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, PubMed: Fakin 2016, 8 more items - - Germline, Germline/De novo (untested), SUMMARY record, Unknown -, ?, yes - - - - Johan den Dunnen, Jana Zernant, Frans Cremers, Stéphanie Cornelis
+/., +?/., ?/. 3 2 c.94C>A r.(?) p.(Pro32Thr) ACMG likely pathogenic (recessive), pathogenic (recessive), VUS g.94578595G>T g.94113039G>T c.94C>A - ABCA4_001328 ACMG PM2_sup, PP3_m; severity category mild/moderate Journal: Cornelis 2023, PubMed: Hu 2019 - - Germline, SUMMARY record, Unknown - - - - - Stéphanie Cornelis, Fangyuan Hu
+/., +?/., ?/. 5 2 c.95C>T r.(?) p.(Pro32Leu) ACMG likely pathogenic (recessive), pathogenic (recessive), VUS g.94578594G>A g.94113038G>A c.95C>T, c.95C>T, p.Pro32Leu Heterozygous - ABCA4_001327 ACMG PM2_sup, PM3_sup, PP3_m; severity category mild/moderate Journal: Cornelis 2023, PubMed: Goetz 2020, PubMed: Hu 2019 - - Germline, SUMMARY record, Unknown - - - - - Stéphanie Cornelis, Fangyuan Hu
+/., +?/., ?/. 88 2 c.101_106del r.(101_106del), r.(?) p.(Ser34_Leu35del) ACMG, other likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS g.94578583_94578588del, g.94578589_94578594del g.94113033_94113038del 101_106delCTTTAT, ABCA4 c.101_106del(;)1804C>T, V2: c.101_106delCTTTAT, (p.Ser34_Leu35del), 14 more items - ABCA4_000249 alleles in cis or trans; heterozygous, no segregation analysis done, no variant 2nd chromosome, 3 more items Zixi Sun 2020, submitted, PubMed: Chen 2021, PubMed: Consugar 2015, PubMed: Dan 2019, PubMed: Gao 2019, 14 more items - rs745925619 Germline, Germline/De novo (untested), SUMMARY record, Unknown ?, yes - - - - Johan den Dunnen, Stéphanie Cornelis, Fangyuan Hu, Handong Dan, Zixi Sun, Qing Zhu, Lu Tian
+?/. 3 - c.101_106delCTTTAT r.(?) p.(Ser34_Leu35del) - likely pathogenic g.94578589_94578594del g.94113033_94113038del ABCA4 c.101_106del(;)1804C>T; p.(Ser34_Leu35del), ABCA4 c.[101_106del];[?]; p.(Ser34_Leu35del), 1 more item - ABCA4_000249 heterozygous, heterozygous; single variant in a recessive gene, no second allele found PubMed: Chen 2021 - - Germline, Germline/De novo (untested) ?, yes Taiwan Biobank: 0.000334; GnomAD_exome_East: 0.000272; GnomAD_All: 0.0000199 - - - LOVD
+/., +?/. 11 2 c.108del r.(108del), r.(?) p.(Leu37Trpfs*3), p.(Leu37TrpfsTer3) ACMG likely pathogenic, pathogenic (recessive) g.94578583del g.94113027del 106delT, 108delT, 1bpdel(T) Co 36 which creates stop at Co 38, c.106del (p.Leu37fs), 2 more items - ABCA4_000248 no segregation analysis done PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, PubMed: Genead 2009, 7 more items - - Germline, Germline/De novo (untested), SUMMARY record, Unknown ?, yes - - - - Stéphanie Cornelis
-?/. 1 - c.117G>A r.(?) p.(Leu39=) - likely benign g.94578572C>T g.94113016C>T - - ABCA4_001245 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/., +?/., ?/. 12 2 c.122G>A r.(122g>a), r.(?) p.(Trp41*), p.(Trp41Ter) ACMG likely pathogenic, pathogenic, pathogenic (recessive), VUS g.94578567C>T g.94113011C>T c.121G>A, c.122G>A, c.122G>A (p.Trp41*), c.122G>A (p.Trp41Ter), c.122G>A p.(W41*), 2 more items - ABCA4_000247 ACMG PM2, PVS1, PP5, no segregation analysis done, no variant 2nd chromosome, 1 more item Prevention Genetics, PubMed: Bauwens 2019, PubMed: Kellner 2009, PubMed: Khan 2019, PubMed: Khan 2020, 6 more items - - CLASSIFICATION record, Germline, SUMMARY record, Unknown -, ?, no, yes - - - - Johan den Dunnen, Stéphanie Cornelis, VKGL-NL_Nijmegen
+/., +?/. 4 2 c.123G>A r.(123g>a), r.(?) p.(Trp41*), p.(Trp41Ter) ACMG likely pathogenic, pathogenic (recessive) g.94578566C>T g.94113010C>T c.123G>A p.Trp41* Het, W41X - ABCA4_000246 - Bianco 2023, submitted, Prevention Genetics, PubMed: Cideciyan 2009, 1 more item - - Germline, SUMMARY record, Unknown -, yes 1, 120980, 0, 0.000008266 - - - Stéphanie Cornelis, Lorenzo Bianco
?/. 2 2 c.140C>T r.(?) p.(Pro47Leu) ACMG VUS g.94578549G>A g.94112993G>A c.140C>T, p.Pro47Leu Heterozygous - ABCA4_002241 ACMG PP3; severity category benign Journal: Cornelis 2023, PubMed: Goetz 2020 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
-/. 1 - c.141= r.(=) p.(Pro47=) - benign g.94578548T>C - ABCA4(NM_000350.3):c.141A>G (p.P47=) - ABCA4_000950 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-/., ?/. 6 2 c.141G>A r.(?) p.(Pro47=) ACMG benign, VUS g.94578548=, g.94578548C>T g.94112992=, g.94112992C>T c.141A>G, c.141A>G, p.Pro47 Homozygous, c.141A>G, p.Pro47= Homozygous - ABCA4_002240 ACMG BS1, BP4_m, BP7 Journal: Cornelis 2023, PubMed: Goetz 2020 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
+?/., ?/. 3 2 c.156T>G r.(?) p.(His52Gln) ACMG likely pathogenic (recessive), VUS g.94578533A>C g.94112977A>C c.156T>G, p.His52Gln Heterozygous - ABCA4_002239 ACMG PP3; severity category not assesed Journal: Cornelis 2023, PubMed: Corradi 2023, Journal: Corradi 2023, PubMed: Goetz 2020 - - Germline, SUMMARY record, Unknown - - - - - Stéphanie Cornelis, Zelia Corradi
+/., +?/. 7 2 c.157G>T r.(?) p.(Glu53*), p.(Glu53Ter) ACMG likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.13174G>T, g.94578532C>A g.94112976C>A c.157G>T, c.157G>T p.E53*, c.157G>T, p.E53*, p.E53X - ABCA4_002238 ACMG PVS1, PS4, PM3_sup, PP3_m; severity category severe, compound heterozygous Journal: Cornelis 2023, PubMed: Groselj 2012, PubMed: Jiang 2016, PubMed: Liu 2020, PubMed: Liu-2020, 1 more item - - Germline, SUMMARY record, Unknown yes - - - - Stéphanie Cornelis, Lu Tian
+/., +?/., ?/. 15 2 c.160T>G r.(160u>g), r.(?), r.spl? p.(Cys54Gly), p.(Cys54Gly, ?), p.(Cys54Gly,?) ACMG likely pathogenic, likely pathogenic (recessive), pathogenic, VUS g.94578529A>C g.94112973A>C c.160T>G, c.160T>G p.(C54G), c.160T>G(;)587C>T, C54T, p.C54G, [c.160T>G], 1 more item - ABCA4_000245 no segregation analysis done, no variant 2nd chromosome, segregation analysis done when possible, 1 more item PubMed: Chen 2010, PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, 7 more items - - Germline, SUMMARY record, Unknown ?, no, yes - - - - Frans Cremers, Stéphanie Cornelis, Rebekkah Hitti-Malin
+?/. 2 2i c.160+1G>A r.spl p.? ACMG likely pathogenic, likely pathogenic (recessive) g.94578528C>T g.94112972C>T 160+1g > a - ABCA4_000244 - PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, PubMed: Webster 2001 - - Germline, SUMMARY record - - - - - Stéphanie Cornelis
+/., +?/. 6 2i c.160+2T>C r.spl, r.spl? p.(?), p.? ACMG likely pathogenic, pathogenic, pathogenic (recessive) g.94578527A>G g.94112971A>G c.160+2T4C, c.160+2T>C, c.160+2T>C p.(?) - ABCA4_000243 no variant 2nd chromosome, unknown variant 2nd allele PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, PubMed: Khan 2019, 4 more items - - Germline, SUMMARY record, Unknown ?, no - - - - Stéphanie Cornelis
+?/., ?/. 4 2i c.160+5G>A r.(?) p.(?), p.? ACMG likely pathogenic, VUS g.94578524C>T g.94112968C>T c.160+5G>A, splice sitealteration, c.IVS2+5G>Ac.160+5G>A, Heterozygous - ABCA4_002237 ACMG PS4, PM3, PP3; causative variant of unknown severity Journal: Cornelis 2023, PubMed: Fujinami 2019, PubMed: Goetz 2020 - - SUMMARY record, Unknown - - - - - Stéphanie Cornelis
+?/., +?/? 7 2i c.160+5G>C r.spl?, r.[67_160del,161_302delins161+1_161+14,=], r.[=,67_160del,161_302delinsguaaccauagcagg] p.(?), p.[(=,Ile23Alafs∗24,His55Asnfs∗63)], p.[Ile23AlafsTer24,His55AsnfsTer63,=], 1 more item ACMG likely pathogenic, likely pathogenic (recessive), NA g.94578524C>G g.94112968C>G c.160+5G>C (p.?), c.160+5G>C, Heterozygous, c.160+5G>C, splice sitealteration - ABCA4_000068 expression cloning midigene splicing construct: 0.343 correctly spliced RNA, 2 more items PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, PubMed: Fujinami 2019, 4 more items - - Germline, In vitro (cloned), SUMMARY record, Unknown - - - - - Monika Oldak, Frans Cremers, Stéphanie Cornelis
+/., +?/., ?/. 4 2i c.161-395G>A r.spl, r.[161_302del,=,160_161ins161-484_161–394] p.[(Cys54Serfs*14,=,Cys54Cysfs*75)], p.[(His55ProfsTer136,=)], p.[Cys54Serfs*14,=,Cys54Cysfs*75] ACMG NA, pathogenic (recessive), VUS g.94577530C>T g.94111974C>T - - ABCA4_002453 expression cloning midi-gene splicing assay, 0.36 normally spliced mRNA; predicted severity moderate, 1 more item PubMed: Corradi 2023, Journal: Corradi 2023, PubMed: Tian 2024 - - Germline, In vitro (cloned) yes - - - - Johan den Dunnen, Lu Tian, Zelia Corradi
+?/., ?/. 4 2i c.161-23T>A r.spl? p.(?), p.? ACMG likely pathogenic (recessive), VUS g.94577158A>T g.94111602A>T c.161-23T>A p.[=,Cys54Serfs*14] - ABCA4_001065 ACMG PM2_sup, PM3_sup, PP3; severity category uncertain, no segregation analysis done Journal: Cornelis 2023, PubMed: Khan 2019 - - SUMMARY record, Unknown no - - - - Stéphanie Cornelis
+?/., ?/. 3 2i c.161-23T>G r.[=,161_302del] p.[=,Cys54Serfs*14], p.[=,Cys54SerfsTer14] ACMG likely pathogenic (recessive), VUS g.94577158A>C g.94111602A>C c.161-23T>G, [161-23T>G] - ABCA4_002235 ACMG PS4, BP4_m; severity category mild, no segregation analysis done Bauwens 2019, Journal: Cornelis 2023, PubMed: Bauwens 2019 - - SUMMARY record, Unknown ? - - - - Stéphanie Cornelis
-?/., ?/. 3 2i c.161-5T>C r.(?) p.(?), p.? ACMG likely benign, VUS g.94577140A>G g.94111584A>G – c.161-5T>C - ABCA4_001520 ACMG PM2_sup, BP4_m; severity category not assesed, no variant 2nd chromosome Journal: Cornelis 2023, PubMed: Tsipi 2016 - - Germline, SUMMARY record, Unknown - - - - - Stéphanie Cornelis
+/., +?/. 3 2i c.161-2A>G r.spl p.? ACMG likely pathogenic, pathogenic (recessive) g.94577137T>C g.94111581T>C c.161-2A>G - ABCA4_000242 not statistically tested, classification unknown PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, PubMed: Xin 2015 - - Germline, SUMMARY record - - - - - Stéphanie Cornelis
+/., +?/. 8 2i c.161-1G>A r.spl p.? ACMG likely pathogenic, pathogenic (recessive) g.94577136C>T g.94111580C>T c.161-1G.A (p.?), c.161-1G>A (p.?), c.161-1G>A IVS2-1 G>A, c.161G>A, IVS2-1 G>A, 2 more items - ABCA4_000116 no segregation analysis done PubMed: Collison 2019, PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, 5 more items - - Germline, SUMMARY record, Unknown -, ?, yes - - - - Stéphanie Cornelis
+?/. 1 2i c.161-1G>T r.spl? p.? ACMG pathogenic g.94577136C>A - - - ABCA4_001444 - Mena et al., 2020 submitted. - - Germline yes - - - - Marcela Mena
+/., +?/., ?/. 155 3, ? c.161G>A r.(?), r.spl, r.spl?, r.[(161_302del,161g>a)], r.[161_302del,161g>a] p.(Cys54Tyr), p.(Cys54Tyr, ?), p.[(Cys54Serfs*14,Cys54Tyr)], p.[Cys54Serfs*14,Cys54Tyr], 1 more item ACMG likely pathogenic, likely pathogenic (recessive), NA, pathogenic, pathogenic (recessive), VUS g.94577135C>T g.94111579C>T 161G > A, 161G>A, 2 c.156T>G p.His52Gln Heterozygous, ABCA4 c.161G>A p.(Cys54Tyr) het, 25 more items - ABCA4_000240 compound heterozygous, probably causal, no segregation analysis done, no variant 2nd chromosome, 2 more items Bianco 2023, submitted, Fadaie 2019, Prevention Genetics, PubMed: Alabduljalil 2019, PubMed: Chen 2019, 49 more items - - Germline, Germline/De novo (untested), In vitro (cloned), SUMMARY record, Unknown -, ?, no, yes 2, 121210, 0, 0.0000165, 2/899 cases, ExAC 2, 121210, 0, 0.0000165 - - - Global Variome, with Curator vacancy, Stéphanie Cornelis, Zeinab Fadaie, Zeinab Fadaie, Lorenzo Bianco, Zelia Corradi
+/., +?/. 6 3 c.161G>T r.(?), r.161_302del, r.spl p.(Cys54Phe), p.(Cys54SerfsTer14), p.(Cys54Serfs∗14), p.Cys54SerfsTer14 ACMG likely pathogenic, NA, pathogenic (recessive) g.94577135C>A g.94111579C>A - - ABCA4_000241 expression cloning midigene splicing construct: no correctly spliced RNA, 1 more item PubMed: Cornelis 2017, Journal: Cornelis 2017, Journal: Cornelis 2023, PubMed: Oishi 2014, 2 more items - - Germline, In vitro (cloned), SUMMARY record -, yes ExAC 1, 121210, 0, 0.00000825 - - - Frans Cremers, Stéphanie Cornelis, Lu Tian
+?/., ?/. 3 3 c.164A>C r.(?) p.(His55Pro) ACMG likely pathogenic, likely pathogenic (recessive), VUS g.94577132T>G g.94111576T>G 164A>C (H55P), 164A?C H55P - ABCA4_001545 ACMG PM2_sup, PM5, PP3_m; severity category not assesed, no variant 2nd chromosome Journal: Cornelis 2023, PubMed: Downs 2007 - - Germline, SUMMARY record, Unknown - - - - - Julia Lopez, Stéphanie Cornelis
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