ABCA4 c.4253+43G>A

Variant ID: 1-94496509-C-T

NM_000350.2(ABCA4):c.4253+43G>A

This variant was identified in 19 publications

View GRCh38 version.




Publications:


Using single molecule Molecular Inversion Probes as a cost-effective, high-throughput sequencing approach to target all genes and loci associated with macular diseases.

Human Mutation
Hitti-Malin, Rebekkah J RJ; Dhaenens, Claire-Marie CM; Panneman, Daan M DM; Corradi, Zelia Z; Khan, Mubeen M; den Hollander, Anneke I AI; Farrar, G Jane GJ; Gilissen, Christian C; Hoischen, Alexander A; van de Vorst, Maartje M; Bults, Femke F; Boonen, Erica G M EGM; Saunders, Patrick P; , ; Roosing, Susanne S; Cremers, Frans P M FPM
Publication Date: 2022-10-19

Variant appearance in text: ABCA4: 4253+43G>A
PubMed Link: 36259723
Variant Present in the following documents:
  • HUMU-43-2234-s012.xlsx, sheet 1
  • HUMU-43-2234-s007.xlsx, sheet 1
View BVdb publication page



Phenotyping of ABCA4 Retinopathy by Machine Learning Analysis of Full-Field Electroretinography.

Translational Vision Science & Technology
Glinton, Sophie L SL; Calcagni, Antonio A; Lilaonitkul, Watjana W; Pontikos, Nikolas N; Vermeirsch, Sandra S; Zhang, Gongyu G; Arno, Gavin G; Wagner, Siegfried K SK; Michaelides, Michel M; Keane, Pearse A PA; Webster, Andrew R AR; Mahroo, Omar A OA; Robson, Anthony G AG
Publication Date: 2022-09-01

Variant appearance in text: ABCA4: 4253+43G>A
PubMed Link: 36178783
Variant Present in the following documents:
  • Main text
  • tvst-11-9-34_s002.pdf
  • tvst-11-9-34.pdf
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Rare and common variants in ROM1 and PRPH2 genes trans-modify Stargardt/ABCA4 disease.

Plos Genetics
Zernant, Jana J; Lee, Winston W; Wang, Jun J; Goetz, Kerry K; Ullah, Ehsan E; Nagasaki, Takayuki T; Su, Pei-Yin PY; Fishman, Gerald A GA; Tsang, Stephen H SH; Tumminia, Santa J SJ; Brooks, Brian P BP; Hufnagel, Robert B RB; Chen, Rui R; Allikmets, Rando R
Publication Date: 2022-03

Variant appearance in text: ABCA4: 4253+43G>A
PubMed Link: 35353811
Variant Present in the following documents:
  • Main text
  • pgen.1010129.pdf
View BVdb publication page



Impact of Next Generation Sequencing in Unraveling the Genetics of 1036 Spanish Families With Inherited Macular Dystrophies.

Investigative Ophthalmology & Visual Science
Del Pozo-Valero, Marta M; Riveiro-Alvarez, Rosa R; Martin-Merida, Inmaculada I; Blanco-Kelly, Fiona F; Swafiri, Saoud S; Lorda-Sanchez, Isabel I; Trujillo-Tiebas, Maria José MJ; Carreño, Ester E; Jimenez-Rolando, Belen B; Garcia-Sandoval, Blanca B; Corton, Marta M; Avila-Fernandez, Almudena A; Ayuso, Carmen C
Publication Date: 2022-02-01

Variant appearance in text: STGD: 4253+43G>A
PubMed Link: 35119454
Variant Present in the following documents:
  • Main text
  • iovs-63-2-11_s002.pdf
  • iovs-63-2-11.pdf
View BVdb publication page



Impact of Next Generation Sequencing in Unraveling the Genetics of 1036 Spanish Families With Inherited Macular Dystrophies.

Investigative Ophthalmology & Visual Science
Del Pozo-Valero, Marta M; Riveiro-Alvarez, Rosa R; Martin-Merida, Inmaculada I; Blanco-Kelly, Fiona F; Swafiri, Saoud S; Lorda-Sanchez, Isabel I; Trujillo-Tiebas, Maria José MJ; Carreño, Ester E; Jimenez-Rolando, Belen B; Garcia-Sandoval, Blanca B; Corton, Marta M; Avila-Fernandez, Almudena A; Ayuso, Carmen C
Publication Date: 2022-02-01

Variant appearance in text: STGD: 4253+43G>A
PubMed Link: 35119454
Variant Present in the following documents:
  • Main text
  • iovs-63-2-11_s002.pdf
  • iovs-63-2-11.pdf
View BVdb publication page



A genotype-phenotype correlation matrix for ABCA4 disease based on long-term prognostic outcomes.

Jci Insight
Lee, Winston W; Zernant, Jana J; Su, Pei-Yin PY; Nagasaki, Takayuki T; Tsang, Stephen H SH; Allikmets, Rando R
Publication Date: 2022-01-25

Variant appearance in text: ABCA4: 4253+43G>A
PubMed Link: 34874912
Variant Present in the following documents:
  • jciinsight-7-156154.pdf
  • jciinsight-7-156154-s174.xlsx, sheet 1
View BVdb publication page



High-Throughput Sequencing to Identify Mutations Associated with Retinal Dystrophies.

Genes
Song, Fei F; Owczarek-Lipska, Marta M; Ahmels, Tim T; Book, Marius M; Aisenbrey, Sabine S; Menghini, Moreno M; Barthelmes, Daniel D; Schrader, Stefan S; Spital, Georg G; Neidhardt, John J
Publication Date: 2021-08-20

Variant appearance in text: ABCA4: 4253+43G>A
PubMed Link: 34440443
Variant Present in the following documents:
  • Main text
View BVdb publication page



An Overview of the Genetics of ABCA4 Retinopathies, an Evolving Story.

Genes
Al-Khuzaei, Saoud S; Broadgate, Suzanne S; Foster, Charlotte R CR; Shah, Mital M; Yu, Jing J; Downes, Susan M SM; Halford, Stephanie S
Publication Date: 2021-08-13

Variant appearance in text: ABCA4: 4253+43G>A
PubMed Link: 34440414
Variant Present in the following documents:
  • Main text
  • genes-12-01241.pdf
View BVdb publication page



Retinal Pigment Epithelium Atrophy in Recessive Stargardt Disease as Measured by Short-Wavelength and Near-Infrared Autofluorescence.

Translational Vision Science & Technology
Jauregui, Ruben R; Nuzbrokh, Yan Y; Su, Pei-Yin PY; Zernant, Jana J; Allikmets, Rando R; Tsang, Stephen H SH; Sparrow, Janet R JR
Publication Date: 2021-01

Variant appearance in text: ABCA4: 4253+43G>A
PubMed Link: 33505770
Variant Present in the following documents:
  • tvst-10-1-3_s002.pdf
View BVdb publication page



Deep learning segmentation of hyperautofluorescent fleck lesions in Stargardt disease.

Scientific Reports
Charng, Jason J; Xiao, Di D; Mehdizadeh, Maryam M; Attia, Mary S MS; Arunachalam, Sukanya S; Lamey, Tina M TM; Thompson, Jennifer A JA; McLaren, Terri L TL; De Roach, John N JN; Mackey, David A DA; Frost, Shaun S; Chen, Fred K FK
Publication Date: 2020-10-05

Variant appearance in text: ABCA4: 4253+43G>A
PubMed Link: 33020556
Variant Present in the following documents:
  • Main text
  • 41598_2020_Article_73339.pdf
View BVdb publication page



Association of Sex With Frequent and Mild ABCA4 Alleles in Stargardt Disease.

Jama Ophthalmology
Runhart, Esmee H EH; Khan, Mubeen M; Cornelis, Stéphanie S SS; Roosing, Susanne S; Del Pozo-Valero, Marta M; Lamey, Tina M TM; Liskova, Petra P; Roberts, Lisa L; Stöhr, Heidi H; Klaver, Caroline C W CCW; Hoyng, Carel B CB; Cremers, Frans P M FPM; Dhaenens, Claire-Marie CM; ,
Publication Date: 2020-10-01

Variant appearance in text: STGD1: 4253+43G>A
PubMed Link: 32815999
Variant Present in the following documents:
  • Main text
View BVdb publication page



Detailed Phenotyping and Therapeutic Strategies for Intronic ABCA4 Variants in Stargardt Disease.

Molecular Therapy. Nucleic Acids
Khan, Mubeen M; Arno, Gavin G; Fakin, Ana A; Parfitt, David A DA; Dhooge, Patty P A PPA; Albert, Silvia S; Bax, Nathalie M NM; Duijkers, Lonneke L; Niblock, Michael M; Hau, Kwan L KL; Bloch, Edward E; Schiff, Elena R ER; Piccolo, Davide D; Hogden, Michael C MC; Hoyng, Carel B CB; Webster, Andrew R AR; Cremers, Frans P M FPM; Cheetham, Michael E ME; Garanto, Alejandro A; Collin, Rob W J RWJ
Publication Date: 2020-09-04

Variant appearance in text: ABCA4: 4253+43G>A
PubMed Link: 32653833
Variant Present in the following documents:
  • Main text
  • main.pdf
  • mmc6.pdf
View BVdb publication page



Clinical spectrum, genetic complexity and therapeutic approaches for retinal disease caused by ABCA4 mutations.

Progress In Retinal And Eye Research
Cremers, Frans P M FPM; Lee, Winston W; Collin, Rob W J RWJ; Allikmets, Rando R
Publication Date: 2020-11

Variant appearance in text: ABCA4: 4253+43G>A
PubMed Link: 32278709
Variant Present in the following documents:
  • Main text
  • nihms-1627942.pdf
View BVdb publication page



Findings from a Genotyping Study of Over 1000 People with Inherited Retinal Disorders in Ireland.

Genes
Whelan, Laura L; Dockery, Adrian A; Wynne, Niamh N; Zhu, Julia J; Stephenson, Kirk K; Silvestri, Giuliana G; Turner, Jacqueline J; O'Byrne, James J JJ; Carrigan, Matthew M; Humphries, Peter P; Keegan, David D; Kenna, Paul F PF; Farrar, G Jane GJ
Publication Date: 2020-01-16

Variant appearance in text: ABCA4: 4253+43G>A
PubMed Link: 31963381
Variant Present in the following documents:
  • Main text
  • genes-11-00105.pdf
View BVdb publication page



Prevalence of ABCA4 Deep-Intronic Variants and Related Phenotype in An Unsolved "One-Hit" Cohort with Stargardt Disease.

International Journal Of Molecular Sciences
Nassisi, Marco M; Mohand-Saïd, Saddek S; Andrieu, Camille C; Antonio, Aline A; Condroyer, Christel C; Méjécase, Cécile C; Varin, Juliette J; Wohlschlegel, Juliette J; Dhaenens, Claire-Marie CM; Sahel, José-Alain JA; Zeitz, Christina C; Audo, Isabelle I
Publication Date: 2019-10-11

Variant appearance in text: ABCA4: 4253+43G>A
PubMed Link: 31614660
Variant Present in the following documents:
  • Main text
  • ijms-20-05053-s001.pdf
  • ijms-20-05053.pdf
View BVdb publication page



ABCA4-associated disease as a model for missing heritability in autosomal recessive disorders: novel noncoding splice, cis-regulatory, structural, and recurrent hypomorphic variants.

Genetics In Medicine : Official Journal Of The American College Of Medical Genetics
Bauwens, Miriam M; Garanto, Alejandro A; Sangermano, Riccardo R; Naessens, Sarah S; Weisschuh, Nicole N; De Zaeytijd, Julie J; Khan, Mubeen M; Sadler, Françoise F; Balikova, Irina I; Van Cauwenbergh, Caroline C; Rosseel, Toon T; Bauwens, Jim J; De Leeneer, Kim K; De Jaegere, Sarah S; Van Laethem, Thalia T; De Vries, Meindert M; Carss, Keren K; Arno, Gavin G; Fakin, Ana A; Webster, Andrew R AR; de Ravel de l'Argentière, Thomy J L TJL; Sznajer, Yves Y; Vuylsteke, Marnik M; Kohl, Susanne S; Wissinger, Bernd B; Cherry, Timothy T; Collin, Rob W J RWJ; Cremers, Frans P M FPM; Leroy, Bart P BP; De Baere, Elfride E
Publication Date: 2019-08

Variant appearance in text: ABCA4: 4253+43G>A
PubMed Link: 30670881
Variant Present in the following documents:
  • Main text
  • 41436_2018_420_MOESM1_ESM.xlsx, sheet 2
View BVdb publication page



Deep-intronic ABCA4 variants explain missing heritability in Stargardt disease and allow correction of splice defects by antisense oligonucleotides.

Genetics In Medicine : Official Journal Of The American College Of Medical Genetics
Sangermano, Riccardo R; Garanto, Alejandro A; Khan, Mubeen M; Runhart, Esmee H EH; Bauwens, Miriam M; Bax, Nathalie M NM; van den Born, L Ingeborgh LI; Khan, Muhammad Imran MI; Cornelis, Stéphanie S SS; Verheij, Joke B G M JBGM; Pott, Jan-Willem R JR; Thiadens, Alberta A H J AAHJ; Klaver, Caroline C W CCW; Puech, Bernard B; Meunier, Isabelle I; Naessens, Sarah S; Arno, Gavin G; Fakin, Ana A; Carss, Keren J KJ; Raymond, F Lucy FL; Webster, Andrew R AR; Dhaenens, Claire-Marie CM; Stöhr, Heidi H; Grassmann, Felix F; Weber, Bernhard H F BHF; Hoyng, Carel B CB; De Baere, Elfride E; Albert, Silvia S; Collin, Rob W J RWJ; Cremers, Frans P M FPM
Publication Date: 2019-08

Variant appearance in text: STGD1: 4253+43G>A
PubMed Link: 30643219
Variant Present in the following documents:
  • Main text
  • 41436_2018_Article_414.pdf
  • 41436_2018_414_MOESM1_ESM.pdf
View BVdb publication page



Detailed genetic characteristics of an international large cohort of patients with Stargardt disease: ProgStar study report 8.

The British Journal Of Ophthalmology
Fujinami, Kaoru K; Strauss, Rupert W RW; Chiang, John Pei-Wen JP; Audo, Isabelle S IS; Bernstein, Paul S PS; Birch, David G DG; Bomotti, Samantha M SM; Cideciyan, Artur V AV; Ervin, Ann-Margret AM; Marino, Meghan J MJ; Sahel, José-Alain JA; Mohand-Said, Saddek S; Sunness, Janet S JS; Traboulsi, Elias I EI; West, Sheila S; Wojciechowski, Robert R; Zrenner, Eberhart E; Michaelides, Michel M; Scholl, Hendrik P N HPN; , ; ,
Publication Date: 2019-03

Variant appearance in text: ABCR: 4253+43G>A
PubMed Link: 29925512
Variant Present in the following documents:
  • bjophthalmol-2018-312064supp004.pdf
  • bjophthalmol-2018-312064supp005.pdf
View BVdb publication page



Extremely hypomorphic and severe deep intronic variants in the ABCA4 locus result in varying Stargardt disease phenotypes.

Cold Spring Harbor Molecular Case Studies
Zernant, Jana J; Lee, Winston W; Nagasaki, Takayuki T; Collison, Frederick T FT; Fishman, Gerald A GA; Bertelsen, Mette M; Rosenberg, Thomas T; Gouras, Peter P; Tsang, Stephen H SH; Allikmets, Rando R
Publication Date: 2018-08

Variant appearance in text: ABCA4: 4253+43G>A
PubMed Link: 29848554
Variant Present in the following documents:
  • Main text
  • MCS002733Zer.pdf
  • supp_mcs.a002733_Supplemental_Figure_1.pdf
View BVdb publication page



Analysis of the ABCA4 gene by next-generation sequencing.

Investigative Ophthalmology & Visual Science
Zernant, Jana J; Schubert, Carl C; Im, Kate M KM; Burke, Tomas T; Brown, Carolyn M CM; Fishman, Gerald A GA; Tsang, Stephen H SH; Gouras, Peter P; Dean, Michael M; Allikmets, Rando R
Publication Date: 2011-10-31

Variant appearance in text: STGD1: 4253+43G>A
PubMed Link: 21911583
Variant Present in the following documents:
  • Main text
View BVdb publication page