Unique variants in the BEST1 gene

This database is one of the "Eye disease" gene variant databases.
Information The variants shown are described using the NM_004183.3 transcript reference sequence.

496 entries on 5 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 4 430A>G r.(?) p.(Ser144Gly) - likely pathogenic (recessive) g.61723372A>G g.61955900A>G BEST1 430A>G, p.S144G - BEST1_000331 heterozygous PubMed: Lacassagne 2011 - - Germline yes - - - - LOVD
+?/. 1 8 904G>C r.(?) p.(Asp302His) - likely pathogenic g.61727006G>C g.61959534G>C VMD2 904G>C, D302H - BEST1_000287 heterozygous PubMed: Marchant 2002 - - Unknown ? - - - - LOVD
+?/. 1 8 909T>A r.(?) p.(Asp303Glu) - likely pathogenic g.61727011T>A g.61959539T>A VMD2 909T>A, D303E - BEST1_000290 heterozygous PubMed: Marchant 2002 - - Germline yes - - - - LOVD
+?/. 1 8 923A>G r.(?) p.(Asn308Ser) - likely pathogenic g.61727025A>G g.61959553A>G VMD2 923A>G, N308S - BEST1_000296 heterozygous PubMed: Marchant 2002 - - Germline yes - - - - LOVD
+/., +?/. 5 1i c.-37+1G>T r.(?), r.spl, r.spl? p.(?), p.? - likely pathogenic, pathogenic, pathogenic (recessive) g.61717900G>T g.61950428G>T 11:61717900G>T ENST00000449131.2:c.-29+1G>T, BEST1 c.-29+1G>T,, c.-37+1G>T, 1 more item - BEST1_000116 homozygous, homozygous, different transcript, NM_001139443.1:c.-29+1G>T PubMed: Boon 2013, PubMed: Carss 2017, PubMed: Maggi_2021, PubMed: Turro 2020 - - Germline, Germline/De novo (untested), Unknown ?, yes - - - - LOVD
+?/. 3 1i c.-37+5G>A r.(?), r.spl? p.(?), p.0? - likely pathogenic g.61717904G>A, g.61719312G>A g.61950432G>A, g.61951840G>A BEST1 c.-29+5G>A, p.0?, BEST1 c.-37+5G>A, splice site, BEST1 c.388C>A; c.-37+5G>A - BEST1_000216 compound heterozygous, heterozygous, 1 more item PubMed: Birtel 2020, PubMed: Fung 2015, PubMed: Zanolli 2020 - - Germline, Unknown ?, yes - - - - LOVD
+?/. 1 _1i_2i c.(?_-36-15)_(152+1_153-1)del r.0? p.0? - likely pathogenic g.(?_61719278)_(61719431_61722578)del g.(?_61951806)_(61951959_61955106)del g.61719228_61719480del - BEST1_000243 - PubMed: Ellingford 2017 - - Germline - - - - - Johan den Dunnen
+?/. 1 1i_2i c.(-37+1_-36-1)_(152+1_153-1)del r.(?) p.0? - likely pathogenic g.(61717900_61719242)_(61719431_61722578)del g.(61950428_61951770)_(61951959_61955106)del del ex2 1-?_152+?del, no protein - BEST1_000448 - PubMed: Boon 2013 - - Unknown ? - - - - LOVD
+?/. 2 - c.-29+1G>T r.spl p.(?) - likely pathogenic g.61719251C>T g.61951779C>T BEST1 c.-29+1G>T, splicing - BEST1_000468 heterozygous, homozygous PubMed: Casalino 2020 - - Germline/De novo (untested) ? - - - - LOVD
+?/. 1 10_11_ c.1265_*341{0} r.? p.(His422fs) - likely pathogenic g.61729891_61733239del g.61962419_61965767del BEST1 deletion of 9348 bases (61729891e61733239), H422fsX431 - BEST1_000444 9348 bp deletion PubMed: Dalvin 2016 - - De novo - - - - - LOVD
+?/., -?/., ?/. 4 - c.? r.(?), r.? p.(?), p.0?, p.? - likely benign, likely pathogenic, VUS g.? g.? 1060C>T (F354W), 23C>T, BEST1 exons 1-2 deletion, R41S - BEST1_000000, DRD4_000002 heterozygous, nucleotide variant not written PubMed: Casalino 2020, PubMed: Renner 2005, PubMed: Xu 2014, PubMed: Zhuk 2006 - - Germline, Germline/De novo (untested), Unknown ? 1/11 cases, 1/314 case chromosomes - - - Julia Lopez
+?/. 1 2 c.1A>G r.(?) p.(Met1?) - likely pathogenic g.61719279A>G g.61951807A>G BEST1 c.1 A > G, p.M1V - BEST1_000450 homozygous PubMed: Shi 2020 - - Germline yes - - - - LOVD
+?/. 2 - c.4A>G r.(?) p.(Thr2Ala) - likely pathogenic g.61719282A>G g.61951810A>G BEST1 c.4A>G, p.(Thr2Ala) - BEST1_000341 heterozygous PubMed: Katagiri 2015 - - Germline, Unknown ?, yes - - - - LOVD
+?/. 2 - c.5C>A r.(?) p.(Thr2Asn) - likely pathogenic g.61719283C>A g.61951811C>A BEST1 Thr2Asn (c.5C->A) - BEST1_000298 heterozygous PubMed: Wong 2009 - - Germline, Unknown yes - - - - LOVD
+/., +?/. 8 2 c.5C>G r.(?) p.(Thr2Ser) ACMG likely pathogenic, pathogenic g.61719283C>G g.61951811C>G BEST1 c.5C.G [p.Thr2Ser], BEST1 c.5C>G, p.(Thr2Ser) - BEST1_000381 heterozygous PubMed: Gao 2019, PubMed: Guo 2018 - - Germline, Unknown ?, yes - - - - LOVD
+/. 1 2 c.5C>T r.(?) p.(Thr2Ile) ACMG likely pathogenic g.61719283C>T g.61951811C>T BEST1 c.5C>T, p.(Thr2Ile) - BEST1_000382 heterozygous PubMed: Frecer 2019 - - Unknown ? - - - - LOVD
+?/. 1 - c.8T>A r.(?) p.(Ile3Asn) - likely pathogenic g.61719286T>A g.61951814T>A BEST1 c.8T>A (p.I3N) - BEST1_000354 heterozygous PubMed: Matson 2015 - - Germline yes - - - - LOVD
+?/. 1 - c.8T>C r.(?) p.(Ile3Thr) - likely pathogenic g.61719286T>C g.61951814T>C BEST1 c.8T>C, p.(Ile3Thr) - BEST1_000299 heterozygous PubMed: Boon 2009 - - Germline yes - - - - LOVD
+?/. 4 2 c.10A>G r.(?) p.(Thr4Ala) - likely pathogenic g.61719288A>G g.61951816A>G BEST1 A>G10, T4A, BEST1 p.Thr4Ala - BEST1_000300 heterozygous, no nucleotide annotation, writen, extrapolated from protein change; heterozygous PubMed: Querques 2009, PubMed: Querques 2014 - - Germline yes - - - - LOVD
+/., +?/. 7 2 c.11C>T r.(?) p.(Thr4Ile) ACMG likely pathogenic g.61719289C>T g.61951817C>T Allele 1 c.11C>T (p.Thr4IIe), Allele 2 Wildtype, BEST1 c.11C>T, p.(Thr4Ile), 3 more items - BEST1_000207 heterozygous, heterozygous, individual unsolved, causality of variants unknown, solved, heterozygous PubMed: Frecer 2019, PubMed: Khan 2019, PubMed: Rodriguez-Munoz 2020, PubMed: Tian 2014, 1 more item - - Germline, Germline/De novo (untested), Unknown ?, yes - - - - LOVD
+?/. 2 2 c.15C>A r.(?) p.(Tyr5*) - likely pathogenic (recessive) g.61719293C>A g.61951821C>A BEST1 c.15C>A c., p.Y5X, BEST1 c.15C>A, p.Y5X - BEST1_000328 heterozygous PubMed: Lacassagne 2011 - - Germline yes - - - - LOVD
+/., +?/. 26 2 c.16A>C r.(?) p.(Thr6Pro) - likely pathogenic, pathogenic g.61719294A>C g.61951822A>C BEST1 A120C, T6P, BEST1 A16C, T6P, BEST1 c.16A>C, p.(Thr6Pro), c.16A>C, 2 more items - BEST1_000004 heterozygous, VKGL data sharing initiative Nederland PubMed: Booij-2011, PubMed: Boon 2007, PubMed: Boon 2009, PubMed: Kramer 2000, PubMed: Petrukhin 1998 - - CLASSIFICATION record, Germline, Unknown ?, yes 0/50 - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
+?/. 1 2 c.16A>G r.(?) p.(Thr6Ala) - likely pathogenic g.61719294A>G g.61951822A>G VMD2 gene missense mutation in exon 2 (Thr6Ala [ACA>GCA]) - BEST1_000301 no nucleotide annotation, writen, extrapolated from protein change; heterozygous PubMed: Apushkin 2006 - - De novo yes - - - - LOVD
+/., +?/. 3 2 c.17C>G r.(?) p.(Thr6Arg) - likely pathogenic, pathogenic g.61719295C>G g.61951823C>G BEST1 ACA-AGA, Thr6Arg, BEST1 c.17C>G, p.T6R, BEST1(NM_004183.3):c.17C>G (p.T6R) - BEST1_000084 heterozygous, VKGL data sharing initiative Nederland PubMed: Lotery 2000, PubMed: Tian 2014 - - CLASSIFICATION record, Unknown ?, yes - - - - VKGL-NL_Rotterdam
+/., +?/. 3 2 c.17C>T r.(?) p.(Thr6Ile) - likely pathogenic, pathogenic (dominant) g.61719295C>T g.61951823C>T BEST1 c.17C>T, p.Thr6Ile, BEST1, variant 1: c.17C>T/p.T6I - BEST1_000121 heterozygous, solved, heterozygous PubMed: Birtel 2018, PubMed: Gliem 2020, PubMed: Weisschuh 2020 - - Germline, Unknown ? - - - - LOVD
+/., +?/. 6 2i c.20G>A r.(?) p.(Ser7Asn) - likely pathogenic, pathogenic, pathogenic (!) g.61719298G>A g.61951826G>A BEST1 c.20G>A, p.(Ser7Asn), BEST1 c.20G>A, p.S7N - BEST1_000342 heterozygous, incomplete penetrance, variant in unaffected mother PubMed: Fan 2020, PubMed: Katagiri 2015, PubMed: Liu 2023, PubMed: Tian 2014 rs199508634 rs199508634 Germline, Unknown yes - - - - Johan den Dunnen
+/., +?/. 8 2 c.25G>A r.(?) p.(Val9Met) ACMG likely pathogenic, pathogenic (dominant) g.61719303G>A g.61951831G>A BEST1 c.25G>A, p.(Val9Met), BEST1 c.[25G>A];[25=], V1: c.25G>A, (p.Val9Met), BEST1 G25A, V9M, 3 more items - BEST1_000005 heterozygous, VKGL data sharing initiative Nederland, 1 more item PubMed: Birtel 2018, PubMed: Chen 2021, PubMed: Chen 2021, PubMed: Cohn 2010, PubMed: Kramer 2000, 2 more items - - CLASSIFICATION record, Germline, Unknown ?, yes Taiwan Biobank: 0; GnomAD_exome_East: 0; GnomAD_All: 0 - - - VKGL-NL_Nijmegen
+?/. 1 - c.25G>Y r.(?) p.? - likely pathogenic g.61719303G>Y - p.V9L - BEST1_000183 - PubMed: Meunier 2011 - - Germline - - - - - LOVD
+?/., ?/. 11 2 c.26T>C r.(?) p.(Val9Ala) - likely pathogenic, VUS g.61719304T>C g.61951832T>C BEST1 c.26T>C, p.(Val9Ala), BEST1 c.26T>C, p.Val9Ala, BEST1 p.Val9Ala, BEST1 T130C, V9A, 1 more item - BEST1_000244 heterozygous, no nucleotide annotation, extrapolated from databases heterozygous, 2 more items PubMed: Augstburger 2019, PubMed: Petrukhin 1998, PubMed: Querques 2009, PubMed: Querques 2011, 2 more items - - Germline, Unknown ?, yes 0/50 - - - LOVD
+/. 1 2 c.26T>G r.(?) p.(Val9Gly) ACMG likely pathogenic g.61719304T>G g.61951832T>G BEST1 c.26T>G, p.(Val9Gly) - BEST1_000383 heterozygous PubMed: Frecer 2019 SCV000599452 - Germline yes - - - - LOVD
+?/., ?/. 11 2 c.28G>A r.(?) p.(Ala10Thr) - likely pathogenic, VUS g.61719306G>A g.61951834G>A BEST1 c.28G>A, A10T, BEST1 c.28G>A, p.(Ala10Thr), BEST1 c.28G>A, p.Ala10Thr, BEST1 G28A, A10T, 4 more items - BEST1_000168 heterozygous, heterozygous, ACMG unclassified - no access to supplementary table 2, 1 more item PubMed: Alapati 2014, PubMed: Duncker 2014, PubMed: Hull 2020, PubMed: Ji 2019, PubMed: Kramer 2000, 4 more items - - Germline, Unknown ?, yes - - - - LOVD
+?/. 8 2 c.29C>T r.(?) p.(Ala10Val) ACMG likely pathogenic g.61719307C>T g.61951835C>T BEST1 c.29C>T, p.Ala10Val, BEST1 C133T, A10V, BEST1 p.Ala10Val - BEST1_000098 heterozygous, no nucleotide annotation, extrapolated from databases heterozygous, 2 more items PubMed: Bakall 1999, PubMed: Casalino 2020, PubMed: Querques 2011, PubMed: Querques 2014, 1 more item - - CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown ?, yes 0/53 controls, 1/2420 IRD families - - - Global Variome, with Curator vacancy, VKGL-NL_Nijmegen
+?/. 2 2 c.32A>T r.(?) p.(Asn11Ile) - likely pathogenic g.61719310A>T g.61951838A>T BEST1 c.32A>T, p.(Asn11Ile), VMD2 c.32A>T, Asn11Ile - BEST1_000245 heterozygous PubMed: Augstburger 2019, PubMed: Kramer 2003 - - Germline, Unknown ?, yes - - - - LOVD
+?/. 1 - c.33T>G r.(?) p.(Asn11Lys) - likely pathogenic g.61719311T>G g.61951839T>G BEST1 N11K - BEST1_000384 no nucleotide annotation, extrapolated from protein and databases; heterozygous PubMed: Nachtigal 2020 - - Unknown ? - - - - LOVD
+?/. 2 - c.35C>A r.(?) p.(Ala12Asp) ACMG likely pathogenic g.61719313C>A g.61951841C>A BEST1 c.[35C>A];[35=], V1: c.35C>A, (p.Ala12Asp), BEST1 c.[35C>A];[35=]; p.(Ala12Asp) - BEST1_000239 heterozygous PubMed: Chen 2021, PubMed: Chen 2021 - - Germline, Unknown ?, yes Taiwan Biobank: 0; GnomAD_exome_East: 0; GnomAD_All: 0 - - - LOVD
+?/. 2 - c.37C>G r.(?) p.(Arg13Gly) - likely pathogenic g.61719315C>G g.61951843C>G BEST1 c.37C>G;p.R13G, BEST1, variant 1: c.37C>G/p.R13G, variant 2: c.37C>G/p.R13G - BEST1_000220 heterozygous, possibly solved, homozygous PubMed: Nowomiejska 2021, PubMed: Weisschuh 2020 - - Unknown ? - - - - LOVD
+/., +?/. 9 2 c.37C>T r.(?) p.(Arg13Cys), p.R13C - likely pathogenic, pathogenic (dominant) g.61719279A>G, g.61719315C>T g.61951807A>G, g.61951843C>T Allele 1 c.37C>T (p.Arg13Cys), Allele 2 Wildtype, BEST1 c.37C>T, p.(Arg13Cys), 2 more items - BEST1_000006 heterozygous, homozygous, VKGL data sharing initiative Nederland PubMed: Augstburger 2019, PubMed: Birtel 2018, PubMed: Birtel 2020, PubMed: Khan 2019, 2 more items - rs886041141 CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown ?, yes - - - - VKGL-NL_Nijmegen
+/., +?/. 9 2 c.38G>A r.(?) p.(Arg13His) ACMG likely pathogenic, pathogenic g.61719316G>A g.61951844G>A BEST1 c.38G>A (p.R13H), BEST1 c.38G>A, p.(Arg13His), BEST1 c.38G>A, p.R13H, 2 more items - BEST1_000246 compound heterozygous, heterozygous, homozygous PubMed: Caldwell 1999, PubMed: Gao 2018, PubMed: Gao 2019, PubMed: Luo 2018, PubMed: Tian 2014, 1 more item - - Germline, Unknown ?, yes 0, 0/49 controls - - - LOVD
+?/. 3 - c.43G>C r.(?) p.(Gly15Arg) - likely pathogenic g.61719321G>C g.61951849G>C BEST1 G15R - BEST1_000356 heterozygous PubMed: Glavak 2016 - - Germline yes - - - - LOVD
+/., +?/. 10 2 c.44G>A r.(?) p.(Gly15Asp) ACMG likely pathogenic g.61719322G>A g.61951850G>A BEST1 c.44G>A, p.(Gly15Asp), BEST1 c.44G>A;p.G15D, BEST1 G>A44, G15D, BEST1 p.G15D, 1 more item - BEST1_000221 heterozygous, solved, heterozygous PubMed: Augstburger 2019, PubMed: Frecer 2019, PubMed: Kaden 2017, PubMed: Nowomiejska 2021, 2 more items - - Germline, Unknown ?, yes - - - - LOVD
+?/. 2 - c.47C>A r.(?) p.(Ser16Tyr) - likely pathogenic g.61719325C>A g.61951853C>A VMD2 c.47C>A, p.Ser16Tyr - BEST1_000069 heterozygous, VKGL data sharing initiative Nederland PubMed: Boon 2007 - - CLASSIFICATION record, Germline yes - - - - VKGL-NL_Nijmegen
+/., +?/., ?/. 23 2 c.47C>T r.(?) p.(Ser16Phe) ACMG likely pathogenic, VUS g.61719325C>T g.61951853C>T BEST1 151C>T, S16F, BEST1 c.47C>T, BEST1 c.47C>T, p.(Ser16Phe), BEST1 c.47C>T, p.S16F, 3 more items - BEST1_000169 heterozygous, no nucleotide annotation, writen, extrapolated from protein change; heterozygous, 1 more item PubMed: Alapati 2014, PubMed: Arora 2016, PubMed: Augstburger 2019, PubMed: de Souza 2019, 5 more items - rs281865210 Germline, Unknown ?, yes - - - - LOVD
+?/. 2 2 c.50T>G r.(?) p.(Phe17Cys) - likely pathogenic g.61719328T>G g.61951856T>G BEST1 154T>G, F17C, BEST1 TTC-TGC, Phe17Cys - BEST1_000247 heterozygous PubMed: Lotery 2000, PubMed: Marchant 2001 - - Unknown ? - - - - LOVD
+?/. 1 2 c.52dup r.(?) p.(Ser18Phefs*34) ACMG likely pathogenic g.61719330dup g.61951858dup BEST1 c.52dup, p.(Ser18Phefs*34) - BEST1_000385 1 more item PubMed: Gao 2019 - - Germline yes - - - - LOVD
+?/. 1 - c.55C>T r.(?) p.(Arg19Cys) - likely pathogenic g.61719333C>T g.61951861C>T BEST1 c.55C>T, p.R19C - BEST1_000386 heterozygous PubMed: Tian 2014 - - Unknown yes - - - - LOVD
+/., +?/. 5 2 c.58C>G r.(?) p.(Leu20Val) ACMG likely pathogenic, pathogenic g.61719336C>G g.61951864C>G BEST1 c.58C>G [p.Leu20Val], BEST1 c.58C>G, p.(Leu20Val) - BEST1_000199 heterozygous PubMed: Bitner 2012, PubMed: Gao 2019, PubMed: Guo 2018, PubMed: Jespersgaar 2019 - - Germline, Unknown ?, yes - - - - LOVD
+?/. 2 2 c.61C>G r.(?) p.(Leu21Val) - likely pathogenic g.61719339C>G g.61951867C>G BEST1 C61G, L21V - BEST1_000248 heterozygous PubMed: Kramer 2000 - - Germline yes - - - - LOVD
+/. 1 2 c.62T>G r.(?) p.(Leu21Arg) - pathogenic (dominant) g.61719340T>G g.61951868T>G - - BEST1_000122 - PubMed: Birtel 2018 - - Germline - - - - - LOVD
+?/. 1 - c.70T>C r.(70u>c) p.(Trp24Arg) ACMG likely pathogenic g.61719348T>C g.61951876T>C - - BEST1_000463 - - - - De novo - - - - - Oscar F Chacon-Camacho
+/. 1 - c.72G>A r.(?) p.(Trp24Ter) - pathogenic (recessive) g.61719350G>A g.61951878G>A - - BEST1_000167 - PubMed: Consugar 2015 - - Germline yes - - - - LOVD
+?/., ?/. 16 2 c.72G>T r.(?) p.(Trp24Cys) - likely pathogenic, VUS g.61719350G>T g.61951878G>T BEST1 c.72G>T;p.W24C, BEST1 TGG->TGT, W24C, BEST1 TGG-TGT, Trp24Cys, BEST1 W24C, 1 more item - BEST1_000170 heterozygous, solved, heterozygous PubMed: Alapati 2014, PubMed: Chacon-Camacho 2011, PubMed: Lotery 2000, PubMed: Marquardt 1998, 2 more items - - Germline, Unknown ?, yes - - - - LOVD
+/., +?/., ?/. 49 2, p.Arg25Trp c.73C>T r.(?) p.(Arg25Trp) - likely pathogenic, likely pathogenic (recessive), pathogenic, VUS g.61719351C>T g.61951879C>T BEST1 c.73C>T, p.(Arg25Trp), BEST1 c.73C>T, p.Arg25Trp, BEST1 c.73C>T, p.R25W, BEST1 R25W, 6 more items - BEST1_000007 heterozygous, heterozygous, ACMG unclassified - no access to supplementary table 2, 5 more items PubMed: Bernardis 2016, PubMed: Hull 2020, PubMed: Katagiri 2015, PubMed: Khan 2018, PubMed: Sodi 2007, 13 more items - - CLASSIFICATION record, Germline, Unknown ?, yes - - - - VKGL-NL_Nijmegen
+/., +?/. 8 2 c.74G>A r.(?) p.(Arg25Gln), p.(p.Arg25Gln) ACMG likely pathogenic, pathogenic g.61719352G>A g.61951880G>A BEST1 (NM_004183.3; OMIM: 607854): c.74G>A; p.Arg25Gln (hom) (ARB), ?/(ichtiosis), 3 more items - BEST1_000043 heterozygous, homozygous Sharon, submitted, PubMed: Boon 2013, PubMed: Casalino 2020, PubMed: Ehrenberg 2019, 3 more items - rs281865215 Germline, Germline/De novo (untested), Unknown ?, yes 2/2420 IRD families, A= 0.000008 - - - Global Variome, with Curator vacancy, Dror Sharon
+?/. 1 - c.76G>A r.(?) p.(Gly26Ser) - likely pathogenic g.61719354G>A g.61951882G>A BEST1 c.76G > A (p.Gly26Ser) - BEST1_000387 heterozygous PubMed: Campa 2020 - - Unknown ? - - - - LOVD
+?/. 1 2 c.76G>C r.(?) p.(Gly26Arg) - likely pathogenic g.61719354G>C g.61951882G>C VMD2 c.76G>C, Gly26Arg - BEST1_000249 heterozygous PubMed: Kramer 2003 - - Unknown ? - - - - LOVD
+/. 6 1, 2 c.80G>C r.(?) p.(Ser27Thr) ACMG likely pathogenic, pathogenic g.61719358G>C g.61951886G>C BEST1 c.80G>C, p.(Ser27Thr) - BEST1_000147 heterozygous PubMed: Bernardis 2016, PubMed: Frecer 2019 - - Germline, Unknown ?, yes - - - - LOVD
+?/. 1 2 c.81C>G r.(?) p.(Ser27Arg) - likely pathogenic g.61719359C>G g.61951887C>G BEST1 C81G, S27R - BEST1_000250 heterozygous PubMed: Kramer 2000 - - Unknown ? - - - - LOVD
+?/. 1 - c.82A>C r.(?) p.(Ile28Leu) - likely pathogenic g.61719360A>C g.61951888A>C BEST1 c.82A>C, p.(Ile28Leu) - BEST1_000388 heterozygous PubMed: Augstburger 2019 - - Unknown ? - - - - LOVD
+?/. 1 2 c.83T>A r.(?) p.(Ile28Asn) - likely pathogenic g.61719361T>A g.61951889T>A BEST1 c.83T>A, p.Ile28Asn - BEST1_000389 homozygous PubMed: Birtel 2020 - - Unknown ? - - - - LOVD
+?/. 1 2 c.85T>C r.(?) p.(Tyr29His) - likely pathogenic g.61719363T>C g.61951891T>C VMD2 c.85T>C, Tyr29His - BEST1_000251 heterozygous PubMed: Kramer 2003 - - Unknown ? - - - - LOVD
+/., +?/. 2 2 c.86A>G r.(?) p.(Tyr29Cys) ACMG likely pathogenic g.61719364A>G g.61951892A>G BEST1 c.86A>G, p.(Tyr29Cys), VMD2: 174A>G (Y29C) - BEST1_000156 heterozygous PubMed: Downs 2007, PubMed: Frecer 2019 - - Germline yes - - - - Julia Lopez
+?/. 8 2 c.87C>G r.(?) p.(Tyr29*) - likely pathogenic g.61719365C>G g.61951893C>G BEST1 c.87C>G, p.Tyr29stop, VMD2 Tyr29 stop, VMD2 Tyr29 stop, Arg141His - BEST1_000016, BEST1_000252 heterozygous, two dominant mutations in two alleles; compound heterozygous, 1 more item PubMed: Schatz 2006, PubMed: Schatz 2010 - - Germline yes - - - - LOVD
+/., +?/. 11 2 c.89A>G r.(?) p.(Lys30Arg) - likely pathogenic, pathogenic (dominant) g.61719367A>G g.61951895A>G BEST1 AAG-AGG, Lys30Arg, BEST1 c.89A>G:p.Lys30Arg, BEST1 c.89A>G;p.K30R, BEST1 Lys30Arg, ex, 2, 1 more item - BEST1_000099 heterozygous, no nucleotide annotation provided, extrapolated from protein and databases; heterozygous PubMed: Chung 2001, PubMed: Consugar 2015, PubMed: Holtan 2020, PubMed: Kay 2012, PubMed: Lotery 2000, 3 more items - - Germline, Unknown ?, yes 0, 2/899 cases - - - Global Variome, with Curator vacancy
+?/. 1 - c.90G>C r.(?) p.(Lys30Asn) - likely pathogenic g.61719368G>C g.61951896G>C BEST1 p.Lys30Asn - BEST1_000390 1 more item PubMed: Khan 2018 - - Unknown ? - - - - LOVD
+?/. 9 - c.91C>A r.(?) p.(Leu31Met) ACMG likely pathogenic g.61719369C>A g.61951897C>A BEST1 c.102C>T, p.Gly34Gly, BEST1 c.C91A [p.L31M], BEST1 p.(L31M) - BEST1_000391 heterozygous, homozygous, homozygous; mother/father/sister/brother, heterozygous; uncle, homozygous PubMed: Chibani 2019, PubMed: Habibi 2019, PubMed: Hufendiek 2020 - - Germline yes - - - - LOVD
?/. 1 - c.94C>G r.(?) p.(Leu32Val) ACMG VUS g.61719372C>G g.61951900C>G BEST1 nucleotide 1, protein 1:c.94C>G, p.Leu32Val - BEST1_000235 heterozygous, ACMG classified, novel (Table 2) PubMed: Hull 2020 - - Germline ? - - - - LOVD
?/. 1 - c.95T>C r.(?) p.(Leu32Pro) ACMG VUS g.61719373T>C g.61951901T>C BEST1 c.95T>C, p.(Leu32Pro), c.95T>C, p.(Leu32Pro) - BEST1_000200 homozygous PubMed: Jespersgaar 2019 - - Germline ? - - - - LOVD
+?/. 1 - c.97T>C r.(?) p.(Tyr33His) - likely pathogenic g.61719375T>C g.61951903T>C BEST1 c.97T>C, p.Y33H - BEST1_000357 compound heterozygous PubMed: Tian 2014 - - Germline yes - - - - LOVD
+?/. 9 2 c.102C>T r.(?), r.spl p.(Gly34=), p.Gly34= - likely pathogenic, likely pathogenic (recessive) g.61719279A>G, g.61719380C>T g.61951807A>G, g.61951908C>T BEST1 c.102C>T (p.Gly34Gly), BEST1 c.102C>T, G34G, BEST1 c.102C>T, p.G34G, 2 more items - BEST1_000131 heterozygous, homozygous, 2 more items PubMed: Casalino 2020, PubMed: Davidson 2010, PubMed: Gao 2018, PubMed: Khojasteh 2021, 2 more items - rs771898125 Germline, Germline/De novo (untested), Unknown ?, yes - - - - LOVD
+?/. 4 - c.103G>A r.(?) p.(Glu35Lys) - likely pathogenic g.61719381G>A g.61951909G>A BEST1 c.103G>A (p.Glu35Lys), BEST1 c.103G>A, p.E35K, BEST1 p.(E35K) - BEST1_000132 autosomal recessive bestrophinopathy phenotype, no second allele variant found; heterozygous, 1 more item PubMed: Habibi 2019, PubMed: Pfister 2021, PubMed: Stone 2017, PubMed: Tian 2014 - - Germline, Unknown ?, yes - - - - LOVD
-/., -?/. 5 2 c.109T>C r.(=), r.(?) p.(=), p.(Leu37=) - benign, likely benign g.61719387T>C g.61951915T>C BEST1(NM_004183.3):c.109T>C (p.L37=), BEST1(NM_004183.4):c.109T>C (p.L37=), 1 more item - BEST1_000003 VKGL data sharing initiative Nederland PubMed: Zhuk 2006 - rs1800007 CLASSIFICATION record, Germline - -72.70% - - - Julia Lopez, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen
+?/. 2 - c.113T>G r.(?) p.(Ile38Ser) ACMG likely pathogenic, VUS g.61719391T>G g.61951919T>G BEST1 c.T113G (p.Ile38Ser) - BEST1_000195 heterozygous PubMed: Jun 2017 - - Germline/De novo (untested), Unknown ? - - - - Jinu Han
+?/. 4 - c.119T>C r.(?) p.(Leu40Pro) - likely pathogenic g.61719397T>C g.61951925T>C BEST1 c.119T>C, L40P, BEST1 c.119T>C, Leu40Pro, BEST1 c.119T>C, p.L40P - BEST1_000358 autosomal recessive bestrophinopathy phenotype, no second allele variant found; heterozygous, 1 more item PubMed: Hwan Lee 2020, PubMed: Lee 2015, PubMed: Tian 2014 - - Germline, Unknown yes - - - - LOVD
+?/. 13 2, p.(Leu41Pro) c.122T>C r.(?) p.(Leu41Pro) - likely pathogenic g.61719400T>C g.61951928T>C BEST1 c.122T>C (p.Leu41Pro), BEST1 c.122T>C p.(Leu41Pro), BEST1 c.122T>C, (p.L41P), 3 more items - BEST1_000253 compound heterozygous, heterozygous, homozygous PubMed: Burgess 2008, PubMed: Casalino 2020, PubMed: Kramer 2003, PubMed: Pfister 2021, 2 more items - - Germline, Germline/De novo (untested), Unknown ?, yes - - - - LOVD
?/. 2 - c.128A>G r.(?) p.(Tyr43Cys) - VUS g.61719406A>G g.61951934A>G BEST1(NM_004183.4):c.128A>G (p.Y43C) - BEST1_000044 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen, VKGL-NL_AMC
+?/. 2 - c.130T>C r.(?) p.(Tyr44His) - likely pathogenic g.61719408T>C g.61951936T>C BEST1 c.130T>C, p.Y44H - BEST1_000392 heterozygous PubMed: Tian 2014 - - Unknown yes - - - - LOVD
+/., +?/. 5 - c.139C>T r.(?) p.(Arg47Cys) ACMG likely pathogenic, likely pathogenic (recessive), pathogenic g.61719417C>T g.61951945C>T BEST1 Arg47Cys, BEST1 c.139C>T, (p.Arg47Cys), BEST1 p.Arg47Cys (homozygous) - BEST1_000329 ACMG PM1, PP2, PM2, PM5, PP3, PP5; no variant 2nd chromosome, heterozygous, 2 more items PubMed: Khan 2018, PubMed: Kinnick 2011, PubMed: Sodi 2011, PubMed: Weisschuh 2024 305117 - Germline, Unknown ?, yes - - - - Johan den Dunnen
+/., +?/. 13 2 c.140G>A r.(?) p.(Arg47His) ACMG likely pathogenic, pathogenic, VUS g.61719418G>A g.61951946G>A BEST1 c.102C>T, p.Gly34Gly, BEST1 c.140 G>A, p.Arg47His, BEST1 c.140G>A, p.(Arg47His), 4 more items - BEST1_000196 heterozygous, heterozygous; parents not available, Homozygous, 1 more item PubMed: Gao 2018, PubMed: Gao 2019, PubMed: Hufendiek 2020, PubMed: Jespersgaar 2019, PubMed: Liu 2016, 4 more items - - Germline, Germline/De novo (untested), Unknown ?, yes - - - - Jinu Han
+?/. 2 - c.146T>A r.(?) p.(Ile49Asn) - likely pathogenic g.61719424T>A g.61951952T>A BEST1 c.146T>A, p.(Ile49Asn) - BEST1_000393 heterozygous PubMed: Gao 2019 - - Germline, Unknown ?, yes - - - - LOVD
-?/. 1 - c.152+6G>T r.(=) p.(=) - likely benign g.61719436G>T - BEST1(NM_001363592.1):c.152+6G>T - BEST1_000205 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.152+8T>C r.(=) p.(=) - likely benign g.61719438T>C - BEST1(NM_001139443.2):c.-29+1539T>C - BEST1_000478 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. 1 - c.153-8A>C r.(=) p.(=) - benign g.61722571A>C g.61955099A>C BEST1(NM_001139443.2):c.-28-8A>C - BEST1_000045 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+?/. 1 3 c.155T>C r.(?) p.(Leu52Pro) - likely pathogenic g.61722581T>C g.61955109T>C BEST1 c.155T>C, L52P - BEST1_000394 heterozygous PubMed: Gao 2018 - - Germline yes - - - - LOVD
+?/. 1 - c.169G>T r.(?) p.(Glu57Ter) - likely pathogenic g.61722595G>T g.61955123G>T BEST1 c.169G>T, p.Glu57Ter - BEST1_000469 heterozygous PubMed: Casalino 2020 - - Germline/De novo (untested) ? - - - - LOVD
+?/. 2 3 c.172_173dupCA r.(?) p.(Gln58Hisfs*4) - likely pathogenic (recessive) g.61722598_61722599dup g.61955126_61955127dup BEST1 c.172_173dupCA, p.Gln58HisfsX4 - BEST1_000330 compound heterozygous PubMed: Borman 2011 - - Germline yes - - - - LOVD
+?/. 1 - c.173A>T r.(?) p.(Gln58Leu) - likely pathogenic g.61722599A>T g.61955127A>T VMD2 Q58L - BEST1_000254 1 more item PubMed: Renner 2005 - - Unknown ? - - - - LOVD
+?/. 1 - c.174_176del r.(?) p.(Gln59del) - likely pathogenic g.61722600_61722602del g.61955128_61955130del BEST1 c.174_176del (p.Q158del) - BEST1_000395 1 more item PubMed: Luo 2018 - - Germline yes - - - - LOVD
+?/. 1 - c.175_176dup r.(?) p.(Gln59Hisfs*3) - likely pathogenic g.61722601_61722602dup g.61955129_61955130dup BEST1 c.175-176dup, p.Gln59Hisfs - BEST1_000343 homozygous PubMed: Boon 2013 - - Unknown ? - - - - LOVD
+?/. 1 3 c.176A>T r.(?) p.(Gln59Leu) - likely pathogenic g.61722602A>T g.61955130A>T BEST1 A176T, Q58L - BEST1_000255 1 more item PubMed: Kramer 2000 - - Unknown ? - - - - LOVD
+/., +?/. 2 - c.182del r.(?) p.(Met61Serfs*5), p.(Met61SerfsTer5) ACMG likely pathogenic, pathogenic g.61722608del g.61955136del - - BEST1_000093 ACMG PM2, PVS1_MODERATE, PP5_STRONG PubMed: Weisschuh 2024 987408 - Germline, Unknown - - - - - Johan den Dunnen, IMGAG
+?/. 2 - c.199_200del r.(?) p.(Leu67Valfs*164) ACMG pathogenic g.61722625_61722626del g.61955153_61955154del BEST1 c.102C>T, p.Gly34Gly - BEST1_000452 heterozygous; brother #16; parents not available, heterozygous; brother #17; parents not available PubMed: Hufendiek 2020 - - Germline yes - - - - LOVD
-/. 1 - c.201G>C r.(?) p.(Leu67=) - benign g.61722627G>C g.61955155G>C - - BEST1_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 1 - c.205T>C r.(?) p.(Cys69Arg) - likely pathogenic g.61722631T>C g.61955159T>C - - BEST1_000160 - PubMed: Huang 2015 - - Germline - - - - - LOVD
+?/. 3 - c.209A>G r.(?) p.(Asp70Gly) - likely pathogenic g.61722635A>G g.61955163A>G BEST1 M1: rs749295558, c.209A>G, p.(Asp70Gly) - BEST1_000396 - PubMed: Jaffal 2019 - rs749295558 Germline yes - - - - LOVD
-?/. 1 3 c.213C>T r.(=) p.(=) - likely benign g.61722639C>T - VMD2:c.317C>T (S71S) - BEST1_000153 - PubMed: Zhuk 2006 - - Germline - -9.09% - - - Julia Lopez
+?/. 2 - c.214T>C r.(?) p.(Tyr72His) ACMG likely pathogenic g.61722640T>C g.61955168T>C BEST1 c.[34T>C];[34=], V1: c.34T>C, (p.Tyr12His), BEST1 c.[34T>C];[34=]; p.(Tyr12His) - BEST1_000240 different transcript NM_001300787.2::c.34T>C, p.(Tyr12His); heterozygous, 1 more item PubMed: Chen 2021, PubMed: Chen 2021 - - Germline, Unknown ?, yes Taiwan Biobank: 0; GnomAD_exome_East: 0; GnomAD_All: 0 - - - LOVD
+?/. 1 - c.215A>T r.(?) p.(Tyr72Phe) - likely pathogenic g.61722641A>T g.61955169A>T BEST1 c.215A>T, p.(Tyr72Phe) - BEST1_000397 heterozygous PubMed: Augstburger 2019 - - Unknown ? - - - - LOVD
+?/., ?/. 2 p.Ile73Leu c.217A>C r.(?) p.(Ile73Leu) - likely pathogenic, VUS g.61722643A>C g.61955171A>C BEST1 c.217A>C, p.Ile73Leu - BEST1_000344 heterozygous, VKGL data sharing initiative Nederland PubMed: Sodi 2012 - - CLASSIFICATION record, Germline yes - - - - VKGL-NL_Nijmegen
+/. 1 3 c.217A>T r.(?) p.(Ile73Phe) - pathogenic g.61722643A>T - p.I73F - BEST1_000184 - PubMed: Meunier 2011 - - Germline - - - - - LOVD
+?/. 3 3 c.218T>A r.(?) p.(Ile73Asn) ACMG likely pathogenic g.61722644T>A g.61955172T>A BEST1 322T>A, I73N, BEST1 c.218T>A:p.Ile73Asn - BEST1_000100 heterozygous PubMed: Lima de Carvalho 2019, PubMed: Marchant 2001, PubMed: Sharon 2019 - - Germline, Unknown ? 1/2420 IRD families - - - Global Variome, with Curator vacancy
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