Global Variome shared LOVD
BEST1 (bestrophin 1)
LOVD v.3.0 Build 30b [
Current LOVD status
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Curators:
Heidi L. Schulz
and
Claire-Marie Dhaenens
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Unique variants in the BEST1 gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_004183.3 transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Reported
: The number of times this variant has been reported in the database.
Exon
: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA)
: description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change
: description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein
: description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Classification method
: The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification
: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19)
: HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38)
: HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as
: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN
: description of the variant according to ISCN nomenclature
DB-ID
: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks
: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference
: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID
: ID of variant in ClinVar database
dbSNP ID
: the dbSNP ID
Origin
: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation
: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency
: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site
: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP
: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator. NOTE: to get VIP status ask the curator.
Methylation
: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
How to query this table
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Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
all entries containing 'Arg' and 'Ser'
|
Text
Arg|Ser
all entries containing 'Arg' or 'Ser'
!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
=""
all entries with this field empty
=""
Text
="p.0"
all entries exactly matching 'p.0'
!=""
Text
!=""
all entries with this field not empty
!=""
Text
!="p.0"
all entries not exactly matching 'p.0?'
combination
Text
*|Ter !fs
all entries containing '*' or 'Ter' but not containing 'fs'
Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
!
Date
!2020-03
all entries not matching March, 2020
<
Date
<2020
all entries before the year 2020
<=
Date
<=2020-06
all entries in or before June, 2020
>
Date
>2020-06
all entries after June, 2020
>=
Date
>=2020-06-15
all entries on or after June 15th, 2020
combination
Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
all entries exactly matching 23
|
Numeric
23|24
all entries exactly matching 23 or 24
!
Numeric
!23
all entries not exactly matching 23
<
Numeric
<23
all entries lower than 23
<=
Numeric
<=23
all entries lower than, or equal to, 23
>
Numeric
>23
all entries higher than 23
>=
Numeric
>=23
all entries higher than, or equal to, 23
combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
Example
Matches
Asian
all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
To sort on a certain column, click on the column header or on the arrows. If that column is already selected to sort on, the sort order will be swapped. The column currently sorted on has a darker blue background color than the other columns. The up and down arrows next to the column name indicate the current sorting direction. When sorting on any field other than the default, LOVD will sort secondarily on the default sort column.
496 entries on 5 pages. Showing entries 1 - 100.
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Effect
Reported
Exon
DNA 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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