Global Variome shared LOVD
BBS1 (Bardet-Biedl syndrome 1)
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Unique variants in the BBS1 gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_024649.4 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.
214 entries on 3 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
-
c.(159+1_160-1)_(1110+1_1111-1)
r.(?)
p.(Leu54_Pro370del)
-
pathogenic (recessive)
g.?
g.?
BBS1 c.(159+1_160-1)_(1110+1_1111-1), p.(Leu54_Pro370del)
-
NPHS1_000138
compound heterozygous
PubMed: Delvallee 2021
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
-
c.-3291_1111-1020del
r.?
p.?
-
pathogenic
g.66274840_66292574del
-
del exon1_11
-
DPP3_000008
17,734 bp deletion, AluY - AluY
PubMed: Lindstrand 2016
-
-
Germline
-
-
-
-
-
LOVD
-/.
1
-
c.-1555G>A
r.(?)
p.(=)
-
benign
g.66276576G>A
g.66509105G>A
DPP3(NM_130443.3):c.2068G>A (p.E690K)
-
DPP3_000006
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
2i_12i
c.(?_-12-1)_(1110+1_?)del
r.(?)
p.?
-
pathogenic
g.(?_66278118)_(66291354_?)del
-
delexon1_11;het
-
BBS1_000219
-
PubMed: Lindstrand-2014
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/., ?/.
29
13, 16
c.?
r.(?), r.0?, r.?
p.(Asp278fs*9), p.(G318Vfs*61), p.(His504fs*48), p.0?, p.?, p.F534A, p.I296TfsX7, p.M242RfsX83
ACMG
likely pathogenic, pathogenic, pathogenic (recessive), VUS
g.66277761_66300761dup, g.66293652G>T, g.66298461_66298463del, g.?
g.66510290_66533290dup
c.[1169G>T];[1169G>T], C91fsX95/Y321X, D8D/R483X, L548fsX579, M390R/N524del†, p.(Asp278fs*9),
19 more items
-
DRD4_000002
normal 2nd chromosome
PubMed: Billingsley-2010
,
PubMed: Carrigan 2016
,
PubMed: Castro Sanchez 2015
,
PubMed: Deveault-2011
,
7 more items
-
-
Germline, Unknown
yes
0/96 ethnically matched controls
-
-
-
LOVD
+?/.
2
3
c.1A>G
r.?
p.?
-
likely pathogenic
g.66278131A>G
-
M1V, [p.M390R];[p.L505PfsX52]
-
BBS1_000188
-
PubMed: Deveault-2011
,
PubMed: Gerth-2008
-
-
Germline, Unknown
-
-
-
-
-
LOVD
-?/., ?/.
2
-
c.19T>A
r.(?)
p.(Ser7Thr)
-
likely benign, VUS
g.66278149T>A
g.66510678T>A
BBS1(NM_024649.5):c.19T>A (p.(Ser7Thr), p.S7T)
-
BBS1_000063
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
+?/., -/., ?/.
5
3
c.24T>C
r.(=), r.(?)
p.(=), p.(Asp8=)
-
benign, likely pathogenic, VUS
g.66278154T>C
g.66510683T>C
BBS1(NM_024649.5):c.24T>C (p.D8=), D8D/R483X, [p.M390R];[p.N524del]
-
BBS1_000064
VKGL data sharing initiative Nederland
PubMed: Deveault-2011
,
PubMed: Gerth-2008
,
PubMed: Tiwari 2016
-
-
CLASSIFICATION record, Germline, Unknown
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_AMC
+/.
3
3
c.39del
r.(?)
p.(Ala14Profs*61)
-
pathogenic
g.66278169del
-
c.39delA, c.[39del
-
BBS1_000173
-
PubMed: M'hamdi 2014
,
PubMed: Redin-2012
-
-
Germline, Unknown
yes
-
-
-
-
LOVD
+?/.
1
-
c.41C>G
r.(?)
p.(Ala14Gly)
-
likely pathogenic
g.66278171C>G
g.66510700C>G
BBS1, variant 1: c.1169T>G/p.M390R, variant 2: c.41C>G/p.A14G
-
BBS1_000229
possibly solved, compound heterozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
1
c.46A>T
c.46A>T
p.(Ser16Cys)
ACMG
likely pathogenic
g.66278176A>T
g.66510705A>T
BBS1 c.46A>T, p.(Ser16Cys)
-
BBS1_000189
heterozygous
PubMed: Manara 2019
-
rs772917364
Germline
?
-
-
-
-
LOVD
+?/.
1
-
c.47G>C
r.(?)
p.(Ser16Thr)
-
likely pathogenic
g.66278177G>C
-
BBS1(NM_024649.5):c.47G>C (p.S16T)
-
BBS1_000178
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
-
c.48-9C>A
r.(=)
p.(=)
-
VUS
g.66278475C>A
g.66511004C>A
BBS1(NM_024649.5):c.48-9C>A
-
BBS1_000111
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
-
c.48-3C>G
r.(?)
p.?
-
pathogenic (recessive)
g.66278481C>G
g.66511010C>G
-
-
BBS1_000166
-
PubMed: Ece Solmaz 2015
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.48-2A>C
r.spl?
p.?
-
likely pathogenic
g.66278482A>C
-
BBS1(NM_024649.5):c.48-2A>C
-
BBS1_000262
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., +?/.
2
2i
c.48-1G>A
r.spl?
p.?
-
likely pathogenic, pathogenic
g.66278483G>A
-
c.48-1G>A, IVS1-1g/a
-
BBS1_000171
-
PubMed: Harville-2010
,
PubMed: Knopp 2015
-
-
Germline
-
0/90 ethnically matched controls
-
-
-
LOVD
-/.
1
-
c.57C>G
r.(?)
p.(Ala19=)
-
benign
g.66278493C>G
g.66511022C>G
BBS1(NM_024649.5):c.57C>G (p.A19=)
-
BBS1_000112
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.68G>A
r.(?)
p.(Trp23*)
-
likely pathogenic
g.66278504G>A
g.66511033G>A
c.68G>A/p.(W23*)
-
BBS1_000185
homozygous
PubMed: Alvarez-Satta 2014
,
PubMed: Castro-Sanchez 2019
-
-
Germline
?
-
-
-
-
LOVD
?/.
1
-
c.75T>A
r.(?)
p.(Asp25Glu)
-
VUS
g.66278511T>A
g.66511040T>A
-
-
BBS1_000096
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs772010847
Germline
-
7/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+/.
3
-
c.118del
r.(?)
p.(Cys40Alafs*2), p.(Cys40AlafsTer2)
-
pathogenic, pathogenic (recessive), VUS
g.66278554del, g.68705674C>A
g.66511083del, g.68938206C>A
118delT
-
IGHMBP2_000003
-
PubMed: Bravo-Gil 2016
,
PubMed: Sanchez-Navarro 2018
-
-
Germline
-
-
-
-
-
Gerard C.P. Schaafsma
+/., +?/.
12
3i
c.124+1G>A
r.125_159del, r.spl?
p.?, p.L43Gfs*44
-
likely pathogenic, pathogenic
g.66278561G>A
-
c.124+1G>A r.125_159del p.(L43Gfs*44), IVS2+1g/a,
1 more item
-
BBS1_000172
-
PubMed: Abu-Safieh-2012
, Abu-Safieh 2010,
PubMed: Harville-2010
-
-
Germline
-
0/90 ethnically matched controls, 0/96 ethnically matched controls
-
-
-
LOVD
+?/.
1
2
c.124+2T>G
r.(?)
p.(Asn17Alafs*56)
-
likely pathogenic (recessive)
g.66278562T>G
g.66511091T>G
BBS1 c.124+2T>G, p.N17AfsX56
-
BBS1_000244
compound heterozygous; exon 2 skipping
PubMed: Katagiri 2020
-
-
Germline
yes
-
-
-
-
LOVD
-?/.
1
-
c.124+17A>G
r.(=)
p.(=)
-
likely benign
g.66278577A>G
-
BBS1(NM_024649.5):c.124+17A>G
-
BBS1_000250
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
2i_6i
c.(124+1_125-1)_(518+1_519-1)del
r.?
p.?
-
pathogenic
g.(66278561_66278675)_(66283203_66283331)del
g.(66511090_66511204)_(66515732_66515860)del
del ex3-6
-
chr11_008601
-
PubMed: Retterer 2016
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.159G>C
r.(?)
p.(Lys53Asn)
-
VUS
g.66278710G>C
-
-
-
BBS1_000170
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Jinu Han
?/.
1
-
c.163G>A
r.(?)
p.(Val55Met)
-
VUS
g.66281880G>A
g.66514409G>A
-
-
BBS1_000097
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs181765153
Germline
-
5/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
-
c.175C>G
r.(?)
p.(Leu59Val)
-
VUS
g.66281892C>G
-
BBS1(NM_024649.5):c.175C>G (p.(Leu59Val))
-
BBS1_000259
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.194A>G
r.(?)
p.(Gln65Arg)
-
VUS
g.66281911A>G
-
BBS1(NM_024649.5):c.194A>G (p.(Gln65Arg))
-
BBS1_000263
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
4
c.217G>T
r.(?)
p.(Gly73*)
-
pathogenic
g.66281934G>T
-
c.217G>T
-
BBS1_000190
-
PubMed: Muller-2010
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
3
4
c.223_224del
r.(?)
p.(Leu75Glyfs*23)
-
likely pathogenic, pathogenic
g.66281940_66281941del
g.66514469_66514470del
223_224delCT, c.223_224delCT(h)
-
BBS1_000153
-
PubMed: Janssen-2011
,
PubMed: Stone 2017
-
-
Germline
-
0.009
-
-
-
LOVD
-?/., ?/.
5
-
c.235G>A
r.(?)
p.(Glu79Lys)
-
likely benign, VUS
g.66281952G>A
g.66514481G>A
BBS1(NM_024649.4):c.235G>A (p.E79K), BBS1(NM_024649.5):c.235G>A (p.E79K, p.(Glu79Lys))
-
BBS1_000113
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
-?/.
2
-
c.243G>A
r.(?)
p.(Pro81=)
-
likely benign
g.66281960G>A
g.66514489G>A
BBS1(NM_024649.5):c.243G>A (p.P81=)
-
BBS1_000070
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
?/.
1
-
c.299G>A
r.(?)
p.(Arg100Gln)
-
VUS
g.66282016G>A
-
BBS1(NM_024649.5):c.299G>A (p.(Arg100Gln))
-
BBS1_000264
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/., -?/.
2
-
c.316C>G
r.(?)
p.(Leu106Val)
-
benign, likely benign
g.66282033C>G
g.66514562C>G
BBS1(NM_024649.4):c.316C>G (p.L106V), BBS1(NM_024649.5):c.316C>G (p.L106V)
-
BBS1_000065
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
+/.
1
6
c.320_332dup
r.(?)
p.(Val112Phefs*9)
-
pathogenic
g.66282037_66282049dup
-
320-332Dup13pb
-
BBS1_000191
-
PubMed: Muller-2010
-
-
Germline
-
-
-
-
-
LOVD
+?/., ?/.
3
4
c.329C>A
r.(?)
p.(Pro110His)
-
likely pathogenic, VUS
g.66282046C>A
g.66514575C>A
BBS1 c.329C>A, p.Pro110His, C329A
-
BBS1_000098
heterozygous
PubMed: Katagiri 2014
,
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
,
PubMed: Liu 2020
-
rs750288768
Germline, Germline/De novo (untested)
?
1/64, 2/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
-?/.
1
-
c.360C>T
r.(?)
p.(Pro120=)
-
likely benign
g.66282077C>T
g.66514606C>T
BBS1(NM_024649.4):c.360C>T (p.P120=)
-
BBS1_000066
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
2
-
c.363C>A
r.(?)
p.(Tyr121Ter)
-
pathogenic
g.66282080C>A
g.66514609C>A
BBS1(NM_024649.5):c.363C>A (p.Y121*)
-
BBS1_000067
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
-/., ?/.
5
4
c.378G>A
r.(=), r.(?)
p.(=), p.(Leu126=)
-
benign, VUS
g.66282095G>A
g.66514624G>A
BBS1(NM_024649.4):c.378G>A (p.L126=), BBS1(NM_024649.5):c.378G>A (p.L126=)
-
BBS1_000061
VKGL data sharing initiative Nederland
PubMed: Almomani 2011
{dbSNP:2298806},
PubMed: Smaoui 2006
-
rs2298806
CLASSIFICATION record, Germline
-
6/19 families BBS
-
-
-
Gerard C.P. Schaafsma
,
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+?/.
1
7
c.382C>T
r.(?)
p.(Gln128*)
-
likely pathogenic
g.66282099C>T
-
c.382C>T
-
BBS1_000192
-
PubMed: Muller-2010
, Beales 2003
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.432+1G>A
r.spl
p.(?)
-
pathogenic
g.66282150G>A
g.66514679G>A
BBS1 c.432+1G>A
-
BBS1_000222
heterozygous
PubMed: Zampaglione 2020
-
-
Unknown
?
-
-
-
-
LOVD
-?/.
1
-
c.432+13C>T
r.(=)
p.(=)
-
likely benign
g.66282162C>T
-
BBS1(NM_024649.5):c.432+13C>T
-
BBS1_000251
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.433-5C>T
r.spl?
p.?
-
likely benign
g.66283006C>T
g.66515535C>T
BBS1(NM_024649.4):c.433-5C>T (p.?)
-
BBS1_000114
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.433-3C>T
r.spl?
p.?
-
likely benign
g.66283008C>T
g.66515537C>T
BBS1(NM_024649.4):c.433-3C>T (p.?)
-
BBS1_000115
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., +?/.
15
7
c.436C>T
r.(?)
p.(Arg146*), p.(Arg146Ter), p.?
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.66283014C>T
g.66515543C>T
436C>T, 448C>T (Arg146Stop), BBS1(NM_024649.5):c.436C>T (p.R146*), c.436C>T, p.(Arg146*),
4 more items
-
BBS1_000116
solved, compound heterozygous, VKGL data sharing initiative Nederland,
1 more item
PubMed: Castro Sanchez 2015
,
PubMed: Fauser-2003
, Beales 2003,
PubMed: Feuillan-2011
,
7 more items
-
-
CLASSIFICATION record, Germline, Unknown
?, yes
0.009, 0.015
-
-
-
Johan den Dunnen
,
VKGL-NL_AMC
?/.
1
-
c.437G>A
r.(?)
p.(Arg146Gln)
-
VUS
g.66283015G>A
g.66515544G>A
-
-
BBS1_000099
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs759253107
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
-
c.437G>C
r.(?)
p.(Arg146Pro)
-
VUS
g.66283015G>C
g.66515544G>C
-
-
BBS1_000258
-
-
-
-
Germline
-
-
-
-
-
Tamar Ben-Yosef
+/.
1
7
c.442G>A
r.(?)
p.(Glu148Lys)
-
pathogenic
g.66283020G>A
-
c.442G>A
-
BBS1_000193
-
PubMed: Hoskins-2003
-
-
Germline
-
-
-
-
-
LOVD
+/.
3
7
c.448C>T
r.(?)
p.?
-
pathogenic (recessive)
g.66283026C>T
-
c.448C>T
-
DRD4_000002
-
PubMed: M'hamdi-2014
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.472A>G
r.(?)
p.(Ser158Gly)
-
VUS
g.66283050A>G
g.66515579A>G
-
-
BBS1_000100
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
-
c.478C>T
r.(?)
p.(Arg160Trp)
-
VUS
g.66283056C>T
g.66515585C>T
-
-
BBS1_000141
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/., ?/.
13
5, 6, 7
c.479G>A
r.(?)
p.(Arg160Gln), p.?
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.66283057G>A
g.66515586G>A
11:66283057G>A ENST00000318312.7:c.479G>A (Arg160Gln), BBS1(NM_024649.4):c.479G>A (p.R160Q),
5 more items
-
BBS1_000071
ACMG PP3, PM2, PP5_STRONG, Homozygous, normal 2nd chromosome, solved, homozygous,
1 more item
PubMed: Carss 2017
,
PubMed: Deveault-2011
,
PubMed: Duelund Hjortshoj-2010
,
PubMed: Hichri-2005
,
6 more items
370228
-
CLASSIFICATION record, Germline, Unknown
yes
2/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
Dror Sharon
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
-/.
1
-
c.479+20T>C
r.(=)
p.(=)
-
benign
g.66283077T>C
g.66515606T>C
BBS1(NM_024649.5):c.479+20T>C
-
BBS1_000069
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
2
5i
c.480-1G>C
r.spl?
p.?
-
pathogenic
g.66283163G>C
g.66515692G>C
BBS1(NM_024649.5):c.480-1G>C, c.IVS5-1G>C(H)
-
BBS1_000135
VKGL data sharing initiative Nederland
PubMed: Janssen-2011
-
-
CLASSIFICATION record, Germline
-
0.037
-
-
-
VKGL-NL_AMC
+/.
1
8
c.508C>T
r.(?)
p.(Gln170*)
-
pathogenic
g.66283192C>T
-
c.508C>T
-
BBS1_000194
-
PubMed: Muller-2010
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
8i
c.518+1G>A
r.(?)
p.?
-
VUS
g.66283203G>A
-
c.518+1G>A
-
BBS1_000206
-
PubMed: Chen-2011
-
-
Germline
-
-
-
-
-
LOVD
-?/.
1
-
c.519-20C>G
r.(=)
p.(=)
-
likely benign
g.66283312C>G
-
BBS1(NM_024649.5):c.519-20C>G
-
BBS1_000252
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+?/.
1
6i
c.519-2A>G
r.(?)
p.(?)
-
likely pathogenic
g.66283330A>G
g.66515859A>G
BBS1 IVS6 c.519-2A>G p.(?), Ex.12 c.1169T>G p.(Met390Arg)
-
BBS1_000195
compound heterozygous
PubMed: Martin Merida 2019
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
+?/.
1
-
c.526C>T
r.(?)
p.(Gln176Ter)
ACMG
likely pathogenic (recessive)
g.66283339C>T
g.66515868C>T
-
-
BBS1_000254
ACMG PM2, PVS1
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.533A>G
r.(?)
p.(Glu178Gly)
-
VUS
g.66283346A>G
g.66515875A>G
BBS1(NM_024649.5):c.533A>G (p.E178G)
-
BBS1_000078
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.564A>C
r.(?)
p.(Gln188His)
-
VUS
g.66283377A>C
g.66515906A>C
-
-
BBS1_000101
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
-
c.566A>G
r.(?)
p.(His189Arg)
-
VUS
g.66283379A>G
g.66515908A>G
-
-
BBS1_000102
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs749036245
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
-
c.567C>G
r.(?)
p.(His189Gln)
-
VUS
g.66283380C>G
g.66515909C>G
-
-
BBS1_000103
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs768596720
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+/.
2
-
c.589C>T
r.(?)
p.(Gln197Ter)
-
pathogenic
g.66283402C>T
g.66515931C>T
BBS1(NM_024649.4):c.589C>T (p.Q197*), BBS1(NM_024649.5):c.589C>T (p.Q197*)
-
BBS1_000079
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-?/.
1
-
c.591+9C>G
r.(=)
p.(=)
-
likely benign
g.66283413C>G
g.66515942C>G
BBS1(NM_024649.5):c.591+9C>G
-
BBS1_000080
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
7i
c.592-59G>A
r.spl?
p.?
-
pathogenic
g.66287029G>A
-
c.592-59G>A/N
-
BBS1_000224
normal 2nd chromosome
PubMed: Esposito 2017
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
5
8
c.592-21A>T
r.(=), r.[(592_621del,592_723del,=)]
p.(=), p.[(Thr198_Lys207del,Thr198_Lys241del,=)]
-
likely pathogenic, pathogenic (recessive)
g.66287067A>T
g.66519596A>T
-
-
BBS1_000165
effect on splicing predicted from minigene splicing assay, VKGL data sharing initiative Nederland
PubMed: Fadaie 2022
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
VKGL-NL_Nijmegen
,
Zeinab Fadaie
+/.
2
7i_10i
c.592-1_830+1del
r.spl?
p.?
-
pathogenic (recessive)
g.66287087_66288848del
-
c.[592-?_830+?del];[436C>T]
-
BBS1_000235
-
PubMed: Mary-2019
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.599_604del
r.(?)
p.(Ile200_Thr201del)
-
likely pathogenic
g.66287095_66287100del
g.66519624_66519629del
597_602delCATCAC
-
BBS1_000154
-
PubMed: Stone 2017
-
-
Germline
-
-
-
-
-
LOVD
-?/., ?/.
3
9
c.616T>G
r.(?)
p.(Leu206Val)
-
likely benign, VUS
g.66287112T>G
g.66519641T>G
BBS1(NM_024649.5):c.616T>G (p.L206V), c.616T>G(h)
-
BBS1_000117
VKGL data sharing initiative Nederland
PubMed: Janssen-2011
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Utrecht
+/.
2
9
c.664G>C
r.(?)
p.(Val222Leu)
-
pathogenic
g.66287160G>C
-
c.664G>C/c.664G>C (p.G222R)
-
BBS1_000225
-
PubMed: Esposito 2017
-
-
Germline
-
-
-
-
-
LOVD
+/., ?/.
4
9
c.670G>A
r.(?)
p.(Glu224Lys), p.?
ACMG
pathogenic, VUS
g.66287166G>A
g.66519695G>A
c.[670G>A];[670G>A]
-
BBS1_000140
1 heterozygous, no homozygous;
Clinindb (India)
, ACMG PP3, PM2, PP5
PubMed: Narang 2020
,
Journal: Narang 2020
,
PubMed: Redin-2012
,
PubMed: Weisschuh 2024
-
rs193922709
Germline
-
1/2794 individuals
-
-
-
Johan den Dunnen
,
Mohammed Faruq
+?/., -/., -?/., ?/.
7
9
c.700G>A
r.(?)
p.(Glu234Lys)
-
benign, likely benign, likely pathogenic, VUS
g.66287196G>A
g.66519725G>A
BBS1(NM_024649.4):c.700G>A (p.E234K, p.(Glu234Lys)), BBS1(NM_024649.5):c.700G>A (p.E234K), E234K
-
BBS1_000118
4 heterozygous, no homozygous;
Clinindb (India)
, VKGL data sharing initiative Nederland
PubMed: Bryant 2018
,
PubMed: Eichers-2009
, Badano 2003,
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs35520756
CLASSIFICATION record, Germline
-
4/2793 individuals
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
,
Mohammed Faruq
+?/.
1
8
c.723+2T>G
r.(?)
p.(Thr198_Lys241del)
-
likely pathogenic (recessive)
g.66287221T>G
g.66519750T>G
BBS1 c.723+2T>G, p.T198_K241del
-
BBS1_000245
compound heterozygous; exon 8 skipping
PubMed: Katagiri 2020
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
-
c.724-358_1165del
r.?
p.?
-
pathogenic
g.66288383_66293648del
-
del exon9_12 66288383-66293649del
-
BBS1_000158
5,266 bp deletion, 1 bp microhomology (T)
PubMed: Lindstrand 2016
-
-
Germline
-
-
-
-
-
LOVD
-/.
1
-
c.724-17G>T
r.(=)
p.(=)
-
benign
g.66288724G>T
g.66521253G>T
BBS1(NM_024649.5):c.724-17G>T
-
BBS1_000119
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.724-16C>T
r.(=)
p.(=)
-
likely benign
g.66288725C>T
g.66521254C>T
BBS1(NM_024649.5):c.724-16C>T
-
BBS1_000136
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.724-13T>G
r.(=)
p.(=)
-
benign
g.66288728T>G
g.66521257T>G
BBS1(NM_024649.5):c.724-13T>G
-
BBS1_000120
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
4
-
c.724-8G>C
r.(=)
p.(=)
-
benign
g.66288733G>C
g.66521262G>C
BBS1(NM_024649.4):c.724-8G>C, BBS1(NM_024649.5):c.724-8G>C
-
BBS1_000072
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
+?/., -?/.
2
10
c.734C>T
r.(?)
p.(Pro245Leu)
-
likely benign, likely pathogenic
g.66288751C>T
-
BBS1(NM_024649.5):c.734C>T (p.P245L), c.734C>T(h)
-
BBS1_000220
unknown variant 2nd chromosome, VKGL data sharing initiative Nederland
PubMed: Janssen-2011
-
-
CLASSIFICATION record, Germline
-
1.6% ; absent in 96 controls
-
-
-
VKGL-NL_Utrecht
-/.
1
-
c.736-8G>C
r.(?)
p.(=)
-
benign
g.66288745G>C
g.66521274G>C
-
-
BBS1_000248
-
PubMed: Smaoui 2006
-
rs10896125
Germline
-
2/19 families BBS
-
-
-
Johan den Dunnen
?/.
2
-
c.742C>A
r.(?)
p.(Pro248Thr)
-
VUS
g.66288759C>A
-
BBS1(NM_024649.4):c.742C>A (p.P248T), BBS1(NM_024649.5):c.742C>A (p.P248T)
-
BBS1_000121
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-/.
1
-
c.744C>T
r.(?)
p.(Pro248=)
-
benign
g.66288761C>T
g.66521290C>T
BBS1(NM_024649.5):c.744C>T (p.P248=)
-
BBS1_000122
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
2
-
c.751C>G
r.(?)
p.(Leu251Val)
-
VUS
g.66288768C>G
-
BBS1(NM_024649.4):c.751C>G (p.L251V), BBS1(NM_024649.5):c.751C>G (p.(Leu251Val))
-
BBS1_000148
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
+/.
1
-
c.767del
r.(?)
p.(Gln256Argfs*20)
ACMG
pathogenic
g.66288784del
-
c.767delA
-
BBS1_000143
-
PubMed: Sharon 2019
-
-
Germline
-
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
?/.
1
-
c.785G>A
r.(?)
p.(Arg262Gln)
-
VUS
g.66288802G>A
g.66521331G>A
-
-
BBS1_000104
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs747282202
Germline
-
3/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
?/.
1
-
c.785G>C
r.(?)
p.(Arg262Pro)
-
VUS
g.66288802G>C
-
BBS1(NM_024649.5):c.785G>C (p.(Arg262Pro))
-
BBS1_000260
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
2
-
c.815T>C
r.(?)
p.(Ile272Thr)
-
VUS
g.66288832T>C
g.66521361T>C
BBS1 c.815T>C, p.Ile272Thr, c.815T>C, p.Ile272Thr
-
BBS1_000223
heterozygous, homozygous
PubMed: Zampaglione 2020
-
-
Unknown
?
-
-
-
-
LOVD
+/., +?/.
3
10
c.(830G>A), c.830G>A
r.(?)
p.(Arg277Lys), p.(Gly277Glu)
-
likely pathogenic, pathogenic
g.66288847G>A
g.66521376G>A
BBS1 p.R277K, c.830G>A
-
BBS1_000207
no nucleotide annotation, extrapolated from protein and databases; heterozygous
PubMed: Feuillan-2011
,
PubMed: Jacobson 2014
,
PubMed: Panneman 2023
-
-
Germline, Unknown
?
-
-
-
-
Daan Panneman
+/.
1
10i
c.830+1G>A
r.spl?
p.?
-
pathogenic
g.66288848G>A
-
IVS9+1G>A
-
BBS1_000196
-
PubMed: Fauser-2003
-
-
Unknown
-
-
-
-
-
LOVD
+/.
1
-
c.831-797_*5514del
r.0?
p.0?
-
pathogenic
g.66290130_66305022del
-
del exon10_17
-
BBS1_000160
14,892 bp deletion
PubMed: Lindstrand 2016
-
-
Germline
-
-
-
-
-
LOVD
-?/.
2
-
c.831-5C>T
r.spl?
p.?
-
likely benign
g.66290922C>T
g.66523451C>T
BBS1(NM_024649.4):c.831-5C>T (p.?)
-
BBS1_000123
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
+?/.
1
-
c.(830+1_831-1)_(1180+1_1181-1)del
r.?
p.(Asp278Trpfs*20)
-
pathogenic
g.(66288848_66290926)_(66293664_66294119)del
g.(66521377_66523455)_(66526193_66526648)del
831-?_1180+?del
-
BBS1_000174
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Jinu Han
+?/.
1
-
c.831_1110del
r.(?)
p.(Asp278Metfs*3)
-
likely pathogenic
g.66290927_66291353del
g.66523456_66523882del
BBS1 Exon 10-11del, p.R429X
-
BBS1_000226
1 more item
PubMed: Hirano 2020
-
-
Germline
?
-
-
-
-
LOVD
+?/.
1
10
c.837del
r.(?)
p.(Arg280Alafs*18)
-
likely pathogenic
g.66290933del
-
c.836delC/p.(Asp278fs*9)b
-
BBS1_000221
-
PubMed: Alvarez-Satta-2014
-
-
Germline
-
0/100 chromosomes
-
-
-
LOVD
+/.
2
10
c.851del
r.(?)
p.(Asp284Alafs*14), p.(Tyr284SerfsTer5)
-
pathogenic, pathogenic (recessive)
g.66290947del
g.66523476del
851delA, c.851delA
-
BBS1_000167
-
PubMed: Ece Solmaz 2015
,
PubMed: Hoskins-2003
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
3
10
c.863T>G
r.(?)
p.(Leu288Arg)
-
likely pathogenic, pathogenic
g.66290959T>G
-
c.863T>G, c.863T>G/p.(Leu288Arg), p.(Leu288Arg)
-
BBS1_000197
-
PubMed: Alvarez-Satta-2014
,
PubMed: Castro Sanchez 2015
,
PubMed: Muller-2010
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
3
10
c.871C>T
r.(?)
p.(Gln291*)
-
likely pathogenic, pathogenic
g.66290967C>T
-
BBS1(NM_024649.4):c.871C>T (p.Q291*, p.(Gln291*)), c.871C>T(h)
-
BBS1_000149
unknown variant 2nd chromosome, VKGL data sharing initiative Nederland
PubMed: Janssen-2011
-
-
CLASSIFICATION record, Germline
-
2.9% ; absent in 96 controls
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
?/.
1
-
c.887T>C
r.(?)
p.(Ile296Thr)
-
VUS
g.66290983T>C
g.66523512T>C
BBS1(NM_024649.5):c.887T>C (p.I296T)
-
BBS1_000081
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
11
c.889C>T
r.(?)
p.(Arg297Trp)
-
VUS
g.66290985C>T
-
c.889C>T
-
BBS1_000208
-
PubMed: Abu-Safieh-2012
-
-
Germline
-
-
-
-
-
LOVD
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