Global Variome shared LOVD
BBS10 (Bardet-Biedl syndrome 10)
LOVD v.3.0 Build 30b [
Current LOVD status
]
Register as submitter
|
Log in
Curator:
Timothy Barrett
View all genes
View BBS10 gene homepage
View graphs about the BBS10 gene database
Create a new gene entry
View all transcripts
View all transcripts of gene BBS10
Create a new transcript information entry
View all variants
View all variants affecting transcripts
View unique variants in gene BBS10
View all variants in gene BBS10
Full data view for gene BBS10
Create a new data submission
View active genomic custom columns
Enable more genomic custom columns
View all individuals
View all individuals with variants in gene BBS10
Create a new data submission
View active custom columns
Enable more custom columns
View all diseases
View all diseases associated with gene BBS10
Create a new disease information entry
View available phenotype columns
View all screenings
View all screenings for gene BBS10
Create a new data submission
View active custom columns
Enable more custom columns
Submit new data
Unique variants in the BBS10 gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_024685.3 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
All list views have search fields which can be used to search data. You can search for a complete word or you can search for a part of a search term. If you enclose two or more words in double quotes, LOVD will search for the combination of those words only exactly in the order you specify. Note that search terms are case-insensitive and that wildcards such as * are treated as normal text! For all options, like "and", "or", and "not" searches, or searching for prefixes or suffixes, see the table below.
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.
160 entries on 2 pages. Showing entries 1 - 100.
10 per page
25 per page
50 per page
100 per page
Legend
How to query
« First
Prev
1
2
Next
Last »
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
1
c.-52C>T
r.(=)
p.(=)
-
VUS
g.76742190G>A
-
c.-52C>T
-
BBS10_000188
-
PubMed: Abu-Safieh-2012
, Abu-Safieh 2010
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/., ?/.
60
2
c.?
r.(?)
p.(Cys91fs*4), p.(Glu337del), p.(Leu533Leufs*21), p.(Tyr503fs*17), p.(Tyr559*), p.(Tyr559fs*17), p.?,
9 more items
-
likely pathogenic, pathogenic, VUS
g.?
g.?
(c.157-3C>G)+(c.157-3C>G), c.1090del>A, c.272 insertion T, c.687delA(h), C91fsX95/C91fsX95,
35 more items
-
ALX1_000001
no real mutation annotation, most probably a known c.271dup, p.(Cys91Leufs*5) variant; homozygous,
2 more items
PubMed: Billingsley-2010
,
PubMed: Castro Sanchez 2015
,
PubMed: Chen-2011
,
PubMed: Deveault-2011
,
10 more items
-
-
Germline, Unknown
yes
0/96 ethnically matched controls, 50 control individuals
-
-
-
LOVD
+/.
1
1
c.9_14del
r.(?)
p.(Ser3_Met5delinsArg)
-
pathogenic
g.76742125_76742130del
-
c.9_14delTTCTAT(p.Ser3_Met5delinsArg);het
-
BBS10_000199
-
PubMed: Lindstrand-2014
-
-
Germline
-
-
-
-
-
LOVD
+/.
2
1
c.9_15delinsGC
r.(?)
p.(Ser3Argfs*91)
-
pathogenic
g.76742124_76742130delinsGC
g.76348344_76348350delinsGC
c.9_15delinsGC(p.S3Rfs*91)
-
BBS10_000116
-
PubMed: Wang 2016
,
PubMed: Zenteno 2020
-
-
Germline
-
1/143 cases
-
-
-
Johan den Dunnen
+?/.
1
1
c.32T>G
r.(?)
p.(Val11Gly)
-
likely pathogenic
g.76742107A>C
-
S311A/V11G
-
BBS10_000139
-
PubMed: Laurier-2006
-
-
Germline
yes
0/107 Lebanese controls; 0/96 French controls
-
-
-
LOVD
+?/.
1
-
c.39_46del
r.(?)
p.(Ala14Glyfs*79)
-
likely pathogenic
g.76742095_76742102del
g.76348315_76348322del
BBS10 c.39_46delGGCGTTGC
-
BBS10_000222
homozygous
PubMed: Ladino 2018
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
1
c.92C>T
r.(?)
p.(Pro31Leu)
-
likely pathogenic
g.76742047G>A
-
c.92C>T
-
BBS10_000187
-
PubMed: Feuillan-2011
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
2
-
c.98G>A
r.(?)
p.(Gly33Glu)
-
likely pathogenic, pathogenic
g.76742041C>T
g.76348261C>T
BBS10 c.98G>A, p.(G33E), BBS10 p.G33E, p.R709X
-
BBS10_000205
heterozygous, no c. position written in publication, probable position given - c.98G>A
PubMed: Hirano 2020
,
PubMed: Kurata 2018
-
-
Germline
?, yes
-
-
-
-
LOVD
?/.
1
-
c.101G>C
r.(?)
p.(Arg34Pro)
-
VUS
g.76742038C>G
-
BBS10(NM_024685.4):c.101G>C (p.R34P)
-
BBS10_000217
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.124A>T
r.(?)
p.(Thr42Ser)
-
VUS
g.76742015T>A
g.76348235T>A
BBS10(NM_024685.4):c.124A>T (p.T42S)
-
BBS10_000109
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
1
c.128A>G
r.(?)
p.?
-
pathogenic
g.76742011T>C
-
c.128A>G
-
BBS10_000158
A not found at position given, found G instead.
PubMed: Muller-2010
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/., ?/.
34
1
c.145C>T
r.(?)
p.(Arg49Trp)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.76741994G>A
g.76348214G>A
BBS10 c.145C>T, p.(Arg49Trp), BBS10 c.145C>T, p.(Arg49Trp), c.145C>T, c.145C>T (p.R49W),
10 more items
-
BBS10_000097
ACMG PM2, PP5_STRONG, compound heterozygous, heterozygous, homozygous, normal 2nd chromosome,
2 more items
PubMed: Billingsley-2010
,
PubMed: Chen-2011
,
PubMed: Delvallee 2021
,
PubMed: Deveault-2011
,
13 more items
225010
-
CLASSIFICATION record, Germline, Unknown
?, yes
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
VKGL-NL_VUmc
+/., +?/.
7
1
c.164T>C
r.(?)
p.(Leu55Pro)
-
likely pathogenic, pathogenic, VUS
g.76741975A>G
g.76348195A>G
BBS10(NM_024685.4):c.164T>C (p.L55P), c.164T>C, [L55P]+[E274VfsX29], [p.R413X];[p.R413X]
-
BBS10_000096
VKGL data sharing initiative Nederland
PubMed: Billingsley-2010
,
PubMed: Chen-2011
,
PubMed: Deveault-2011
-
-
CLASSIFICATION record, Germline, Unknown
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_VUmc
,
VKGL-NL_AMC
+?/.
1
1
c.180dup
r.(?)
p.(Glu61Argfs*35)
-
likely pathogenic
g.76741959dup
-
c.180dupA
-
BBS10_000186
-
PubMed: Feuillan-2011
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.185A>G
r.(?)
p.(His62Arg)
-
likely pathogenic
g.76741954T>C
g.76348174T>C
BBS10 c.185A>G, p.His62Arg
-
BBS10_000221
heterozygous
PubMed: Putoux 2010
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
1i
c.197+1G>T
r.spl?
p.?
-
likely pathogenic
g.76741941C>A
-
[p.K188T; p.A636V];[p.K188T; p.A636V]
-
BBS10_000185
-
PubMed: Deveault-2011
-
-
Unknown
-
-
-
-
-
LOVD
-?/.
1
-
c.198-3C>T
r.spl?
p.?
-
likely benign
g.76741570G>A
g.76347790G>A
BBS10(NM_024685.4):c.198-3C>T
-
BBS10_000095
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
2
2
c.198G>T
r.(?)
p.(Arg66Ser)
-
pathogenic (recessive)
g.76741567C>A
-
c.[198G>T];[198G>T]
-
BBS10_000214
-
PubMed: Mary-2019
-
-
Germline
-
-
-
-
-
LOVD
+?/.
2
2
c.219_229del
r.(?)
p.(Ser74Lysfs*18)
-
likely pathogenic
g.76741536_76741546del
-
c.219delCAGTCATCTCA
-
BBS10_000157
-
PubMed: Muller-2010
,
PubMed: Stoetzel 2006
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.233G>A
r.(?)
p.(Cys78Tyr)
-
pathogenic
g.94768015G>A
g.93755787G>A
TMEM67 c.233G>A, p.(Cys78Tyr)
-
TMEM67_000217
heterozygous
PubMed: Barabino 2020
-
-
In vitro (cloned)
?
-
-
-
-
LOVD
+/., +?/.
3
2
c.235dup
r.(?)
p.(Thr79Asnfs*17)
ACMG
likely pathogenic, pathogenic
g.76741530dup, g.76741536dup
g.76347756dup
235dupA, BBS10 c.235dup, p.Thr79Asnfs*17, c.235dupA/c.271dupT (T79Nfs*17/p.C91LfsX5)
-
BBS10_000129
heterozygous
PubMed: Barrell 2019
,
PubMed: Esposito 2017
-
-
Germline, Germline/De novo (untested), In vitro (cloned)
?
-
-
-
-
Jinu Han
+/.
1
-
c.270_271insT
r.(?)
p.(Cys91Leufs*5)
-
pathogenic (recessive)
g.76741494_76741495insA
-
12:76741493C>CA ENST00000393262.3:c.271dupT (Cys91LeufsTer5)
-
BBS10_000118
-
PubMed: Carss 2017
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/., ?/.
76
2
c.271dup
c.271dup, r.(?)
p.(Cys91Leufs*5), p.(Cys91LeufsTer5)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.76741494dup, g.76741496dup
g.76347714dup, g.76347716dup
268_269insT, 271dupT, BBS10 c.271dup, p.(Cys91Leufs*5), BBS10 c.271dupT, p.Cys91LeufsTer5, c.271dupT,
12 more items
-
BBS10_000002, BBS10_000092
ACMG PM2, PVS1_STRONG, PP5, PS4, founder mutation, heterozygous, homozygous, solved, homozygous,
4 more items
PubMed: Alvarez-Satta-2014
,
PubMed: Barabino 2020
,
PubMed: Duelund Hjortshoj-2010
,
PubMed: Holtan 2020
,
18 more items
-
rs549625604
CLASSIFICATION record, Germline, Germline/De novo (untested), In vitro (cloned), Unknown
?, yes
1/899 cases, 6/899 cases
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
Karen Stals
,
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
VKGL-NL_VUmc
,
VKGL-NL_AMC
+?/., ?/.
2
2
c.271dupG
r.(?)
p.?
-
likely pathogenic, VUS
g.76741494dupC
-
(c.271dupG; p.Cys91fs), R49W/L414S
-
BBS10_000184
-
PubMed: Dulfer-2010
-
-
Unknown
-
-
-
-
-
LOVD
+/., +?/.
8
-
c.271dupT
r.(?)
p.(Cys91Leufs*5)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.76741496dup
g.76347716dup
BBS10 c.271dupT, p.(Cys91Leufs*5), BBS10 c.271dupT, p.Cys91LeufsX5, BBS10 c.271insT, p.(Cys91fsX95)
-
BBS10_000001, BBS10_000002
compound heterozygous, father's DNA unavaiable; heterozygous, heterozygous, homozygous
PubMed: Brunbjerg-Hey 2018
,
PubMed: Delvallee 2021
,
PubMed: Paolacci 2019
,
PubMed: Putoux 2010
-
-
Germline, Germline/De novo (untested), In vitro (cloned)
?, yes
-
-
-
-
LOVD
+?/.
2
2
c.271_272insT
r.(?)
p.(Cys91Leufs*5)
-
likely pathogenic
g.76741493_76741494insA
-
c.271_272insT
-
BBS10_000183
-
PubMed: Schaefer-2011
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
-
c.272dup
r.(?)
p.(Cys91Trpfs*5)
-
pathogenic
g.76741493dup
g.76347714dup
-
-
BBS10_000110
1 more item
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs549625604
Germline
-
3/2795 individuals
-
-
-
Mohammed Faruq
+/., +?/.
15
2
c.272_273insT
r.(?)
p.(His92Profs*4), p.(His92ProfsTer4)
ACMG
likely pathogenic, pathogenic, VUS
g.76741492_76741493insA
g.76347712_76347713insA
BBS10 c.272_273insT, p.C91Lfs*5, c.272 insertion T, c.272>insertion>T, R49W/R49W,
8 more items
-
BBS10_000094
1 more item
PubMed: Chen-2011
,
PubMed: Deveault-2011
,
PubMed: Deveault-2012
,
PubMed: Zacchia 2021
-
-
Germline, Unknown
?
-
-
-
-
LOVD
+/., +?/.
13
2
c.273C>G
r.(?)
p.(Cys91Trp)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.76741492G>C
g.76347712G>C
BBS10 c.273C>G, p.C91W, BBS10 c.273C>G, p.Cys91Trp, c.273C>G, c.[1044_1045del];[273C>G],
6 more items
-
BBS10_000113
heterozygous, heterozygous; unsolved, VKGL data sharing initiative Nederland
PubMed: Billingsley-2010
,
PubMed: Bravo-Gil 2016
,
PubMed: Deveault-2011
,
PubMed: Gerth-2008
,
4 more items
-
-
CLASSIFICATION record, Germline, Unknown
?, yes
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/.
2
2
c.285A>T
r.(?)
p.(Arg95Ser)
-
likely pathogenic, pathogenic
g.76741480T>A
g.76347700T>A
BBS10 c.285A>T, p.Arg95Ser, c.285A>T
-
BBS10_000182
heterozygous
PubMed: Barrell 2019
,
PubMed: Redin-2012
-
-
Germline, In vitro (cloned)
?
-
-
-
-
LOVD
?/.
1
2
c.285A>Y
r.(?)
p.(Arg95Ser)
-
VUS
g.76741480T>G
-
p.R95S
-
BBS10_000201
-
PubMed: Scheidecker 2015
-
-
Germline
-
-
-
-
-
LOVD
-/., -?/.
2
-
c.372T>A
r.(?)
p.(Ser124=)
-
benign, likely benign
g.76741393A>T
g.76347613A>T
BBS10(NM_024685.4):c.372T>A (p.S124=)
-
BBS10_000093
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_AMC
+/., +?/.
3
2
c.378G>A
r.(?)
p.(Trp126*)
ACMG
likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.76741387C>T
g.76347607C>T
c.378G>A, c.378G>A, p.W126*,
1 more item
-
BBS10_000134
Compound heterozygous
PubMed: Liu-2020
,
PubMed: Tao 2020
,
PubMed: Wang 2018
-
-
Germline
yes
-
-
-
-
LOVD
+/.
2
2
c.391del
r.(?)
p.(Gln131Argfs*4)
-
pathogenic
g.76741374del
-
c.391delC(h)
-
BBS10_000198
-
PubMed: Janssen-2011
-
-
Germline
-
-
-
-
-
LOVD
?/.
2
2
c.416A>C
r.(?)
p.(Gln139Pro)
-
VUS
g.76741349T>G
-
p.Q139P
-
BBS10_000200
-
PubMed: Scheidecker 2015
-
-
Germline
-
-
-
-
-
LOVD
+?/., -/., -?/.
7
2
c.424G>A
r.(?)
p.(Asp142Asn)
-
benign, likely benign, likely pathogenic
g.76741341C>T
g.76347561C>T
BBS10(NM_024685.4):c.424G>A (p.D142N), c.424G>A, c.42>G>A
-
BBS10_000071
6 heterozygous, no homozygous;
Clinindb (India)
, VKGL data sharing initiative Nederland
PubMed: Duelund Hjortshoj-2010
,
PubMed: Feuillan-2011
,
PubMed: Jeziorny-2020
,
1 more item
-
rs142863601
CLASSIFICATION record, Germline
yes
0.01, 6/2795 individuals
-
-
-
Joshi Stephen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
Mohammed Faruq
?/.
1
-
c.442T>C
r.(?)
p.(Tyr148His)
-
VUS
g.76741323A>G
-
BBS10(NM_024685.4):c.442T>C (p.Y148H)
-
BBS10_000135
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.462G>A
r.(?)
p.(Leu154=)
-
benign
g.76741303C>T
g.76347523C>T
BBS10(NM_024685.4):c.462G>A (p.L154=)
-
BBS10_000088
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
2
c.473C>A
r.(?)
p.(Ser158*)
-
likely pathogenic
g.76741292G>T
-
c.473C>A
-
BBS10_000181
-
PubMed: Feuillan-2011
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.488_489del
r.(?)
p.(Arg163AsnfsTer4)
-
pathogenic
g.76741281_76741282del
g.76347501_76347502del
BBS10(NM_024685.4):c.488_489delGA (p.R163Nfs*4)
-
BBS10_000108
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/., ?/.
2
-
c.505T>C
r.(?)
p.(Ser169Pro)
-
likely benign, VUS
g.76741260A>G
g.76347480A>G
BBS10(NM_024685.4):c.505T>C (p.S169P)
-
BBS10_000107
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
+/., +?/.
3
2
c.509T>C
r.(?)
p.(Leu170Ser)
-
likely pathogenic, pathogenic
g.76741256A>G
-
c.509T>C, c.509T>C/c.509T>C (p.L170S)
-
BBS10_000156
-
PubMed: Esposito 2017
,
PubMed: Muller-2010
,
PubMed: Stoetzel 2006
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
4
2
c.530A>G
r.(?)
p.(Tyr177Cys)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.76741235T>C
g.76347455T>C
530A/G (Y177C), BBS10 p.Tyr177Cys (c.530A>G) Homozygous, c.[530A>G];[530A>G]
-
BBS10_000131
Homozygous
PubMed: Atmis 2019
,
PubMed: Harville-2010
,
PubMed: Mary-2019
-
-
Germline
?
0/90 ethnically matched controls
-
-
-
LOVD
+/.
1
-
c.531C>A
r.(?)
p.(Tyr177Ter)
ACMG
pathogenic
g.76741234G>T
g.76347454G>T
BBS10 c.531C>A, p.Y177X
-
BBS10_000211
heterozygous; solved
PubMed: Zacchia 2021
-
-
Unknown
?
-
-
-
-
LOVD
+/., +?/.
2
2
c.539G>A
r.(?)
p.(Gly180Glu)
ACMG
likely pathogenic (recessive), pathogenic
g.76741226C>T
g.76347446C>T
c.539G>A(H), c.539G>A, p.G180E
-
BBS10_000197
Compound heterozygous
PubMed: Janssen-2011
,
PubMed: Tao 2020
-
-
Germline
yes
-
-
-
-
LOVD
+/.
4
2
c.559_561del
r.(?)
p.(His187del)
-
pathogenic (recessive)
g.76741204_76741206del
-
c.[559_561del];[559_561del]
-
BBS10_000213
-
PubMed: Mary-2019
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
2
2
c.563A>C
r.(?)
p.(Lys188Thr)
-
likely pathogenic, pathogenic
g.76741202T>G
-
[K188T;A636V]+[K188T;A636V], [p.M390R];[p.M390R]
-
BBS10_000180
-
PubMed: Billingsley-2010
,
PubMed: Deveault-2011
-
-
Germline, Unknown
-
-
-
-
-
LOVD
+?/.
1
-
c.578T>C
r.(?)
p.(Leu193Ser)
-
likely pathogenic
g.76741187A>G
g.76347407A>G
BBS10, variant 1: c.578T>C/p.L193S, variant 2: c.578T>C/p.L193S
-
BBS10_000209
possibly solved, homozygous
PubMed: Weisschuh 2020
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/.
2
-
c.590A>G
r.(?)
p.(Tyr197Cys)
-
likely pathogenic, pathogenic (recessive)
g.76741175T>C
g.76347395T>C
12:76741175T>C ENST00000393262.3:c.590A>G (Tyr197Cys), BBS10 c.590A>G, p.Tyr197Cys
-
BBS10_000117
heterozygous
PubMed: Carss 2017
,
PubMed: Turro 2020
-
-
Germline, Germline/De novo (untested)
?
-
-
-
-
LOVD
+?/.
1
2
c.592_597del
r.(?)
p.(Phe198_Phe199del)
-
likely pathogenic
g.76741168_76741173del
-
c.591delCTTTTT
-
BBS10_000155
-
PubMed: Muller-2010
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.602G>A
r.(?)
p.(Cys201Tyr)
ACMG
likely pathogenic (recessive)
g.76741163C>T
g.76347383C>T
c.602G>A, p.C201Y
-
BBS10_000204
Compound heterozygous
PubMed: Tao 2020
-
-
Germline
yes
-
-
-
-
LOVD
+/., ?/.
3
2
c.641T>A
c.641T>A, r.(?)
p.(Val214Glu)
ACMG
pathogenic, VUS
g.76741124A>T
g.76347344A>T
BBS10 c.641T>A, p.(Val214Glu), BBS10 c.641T>A, p.V214E, c.641T>A/c.641T>A (p.V214E)
-
BBS10_000154
homozygous, homozygous; solved
PubMed: Esposito 2017
,
PubMed: Manara 2019
,
PubMed: Zacchia 2021
-
-
Germline, Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.669_674dup
r.(?)
p.(Gly224_Val225dup)
-
likely pathogenic
g.76741094_76741099dup
g.76347314_76347319dup
665_666insTGTTGG
-
BBS10_000120
-
PubMed: Stone 2017
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
2
c.680_681delinsAA
r.(?)
p.(Gly227Glu)
-
likely pathogenic
g.76741084_76741085delinsTT
-
c.680_681delGCinsAA
-
BBS10_000210
-
PubMed: Jeziorny-2020
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.687del
r.(?)
p.(Val230Phefs*7)
-
pathogenic
g.76741078del
g.76347298del
BBS10 c.687del, p.(Val230Phefs*7)
-
BBS10_000224
heterozygous
PubMed: Barabino 2020
-
-
In vitro (cloned)
?
-
-
-
-
LOVD
+?/.
1
2
c.687delT
r.(?)
p.(Val230Phefs*7)
-
VUS
g.76741078delA
-
c.687delT
-
BBS10_000179
-
PubMed: Chen-2011
-
-
Germline
-
-
-
-
-
LOVD
+?/.
2
2
c.719T>G
r.(?)
p.(Val240Gly)
-
likely pathogenic
g.76741046A>C
-
c.719T>G
-
BBS10_000153
-
PubMed: Muller-2010
,
PubMed: Stoetzel 2006
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
4
2
c.724del
r.(?)
p.(Gln242Argfs*17)
-
likely pathogenic, pathogenic (recessive)
g.76741041del
-
c.724delC, c.[724del];[724del]
-
BBS10_000152
-
PubMed: Mary-2019
,
PubMed: Muller-2010
,
PubMed: Stoetzel 2006
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
10
2
c.728_731del
r.(?)
p.(Lys243Ilefs*15), p.(Lys243IlefsTer15)
-
likely pathogenic, pathogenic (recessive)
g.76741034_76741037del, g.76741037_76741040del
g.76347257_76347260del
BBS10 c.995_999delAAGA, p.Q242fs258X, BBS10 K243IfsX15, c.728_731del p.(K243Ifs*15),
1 more item
-
BBS10_000126
2 more items
PubMed: Abu-Safieh-2012
, Stoetzel 2006,
PubMed: Fieggen 2016
,
PubMed: Mary-2019
,
PubMed: Patel 2016
,
1 more item
-
-
Germline
yes
0/96 ethnically matched controls, 50/74 BBS families (67%) homozygous, 2 heterozygous
-
-
-
LOVD
+?/., -/., -?/., ?/.
7
2
c.765G>A
c.765G>A, r.(?)
p.(Met255Ile)
ACMG
benign, likely benign, likely pathogenic, VUS
g.76741000C>T
g.76347220C>T
BBS10 c.765G>A, p.(Met255Ile), BBS10(NM_024685.4):c.765G>A (p.M255I, p.(Met255Ile)),
2 more items
-
BBS10_000087
heterozygous, VKGL data sharing initiative Nederland
PubMed: Duelund Hjortshoj-2010
,
PubMed: Jespersgaar 2019
,
PubMed: Manara 2019
-
rs139658279
CLASSIFICATION record, Germline
?, yes
0/100 ethnically matched control chromosomes
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
+/.
1
-
c.766C>T
r.(?)
p.(Arg256Ter)
-
pathogenic
g.76740999G>A
g.76347219G>A
BBS10(NM_024685.4):c.766C>T (p.R256*)
-
BBS10_000086
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
1
-
c.783A>G
r.(?)
p.(Thr261=)
-
likely benign
g.76740982T>C
-
BBS10(NM_024685.4):c.783A>G (p.T261=)
-
BBS10_000216
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
2
c.815_822dup
r.(?)
p.(Phe275Aspfs*6)
-
pathogenic
g.76740944_76740951dup
g.76347164_76347171dup
F275fsX281
-
BBS10_000036
-
{PMID:Waters 2015:25564561),
Journal: Waters 2015
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
2
2
c.850C>T
r.(?)
p.(Gln284*)
-
likely pathogenic, likely pathogenic (recessive)
g.76740915G>A
-
p.(Gln284*)
-
BBS10_000196
-
PubMed: Alvarez-Satta 2017
,
PubMed: Castro Sanchez 2015
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/., -?/., ?/.
4
2
c.886G>A
r.(?)
p.(Ala296Thr)
-
likely benign, likely pathogenic, VUS
g.76740879C>T
g.76347099C>T
BBS10(NM_024685.4):c.886G>A (p.A296T), [A296T]+[=], [p.D104A];[p.R632P]
-
BBS10_000085
normal 2nd chromosome, VKGL data sharing initiative Nederland
PubMed: Billingsley-2010
,
PubMed: Deveault-2011
-
-
CLASSIFICATION record, Germline, Unknown
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
+/.
1
2
c.899A>C
r.(?)
p.(His300Pro)
-
pathogenic
g.76740866T>G
-
c.899A>C(h)
-
BBS10_000195
1 more item
PubMed: Janssen-2011
-
-
Germline
-
-
-
-
-
LOVD
+?/.
3
2
c.907_910delAGTC
r.(?)
p.(Ser303Argfs*3)
-
VUS
g.76740855_76740858delGACT
-
c.907_910delAGTC
-
BBS10_000178
-
PubMed: Chen-2011
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
4
2
c.909_912del
r.(?)
p.(Ser303Argfs*3), p.(Ser303ArgfsTer3)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.76740853_76740856del, g.76740855_76740858del
g.76347075_76347078del
BBS10 c.909_912del, p.(Ser303Argfs*3), c.909delTCAG, S303fsX305
-
BBS10_000012
heterozygous
PubMed: Barabino 2020
,
PubMed: Boissel 2017
,
PubMed: Muller-2010
,
PubMed: Stoetzel 2006
,
1 more item
-
-
Germline, In vitro (cloned)
?, yes
-
-
-
-
Johan den Dunnen
+/.
1
2
c.926T>C
r.(?)
p.(Leu309Pro)
-
pathogenic
g.76740839A>G
-
c.926T>C(p.Leu309Pro);het
-
BBS10_000194
-
PubMed: Lindstrand-2014
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
12
2
c.931T>G
r.(?)
p.(Ser311Ala)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.76740834A>C
g.76347054A>C
BBS10, variant 1: c.931T>G/p.S311A, variant 2: c.931T>G/p.S311A, c.931T>G, S311A, S311A/V11G,
1 more item
-
BBS10_000091
solved, homozygous, VKGL data sharing initiative Nederland
PubMed: Ece Solmaz 2015
,
PubMed: Laurier-2006
,
PubMed: Muller-2010
,
PubMed: Stoetzel 2006
,
1 more item
-
rs137852837
CLASSIFICATION record, Germline
yes
0/107 Lebanese controls; 0/96 French controls
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.955G>C
r.(?)
p.(Val319Leu)
-
VUS
g.76740810C>G
g.76347030C>G
BBS10(NM_024685.4):c.955G>C (p.V319L)
-
BBS10_000106
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.957T>C
r.(?)
p.(Val319=)
-
benign
g.76740808A>G
g.76347028A>G
BBS10(NM_024685.4):c.957T>C (p.V319=)
-
BBS10_000105
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
-
c.959_962del
r.(?)
p.(Ser320IlefsTer5)
-
pathogenic
g.76740810_76740813del
g.76347030_76347033del
BBS10(NM_024685.4):c.959_962delGTTA (p.S320Ifs*5)
-
BBS10_000084
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/.
2
2
c.962A>G
c.962A>G, r.(?)
p.(Tyr321Cys)
ACMG
likely pathogenic, pathogenic
g.76740803T>C
g.76347023T>C
BBS10 c.962A>G, p.(Tyr321Cys), c.2137_2140del/c.962A>G (p.K713Ffs*16/p.Y321C)
-
BBS10_000151
heterozygous
PubMed: Esposito 2017
,
PubMed: Manara 2019
-
-
Germline
?
-
-
-
-
LOVD
+?/.
1
2
c.963T>G
r.(?)
p.(Tyr321*)
-
likely pathogenic
g.76740802A>C
-
c.963T>G
-
BBS10_000150
-
PubMed: Muller-2010
,
PubMed: Stoetzel 2006
-
-
Germline
-
-
-
-
-
LOVD
-/., -?/.
2
-
c.966T>C
r.(?)
p.(Tyr322=)
-
benign, likely benign
g.76740799A>G
g.76347019A>G
BBS10(NM_024685.4):c.966T>C (p.Y322=)
-
BBS10_000083
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_AMC
+?/., -?/.
2
2
c.968C>T
r.(?)
p.(Ala323Val)
-
likely benign, likely pathogenic
g.76740797G>A
g.76347017G>A
BBS10 p.Ala323Val (c.968C>T) Homozygous, c.968C>T
-
BBS10_000177
Homozygous
PubMed: Atmis 2019
,
PubMed: Chen-2011
-
-
Germline
?
-
-
-
-
LOVD
+?/.
1
-
c.989T>C
r.(?)
p.(Val330Ala)
-
likely pathogenic
g.76740776A>G
g.76346996A>G
BBS10 c.989T>C, p.Val330Ala
-
BBS10_000220
heterozygous
PubMed: Barrell 2019
-
-
In vitro (cloned)
?
-
-
-
-
LOVD
+?/.
3
2
c.992T>C
r.(?)
p.(Val331Ala)
-
likely pathogenic
g.76740773A>G
-
c.992T>C/p.(Val331Ala)b,c, p.(Val331Ala)
-
BBS10_000193
-
PubMed: Alvarez-Satta-2014
,
PubMed: Castro Sanchez 2015
-
-
Germline
-
-
-
-
-
LOVD
+?/., ?/.
2
2
c.1013_1015del
r.(?)
p.(Glu338del)
ACMG
likely pathogenic, VUS
g.76740750_76740752del, g.76740755_76740757del
g.76346975_76346977del
c.1009_1011delGAA/p.(Glu337del)b
-
BBS10_000192
ACMG PM2, PM4, PM1_SUPPORTING
PubMed: Alvarez-Satta-2014
,
PubMed: Weisschuh 2024
-
-
Germline
-
0/100 chromosomes
-
-
-
Johan den Dunnen
+/.
1
-
c.1024dup
r.(?)
p.(Ile342AsnfsTer20)
-
pathogenic (recessive)
g.76740741dup
g.76346961dup
1024_1025insA
-
BBS10_000125
-
PubMed: Ece Solmaz 2015
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
2
2
c.1044_1045del
r.(?)
p.(Pro350Ilefs*11)
-
likely pathogenic, pathogenic (recessive)
g.76740720_76740721del
-
c.1043delTT, c.[1044_1045del];[273C>G]
-
BBS10_000149
-
PubMed: Mary-2019
,
PubMed: Muller-2010
,
PubMed: Stoetzel 2006
-
-
Germline
-
-
-
-
-
LOVD
+?/.
2
-
c.1044_1045delTT
r.(?)
p.(Pro350Ilefs*11)
-
likely pathogenic
g.76740721_76740722del
g.76346941_76346942del
BBS10 c.1044-1045delTT , p.Pro350IlefsX11, BBS10 c.1044-1045delTT , p.Pro350IlefsX12
-
BBS10_000219
heterozygous
PubMed: Putoux 2010
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
-
c.1046T>C
r.(?)
p.(Leu349Ser)
-
pathogenic
g.94793953T>C
g.93781725T>C
TMEM67 c.1046T>C, p.(Leu349Ser)
-
TMEM67_000014
heterozygous
PubMed: Barabino 2020
-
-
In vitro (cloned)
?
-
-
-
-
LOVD
+/., +?/.
3
2
c.1063C>T
r.(?)
p.(Gln355*)
ACMG
likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.76740702G>A
g.76346922G>A
c.1063C>T, c.1063C>T, p.Q355*,
1 more item
-
BBS10_000133
Compound heterozygous
PubMed: Liu-2020
,
PubMed: Tao 2020
,
PubMed: Wang 2018
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/.
5
2
c.1091del
c.1091del, r.(?)
p.(Asn364Thrfs*5), p.(Asn364ThrfsTer5)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.76740674del, g.76740675del
g.76346895del
BBS10 c.1091del, p.(Asn364Thrfs*5), c.1091delA, c.[1143T>G];[1091del]
-
BBS10_000115
heterozygous
PubMed: Manara 2019
,
PubMed: Mary-2019
,
PubMed: Muller-2010
,
PubMed: Pierrottet 2014
,
1 more item
-
rs727503818
Germline
yes
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
+?/.
1
2
c.1091delA
r.(?)
p.(Asn364Thrfs*5)
-
likely pathogenic
g.76740674delT
-
c.1091delA
-
BBS10_000176
-
PubMed: Schaefer-2011
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
2
c.1110G>A
r.(?)
p.?
-
pathogenic
g.76740655C>T
-
c.[1110G>A];[1110G>A]
-
BBS10_000175
-
PubMed: Redin-2012
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
2
c.1143T>G
r.(?)
p.(Tyr381*)
-
pathogenic (recessive)
g.76740622A>C
-
c.[1143T>G];[1091del]
-
BBS10_000212
-
PubMed: Mary-2019
-
-
Germline
-
-
-
-
-
LOVD
-?/.
2
-
c.1158G>A
r.(?)
p.(Leu386=)
-
likely benign
g.76740607C>T
g.76346827C>T
BBS10(NM_024685.4):c.1158G>A (p.L386=)
-
BBS10_000082
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/.
1
2
c.1171_1181dup
r.(?)
p.(Ser396Leufs*6)
-
pathogenic
g.76740584_76740594dup
-
c.[1171_1181dupGCATTTATACC]
-
BBS10_000132
-
PubMed: M'hamdi 2014
-
-
Unknown
-
-
-
-
-
LOVD
+/.
1
2
c.1171_1181dupGCATTTATACC
r.(?)
p.(Ser396Leufs*6)
-
pathogenic (recessive)
g.76740584_76740594dupGGTATAAATGC
-
c.[1171_1181dupGCATTTATACC]
-
BBS10_000174
-
PubMed: M'hamdi-2014
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.1183C>G
r.(?)
p.(His395Asp)
-
VUS
g.76740582G>C
g.76346802G>C
-
-
BBS10_000104
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
2
-
c.1184A>G
r.(?)
p.(His395Arg)
-
likely pathogenic
g.76740581T>C
g.76346801T>C
c.1184A>G , H395R
-
BBS10_000159
Homozygous
PubMed: Chakrabarty 2020
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
2
2
c.1184dup
r.(?)
p.(His395Glnfs*14)
-
likely pathogenic
g.76740581dup
-
c.1184dupA
-
BBS10_000173
-
PubMed: Feuillan-2011
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
2
c.1189A>G
r.(?)
p.(Ile397Val)
-
pathogenic
g.76740576T>C
g.76346796T>C
-
-
BBS10_000070
no homozygotes in control subjects; variant tested in zebrafish model confirmed deleterious effect
PubMed: Lim 2014
,
Journal: Lim 2014
-
rs202042386
Germline
yes
3/1791 cases
-
-
-
Marianne Vos (LOVD-team)
+?/.
1
2
c.1202G>A
r.(?)
p.(Gly401Glu)
-
likely pathogenic
g.76740563C>T
-
c.1660G>A
-
BBS10_000138
-
PubMed: Sathya Priya-2015
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
2
c.1227del
r.(?)
p.(Gln409Hisfs*22)
-
likely pathogenic
g.76740538del
-
c.1227delA
-
BBS10_000137
-
PubMed: Sathya Priya-2015
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
2
2
c.1230T>G
r.(?)
p.(His410Gln)
-
likely pathogenic
g.76740535A>C
-
[c.235+1G>A];[c.235+1G>A], [p.F94SfsX9];[p.F94SfsX9]
-
BBS10_000172
-
PubMed: Deveault-2011
,
PubMed: Deveault-2013
-
-
Unknown
-
-
-
-
-
LOVD
+/.
1
2
c.1230T>R
r.(?)
p.(His410Gln)
-
pathogenic
g.76740535A>T
-
[C91LfsX5]+[H410Q]
-
BBS10_000171
-
PubMed: Billingsley-2010
-
-
Germline
-
-
-
-
-
LOVD
10 per page
25 per page
50 per page
100 per page
Legend
How to query
« First
Prev
1
2
Next
Last »
Screenscraping/webscraping (downloading large amounts of data using scripts) is strictly prohibited.
Use our
APIs
to retrieve data.
Powered by
LOVD v.3.0
Build 30b
LOVD software ©2004-2024
Leiden University Medical Center
Database contents © by their respective submitters and curators