Global Variome shared LOVD
NR5A1 (nuclear receptor subfamily 5, group A, member 1)
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Unique variants in the NR5A1 gene
The variants shown are described using the NM_004959.4 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
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!
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'
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123 entries on 2 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.-16+3_-16+6del
r.spl?
p.?
-
VUS
g.127269524_127269527del
g.124507245_124507248del
NR5A1(NM_004959.4):c.-16+3_-16+6delAAGT
-
NR5A1_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
_1_7_
c.(-181_*1522)::[NM_014290.2:(208-742_563+618)]
r.?
p.?
-
pathogenic
g.(100192473_100195138)_(127243515_127269693)inv
-
-
46,XY,inv(9)(q22.33q34.11)
TDRD7_000013
-
PubMed: Lachke 2011
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+?/.
1
3
c.1A>G
r.(?)
p.0?
-
pathogenic
g.127265674T>C
g.124503395T>C
M1V
-
NR5A1_000039
-
PubMed: Philibert 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.3G>A
r.(?)
p.0?
-
pathogenic
g.127265672C>T
g.124503393C>T
-
-
NR5A1_000086
-
PubMed: Lourenço 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.11C>A
r.(?)
p.(Ser4*)
-
pathogenic
g.127265664G>T
g.124503385G>T
-
-
NR5A1_000087
-
Fabbri-Scallet, submitted 2019
-
-
Germline
-
-
-
-
-
Helena Fabbri-Scallet
+?/.
1
3
c.18del
r.(?)
p.(Asp6Glufs*69)
-
pathogenic (dominant)
g.127265657del
g.124503378del
18delC
-
NR5A1_000056
-
PubMed: Hasegawa 2004
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.23A>G
r.(?)
p.(Asp8Gly)
-
pathogenic
g.127265652T>C
g.124503373T>C
-
-
NR5A1_000024
parents not available
PubMed: Sreenivasan 2018
,
Journal: Sreenivasan 2018
-
-
Germline/De novo (untested)
-
-
-
-
-
Vincent Russel Harley
+?/.
1
3
c.31G>T
r.(?)
p.(Glu11*)
-
likely pathogenic
g.127265644C>A
g.124503365C>A
-
-
NR5A1_000085
-
PubMed: Köhler 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.43G>A
r.(?)
p.(Val15Met)
-
pathogenic (dominant)
g.127265632C>T
g.124503353C>T
-
-
NR5A1_000051
-
PubMed: Lin 2007
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.48C>A
r.(?)
p.(Cys16*)
-
pathogenic (dominant)
g.127265627G>T
g.124503348G>T
-
-
NR5A1_000055
-
PubMed: Mallet 2004
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.76G>A
r.(?)
p.(Gly26Arg)
-
likely pathogenic
g.127265599C>T
g.124503320C>T
-
-
NR5A1_000088
-
Fabbri-Scallet, submitted 2019
-
-
Germline
-
-
-
-
-
Helena Fabbri-Scallet
+?/.
1
3
c.86C>G
r.(?)
p.(Thr29Arg)
-
likely pathogenic
g.127265589G>C
g.124503310G>C
-
-
NR5A1_000084
-
PubMed: Philibert 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.95G>A
r.(?)
p.(Ser32Asn)
-
pathogenic
g.127265580C>T
g.124503301C>T
-
-
NR5A1_000025
-
PubMed: Fabbri-Scallet 2017
-
-
Germline
-
-
-
-
-
Helena Fabbri-Scallet
+?/.
1
-
c.96C>G
r.(?)
p.(Ser32Arg)
-
likely pathogenic
g.127265579G>C
-
-
-
NR5A1_000110
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
3
c.98G>C
r.(?)
p.(Cys33Ser)
-
likely pathogenic
g.127265577C>G
g.124503298C>G
-
-
NR5A1_000046
-
PubMed: Kohler 2008
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.103-3C>A
r.spl?
p.?
-
likely pathogenic
g.?
-
-
-
PTCH1_000000
-
PubMed: Köhler 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.104G>A
r.(?)
p.(Gly35Asp)
-
likely pathogenic
g.127265498C>T
g.124503219C>T
-
46,XY
NR5A1_000022
-
PubMed: Eggers 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.104_105delinsAA
r.(?)
p.(Gly35Glu)
-
pathogenic
g.127265497_127265498delinsTT
g.124503218_124503219delinsTT
104_105delGCinsAA
-
NR5A1_000034
-
PubMed: Achermann 1999
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.112A>T
r.(?)
p.(Lys38*)
-
pathogenic
g.127265490T>A
g.124503211T>A
-
-
NR5A1_000028
-
PubMed: Fabbri 2016
-
-
Germline
-
-
-
-
-
Helena Fabbri-Scallet
+/.
1
-
c.115C>T
r.(?)
p.(Arg39Cys)
-
pathogenic
g.127265487G>A
g.124503208G>A
-
-
NR5A1_000031
-
PubMed: Fabbri-Scallet 2017
-
-
Germline
-
-
-
-
-
Helena Fabbri-Scallet
+?/.
2
3
c.116G>C
r.(?)
p.(Arg39Pro)
-
likely pathogenic
g.127265486C>G
g.124503207C>G
-
-
NR5A1_000038
-
PubMed: Allali 2011
,
PubMed: Philibert 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.119C>G
r.(?)
p.(Thr40Arg)
-
likely pathogenic
g.127265483G>C
g.124503204G>C
-
46,XY
NR5A1_000104
-
PubMed: Eggers 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.122T>G
r.(?)
p.(Val41Gly)
-
pathogenic
g.127265480A>C
g.124503201A>C
-
-
NR5A1_000083
-
PubMed: Tajima 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.140A>G
r.(?)
p.(Tyr47Cys)
-
VUS
g.127265462T>C
g.124503183T>C
NR5A1(NM_004959.5):c.140A>G (p.Y47C)
-
NR5A1_000019
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
-
c.140_163del
r.(?)
p.(Ala48_Pro55del)
-
pathogenic
g.127265441_127265464del
g.124503162_124503185del
-
46,XY
NR5A1_000103
-
PubMed: Eggers 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.141_144dup
r.(?)
p.(Cys49Hisfs*39)
-
pathogenic
g.127265458_127265461dup
g.124503179_124503182dup
140_141insCACG
-
NR5A1_000043
-
PubMed: Allali 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.151del
r.(?)
p.(Glu51Argfs*24)
-
pathogenic
g.127265451del
g.124503172del
151delG
-
NR5A1_000040
-
PubMed: Philibert 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/., ?/.
2
3
c.164G>A
r.(?)
p.(Cys55Tyr)
-
likely pathogenic, VUS
g.127265438C>T
g.124503159C>T
NR5A1(NM_004959.4):c.164G>A (p.C55Y)
-
NR5A1_000082
VKGL data sharing initiative Nederland
PubMed: Philibert 2011
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
+?/.
1
-
c.164G>C
r.(?)
p.(Cys55Ser)
-
likely pathogenic
g.127265438C>G
g.124503159C>G
-
-
NR5A1_000089
-
Fabbri-Scallet, submitted 2019
-
-
De novo
-
-
-
-
-
Helena Fabbri-Scallet
+/.
1
-
c.180_181del
r.(?)
p.(Gln61AlafsTer25)
-
pathogenic
g.127265422_127265423del
g.124503143_124503144del
NR5A1(NM_004959.4):c.180_181delGC (p.Q61Afs*25)
-
NR5A1_000018
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
3
c.187A>C
r.(?)
p.(Lys63Gln)
-
likely pathogenic
g.127265415T>G
g.124503136T>G
-
-
NR5A1_000081
-
PubMed: Philibert 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.194G>A
r.(?)
p.(Cys65Tyr)
-
pathogenic
g.127265408C>T
g.124503129C>T
195G > A (Cys65Tyr)
-
NR5A1_000030
-
PubMed: Fabbri 2014
-
-
Germline
yes
-
-
-
-
Helena Fabbri-Scallet
+?/.
1
-
c.206G>C
r.(?)
p.(Arg69Pro)
-
likely pathogenic
g.127265396C>G
-
-
-
NR5A1_000115
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.208T>C
r.(?)
p.(Phe70Leu)
-
VUS
g.127265394A>G
g.124503115A>G
-
-
NR5A1_000108
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.211del
r.(?)
p.(Gln71ArgfsTer4)
-
pathogenic
g.127265392del
g.124503113del
NR5A1(NM_004959.4):c.211delC (p.Q71Rfs*4)
-
NR5A1_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.213G>C
r.(?)
p.(Gln71His)
-
likely pathogenic
g.127265389C>G
-
-
-
NR5A1_000113
-
-
-
rs865902758
CLASSIFICATION record
-
-
-
-
-
MobiDetails
+?/.
1
-
c.232A>T
r.(?)
p.(Met78Leu)
-
likely pathogenic
g.127265370T>A
g.124503091T>A
-
-
NR5A1_000105
-
Fabbri-Scallet, submitted 2019
-
-
Germline
-
-
-
-
-
Helena Fabbri-Scallet
+/.
1
3
c.234G>A
r.(?)
p.(Met78Ile)
-
pathogenic (!)
g.127265368C>T
g.124503089C>T
-
-
NR5A1_000052
inheritance dominant, sex-limited
PubMed: Lin 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.238del
r.(?)
p.(Leu80Trpfs*9)
-
likely pathogenic
g.127265365del
g.124503086del
238delC
-
NR5A1_000026
-
PubMed: Fabbri 2016
-
-
Germline
-
-
-
-
-
Helena Fabbri-Scallet
+?/.
1
3i
c.244+80G>A
r.spl?
p.(=)
-
likely pathogenic
g.127265278C>T
g.124502999C>T
-
-
NR5A1_000080
-
PubMed: Paliwal 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.247G>A
r.(?)
p.(Val83Met)
-
VUS
g.127262992C>T
g.124500713C>T
NR5A1(NM_004959.4):c.247G>A (p.V83M)
-
NR5A1_000016
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
2
3
c.250C>T
r.(?)
p.(Arg84Cys)
-
pathogenic
g.127262989G>A
g.124500710G>A
-
-
NR5A1_000079
-
PubMed: Reuter 2007
-
rs1832458239
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
MobiDetails
+/., +?/.
4
3
c.251G>A
r.(?)
p.(Arg84His)
-
likely pathogenic, pathogenic
g.127262988C>T
g.124500709C>T
NR5A1(NM_004959.4):c.251G>A (p.R84H)
46,XY
NR5A1_000047
father not tested, VKGL data sharing initiative Nederland
PubMed: Eggers 2016
,
PubMed: Kohler 2008
-
-
CLASSIFICATION record, Germline, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
+/.
1
-
c.253_254del
r.(?)
p.(Ala85Argfs*24)
-
pathogenic
g.127262989_127262990del
g.124500710_124500711del
-
46,XY
NR5A1_000102
-
PubMed: Eggers 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.265del
r.(?)
p.(Ala89Profs*120)
-
pathogenic
g.127262974del
g.124500695del
-
46,XY
NR5A1_000101
mother negative
PubMed: Eggers 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.271G>A
r.(?)
p.(Gly91Ser)
-
pathogenic (!)
g.127262968C>T
g.124500689C>T
-
-
NR5A1_000053
inheritance dominant, sex-limited
PubMed: Lin 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
3
c.275G>A
r.(?)
p.(Arg92Gln)
-
pathogenic (recessive)
g.127262964C>T
g.124500685C>T
-
-
NR5A1_000035
-
PubMed: Achermann 2002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.288_304del
r.(?)
p.(Met98Glyfs*45)
-
pathogenic
g.127262937_127262953del
g.124500658_124500674del
-
-
NR5A1_000090
-
Fabbri-Scallet, submitted 2019
-
-
Germline
-
-
-
-
-
Helena Fabbri-Scallet
+?/.
1
3
c.307C>G
r.(?)
p.(Arg103Gly)
-
likely pathogenic
g.127262932G>C
g.124500653G>C
-
-
NR5A1_000078
-
PubMed: Philibert 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
3
c.319C>T
r.(?)
p.(Gln107*)
-
likely pathogenic
g.127262920G>A
g.124500641G>A
-
-
NR5A1_000077
-
PubMed: Köhler 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
3
c.341G>A
r.(?)
p.(Arg114Gln)
-
likely pathogenic
g.127262898C>T
g.124500619C>T
-
-
NR5A1_000076
-
PubMed: Philibert 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.374dup
r.(?)
p.(Met126Aspfs*23)
-
pathogenic
g.127262869dup
g.124500590dup
369insC
-
NR5A1_000041
-
PubMed: Philibert 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.375G>A
r.(?)
p.(Pro125=)
-
benign
g.127262864C>T
g.124500585C>T
NR5A1(NM_004959.4):c.375G>A (p.P125=)
-
NR5A1_000015
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/.
2
3
c.390del
r.(?)
p.(Pro131Argfs*165)
-
likely pathogenic, pathogenic
g.127262849del
g.124500570del
390delG
-
NR5A1_000044
-
PubMed: Allali 2011
,
PubMed: Lourenço 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
3
c.392C>T
r.(?)
p.(Pro131Leu)
-
likely pathogenic
g.127262847G>A
g.124500568G>A
-
-
NR5A1_000075
-
PubMed: Bashamboo 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.407C>T
r.(?)
p.(Pro136Leu)
-
VUS
g.127262832G>A
g.124500553G>A
NR5A1(NM_004959.5):c.407C>T (p.P136L)
-
NR5A1_000014
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
3
c.414C>A
r.(?)
p.(Tyr138*)
-
pathogenic
g.127262825G>T
g.124500546G>T
-
-
NR5A1_000048
father not tested
PubMed: Kohler 2008
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+?/.
1
3
c.424_427dup
r.(?)
p.(Ser143Thrfs*7)
-
pathogenic
g.127262812_127262815dup
g.124500533_124500536dup
424_427dupCCCA
-
NR5A1_000050
parents not tested
PubMed: Kohler 2008
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+?/., -/.
4
3
c.437G>C
r.(?)
p.(Gly146Ala)
-
association, benign
g.127262802C>G
g.124500523C>G
NR5A1(NM_004959.4):c.437G>C (p.G146A)
-
NR5A1_000013
VKGL data sharing initiative Nederland,
1 more item
PubMed: Kohler 2008
,
PubMed: WuQiang 2003
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
+/.
1
-
c.485del
r.(?)
p.(Gly162Alafs*134)
-
pathogenic
g.127262756del
-
-
-
NR5A1_000117
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.493G>C
r.(?)
p.(Gly165Arg)
-
pathogenic
g.127262746C>G
g.124500467C>G
-
-
NR5A1_000023
parents not available
PubMed: Sreenivasan 2018
,
Journal: Sreenivasan 2018
-
-
Germline/De novo (untested)
-
-
-
-
-
Vincent Russel Harley
?/.
1
-
c.521C>T
r.(?)
p.(Pro174Leu)
-
VUS
g.127262718G>A
-
NR5A1(NM_004959.5):c.521C>T (p.P174L)
-
NR5A1_000112
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
1
-
c.532G>A
r.(?)
p.(Gly178Arg)
-
VUS
g.127262707C>T
g.124500428C>T
-
46,XY
NR5A1_000100
-
PubMed: Eggers 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
3
c.536del
r.(?)
p.(Pro179Hisfs*117)
-
likely pathogenic
g.127262704del
g.124500425del
536delC
-
NR5A1_000074
-
PubMed: Coutant 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
3
c.549C>A
r.(?)
p.(Tyr183*)
-
likely pathogenic
g.127262690G>T
g.124500411G>T
-
-
NR5A1_000073
-
PubMed: Warman 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.571C>T
r.(?)
p.(Arg191Cys)
-
pathogenic
g.127262668G>A
g.124500389G>A
-
-
NR5A1_000072
-
PubMed: Bashamboo 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.574G>T
r.(?)
p.(Ala192Ser)
-
VUS
g.127262665C>A
g.124500386C>A
NR5A1(NM_004959.5):c.574G>T (p.A192S)
-
NR5A1_000012
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
-
c.575C>T
r.(?)
p.(Ala192Val)
-
VUS
g.127262664G>A
g.124500385G>A
NR5A1(NM_004959.5):c.575C>T (p.A192V)
-
NR5A1_000107
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+?/.
2
4
c.578T>A
r.(?)
p.(Ile193Asn)
ACMG
likely pathogenic, likely pathogenic (dominant)
g.127262661A>T
g.124500382A>T
-
-
NR5A1_000114
ACMG PM1, PM2, PP2, PP3, PP4
PubMed: Giannakopoulos 2022
,
PubMed: Marinakis 2021
-
-
Germline, Germline/De novo (untested)
?
-
-
-
-
Amalia Sertedaki
,
Jan Traeger-Synodinos
?/.
1
-
c.593C>T
r.(?)
p.(Pro198Leu)
-
VUS
g.127262646G>A
-
-
-
NR5A1_000116
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.624del
r.(?)
p.(Met208Ilefs*39)
-
pathogenic
g.127262617del
g.124500338del
-
46,XY
NR5A1_000099
-
PubMed: Eggers 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.629C>A
r.(?)
p.(Pro210Gln)
-
pathogenic
g.127262610G>T
g.124500331G>T
-
46,XY
NR5A1_000098
-
PubMed: Eggers 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.632A>G
r.(?)
p.(Tyr211Cys)
-
likely pathogenic
g.127262607T>C
-
-
-
NR5A1_000118
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.633C>A
r.(?)
p.(Tyr211*)
-
pathogenic
g.127262606G>T
g.124500327G>T
-
46,XY
NR5A1_000097
-
PubMed: Eggers 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/., ?/.
2
4
c.634G>A
r.(?)
p.(Gly212Ser)
-
likely pathogenic, VUS
g.127262605C>T
g.124500326C>T
NR5A1(NM_004959.5):c.634G>A (p.G212S)
-
NR5A1_000011
VKGL data sharing initiative Nederland
PubMed: Bashamboo 2010
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Utrecht
+?/.
1
4
c.666del
r.(?)
p.(Asn222Lysfs*74)
-
likely pathogenic
g.127262573del
g.124500294del
666delC
-
NR5A1_000071
-
PubMed: Lourenço 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.677T>G
r.(?)
p.(Leu226Arg)
-
likely pathogenic
g.127262562A>C
g.124500283A>C
-
-
NR5A1_000021
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
4
c.691_699del
r.(?)
p.(Leu231_Leu233del)
-
likely pathogenic
g.127262549_127262557del
g.124500270_124500278del
691_699delCTGCAGCTG
-
NR5A1_000070
-
PubMed: Lourenço 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.704C>T
r.(?)
p.(Pro235Leu)
-
VUS
g.127262535G>A
-
-
-
NR5A1_000123
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
4
c.712G>A
r.(?)
p.(Asp238Asn)
-
likely pathogenic
g.127262527C>T
g.124500248C>T
-
-
NR5A1_000069
-
PubMed: Bashamboo 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.741C>A
r.(?)
p.(Cys247*)
-
pathogenic
g.127262498G>T
g.124500219G>T
-
-
NR5A1_000032
-
PubMed: Fabbri-Scallet 2017
-
-
Germline
-
-
-
-
-
Helena Fabbri-Scallet
+?/.
1
5
c.763C>T
r.(?)
p.(Arg255Cys)
-
likely pathogenic
g.127262476G>A
g.124500197G>A
-
-
NR5A1_000068
-
PubMed: Philibert 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
5
c.764G>T
r.(?)
p.(Arg255Leu)
-
pathogenic
g.127262475C>A
g.124500196C>A
-
-
NR5A1_000067
-
PubMed: Biason-Lauber 2000
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
4
c.779C>T
r.(?)
p.(Ala260Val)
-
pathogenic
g.127262460G>A
g.124500181G>A
-
-
NR5A1_000002
not in 200 control chromosomes
-
-
-
Germline
-
-
-
-
-
Angel Chan
+/.
1
-
c.794del
r.(?)
p.(Leu265ArgfsTer31)
-
pathogenic
g.127262445del
g.124500166del
NR5A1(NM_004959.4):c.794delT (p.L265Rfs*31)
-
NR5A1_000010
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
5
c.835T>A
r.(?)
p.(Trp279Arg)
-
pathogenic
g.127262404A>T
g.124500125A>T
-
-
NR5A1_000037
-
PubMed: Allali 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
5
c.836G>A
r.(?)
p.(Trp279*)
-
likely pathogenic
g.127262403C>T
g.124500124C>T
-
-
NR5A1_000066
-
PubMed: Warman 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
5
c.842G>C
r.(?)
p.(Arg281Pro)
-
pathogenic (recessive)
g.127262397C>G
g.124500118C>G
-
-
NR5A1_000036
-
PubMed: Philibert 2011
-
-
Germline
-
-
-
-
father mosaic
Johan den Dunnen
+?/.
1
5i
c.870+8_870+11del
r.spl?
p.(=)
-
likely pathogenic
g.127262360_127262363del
g.124500081_124500084del
870+8_870+11delTCTC
-
NR5A1_000065
-
PubMed: Warman 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
3
-
c.871-20C>T
r.(=)
p.(=)
-
benign
g.127255448G>A
g.124493169G>A
NR5A1(NM_004959.4):c.871-20C>T, NR5A1(NM_004959.5):c.871-20C>T
-
NR5A1_000009
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+/., +?/.
2
5
c.877G>A
r.(?)
p.(Asp293Asn)
-
likely pathogenic, pathogenic
g.127255422C>T
g.124493143C>T
NR5A1(NM_004959.4):c.877G>A (p.D293N)
-
NR5A1_000064
VKGL data sharing initiative Nederland
PubMed: Lourenço 2009
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
+?/.
2
-
c.928C>G
r.(?)
p.(His310Asp)
-
likely pathogenic
g.127255371G>C
g.124493092G>C
-
46,XY
NR5A1_000096
-
PubMed: Eggers 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/., ?/.
3
6
c.937C>T
r.(?)
p.(Arg313Cys)
-
likely pathogenic, pathogenic (recessive), VUS
g.127255362G>A
g.124493083G>A
NR5A1(NM_004959.5):c.937C>T (p.R313C)
-
NR5A1_000008
VKGL data sharing initiative Nederland
Fabbri-Scallet, submitted 2019,
PubMed: Allali 2011
-
-
CLASSIFICATION record, De novo, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Utrecht
,
Helena Fabbri-Scallet
+/.
1
-
c.938G>A
r.(?)
p.(Arg313His)
-
pathogenic
g.127255361C>T
g.124493082C>T
NR5A1(NM_004959.4):c.938G>A (p.R313H)
-
NR5A1_000106
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
6
c.946C>T
r.(?)
p.(Gln316*)
-
likely pathogenic
g.127255353G>A
g.124493074G>A
-
-
NR5A1_000063
-
PubMed: Philibert 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.984_985del
r.(?)
p.(Lys328Asnfs*151)
-
pathogenic
g.127255314_127255315del
g.124493035_124493036del
-
46,XY
NR5A1_000095
-
PubMed: Eggers 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
5
c.990G>A
r.?
p.?
-
likely pathogenic (dominant)
g.127255309C>T
g.124493030C>T
-
-
NR5A1_000119
-
PubMed: Nambot 2018
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.991-1G>C
r.spl
p.?
-
pathogenic
g.127253508C>G
g.124491229C>G
-
46,XY
NR5A1_000094
-
PubMed: Eggers 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.1025C>T
r.(?)
p.(Ser342Leu)
-
VUS
g.127253473G>A
-
NR5A1(NM_004959.5):c.1025C>T (p.S342L)
-
NR5A1_000124
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
1
-
c.1030C>G
r.(?)
p.(Leu344Val)
-
VUS
g.127253468G>C
g.124491189G>C
NR5A1(NM_004959.4):c.1030C>G (p.L344V)
-
NR5A1_000109
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
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