Global Variome shared LOVD
POU3F4 (POU class 3 homeobox 4)
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Global Variome, with Curator vacancy
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Unique variants in the POU3F4 gene
The variants shown are described using the NM_000307.4 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.
99 entries on 1 page. Showing entries 1 - 99.
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Legend
How to query
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.-51136791_-443193inv
r.?
p.?
-
pathogenic (recessive)
g.31626542_82320140inv
g.31608425_82302023inv
-
-
DMD_068719
inversion from DMD intron to 443 kb upstream of POU3F4 gene
PubMed: Chandrasekhar 2023
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
1
_1
c.(?_-1911798)_(-165501_?)del
r.?
p.?
-
pathogenic (recessive)
g.(?_80851535)_(82597832_?)del
g.(?_80851535)_(82597832_?)del
-
-
POU3F4_000103
-
PubMed: Choi 2013
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
_1
c.-1623019_-752234delinsAGTGAAA
r.?
p.?
-
likely pathogenic (recessive)
g.81140314_82011099delinsAGTGAAA
g.81885306_82756091delinsAGTGAAA
-
-
POU3F4_000105
-
PubMed: Jiang 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
_1
c.(-4550374_-1109146)_(-287187_-6102)del
r.?
p.?
-
pathogenic
g.(78212959_81654187)_(82476146_82757231)del
g.(78957951_82399179)_(83221138_83502223)del
-
-
POU3F4_000084
deletion from DXS1225/DXS169 to DXS26/DXS995
PubMed: Arellano 2000
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
1
-
c.-922590_-127125delinsCATCATCTCAGCCCCATC
r.(=|red)
p.?
-
pathogenic (recessive)
g.81840743_82636208delinsCATCATCTCAGCCCCATC
g.82585735_83381200delinsCATCATCTCAGCCCCATC
-
-
POU3F4_000102
795.5 kb deletion 140 kb upstream POU3F4 gene
Journal: Yang 2025
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
1
_1_
c.-617262_*7424074inv
r.?
p.?
-
likely pathogenic (recessive)
g.82146071_90188492inv
g.82891063_90933484inv
chrX:82146071–90188492inv
-
POU3F4_000104
-
PubMed: Jiang 2021
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/.
3
_1_
c.-64_*356{0}
r.0
p.0
-
pathogenic
g.(?_82763269)_(82764774_?)del
g.(?_83508261)_(83509766_?)del
-
-
POU3F4_000000
1Mb deletion, >1.5Mb deletion starting >1250Kb upstream,
1 more item
PubMed: de Kok 1996
-
-
Unknown
-
-
-
-
-
LOVD
+/.
2
_1_
c.-64_*356{0}
r.0?
p.0?
-
pathogenic (recessive)
g.(?_82763269)_(82764774_?)del
g.(?_83508261)_(83509766_?)del
-
-
POU3F4_000000
-
PubMed: de Kok 1995
-
-
Germline/De novo (untested), Unknown
-
-
-
-
-
LOVD
+/.
11
_1, _1_
c.=
r.=
p.=
-
pathogenic
g.?
-
Â
-
POU3F4_000000
150Kb duplication 170Kb upstream of POU3F4 and paracentric inversion, 200Kb deletion 850Kb upstream,
8 more items
PubMed: de Kok 1995
,
PubMed: de Kok 1996
-
-
Unknown
-
-
-
-
-
LOVD
?/.
1
-
c.103C>A
r.(?)
p.(Gln35Lys)
-
VUS
g.82763435C>A
g.83508427C>A
POU3F4(NM_000307.4):c.103C>A (p.Q35K)
-
POU3F4_000048
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.191G>A
r.(?)
p.(Gly64Glu)
-
VUS
g.82763523G>A
g.83508515G>A
POU3F4(NM_000307.4):c.191G>A (p.G64E)
-
POU3F4_000044
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
1
c.200G>A
r.(?)
p.(Trp67*)
-
pathogenic
g.82763532G>A
g.83508524G>A
-
-
POU3F4_000027
-
PubMed: Cremers 2000
-
-
Unknown
-
-
-
-
-
LOVD
+/+
1
1
c.201G>A
r.(?)
p.(Trp67*)
-
pathogenic
g.82763533G>A
g.83508525G>A
-
-
POU3F4_000054
-
MORL Deafness Variation Database
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
1
-
c.206C>T
r.(?)
p.(Ser69Phe)
-
VUS
g.82763538C>T
g.83508530C>T
POU3F4(NM_000307.4):c.206C>T (p.S69F)
-
POU3F4_000049
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.227del
r.(?)
p.(Leu76Argfs*6)
-
likely pathogenic
g.82763559del
-
227delT
-
POU3F4_000077
-
-
-
-
Unknown
-
-
-
-
-
Gunnar Schmidt
+?/.
1
-
c.232C>T
r.(?)
p.(Gln78Ter)
ACMG
likely pathogenic (recessive)
g.82763564C>T
g.83508556C>T
-
-
POU3F4_000085
ACMG PVS1_Strong, PM2
PubMed: Jiang 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/+, +/.
3
1
c.235C>T
r.(?)
p.(Gln79*)
-
pathogenic
g.82763567C>T
g.83508559C>T
Q79X
-
POU3F4_000010
0/260 hearing controls
MORL Deafness Variation Database
,
PubMed: Parzefall 2013
,
1 more item
-
-
De novo, Germline, SUMMARY record
yes
1/218 unrelated cases
-
-
-
Global Variome, with Curator vacancy
,
Zippi Brownstein
,
Thomas Parzefall
+/.
1
1
c.293C>A
r.(?)
p.(Ser98*)
-
pathogenic
g.82763625C>A
g.83508617C>A
-
-
POU3F4_000037
-
PubMed: Marlin 2009
-
-
Unknown
-
-
-
-
-
LOVD
+/+, +/.
2
1
c.341G>A
r.(?)
p.(Trp114*)
-
pathogenic
g.82763673G>A
g.83508665G>A
-
-
POU3F4_000042
-
PubMed: Waryah 2011
,
1 more item
-
-
SUMMARY record, Unknown
-
-
-
-
-
Global Variome, with Curator vacancy
+/.
1
1
c.346del
r.(?)
p.(Ala116Profs*26)
-
pathogenic (recessive)
g.82763678del
g.83508670del
346delG
-
POU3F4_000034
-
PubMed: Lee 2009
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
2
-
c.346dup
r,(?), r.(?)
p.(Ala116GlyfsTer77)
ACMG
likely pathogenic (recessive), pathogenic
g.82763678dup
g.83508670dup
346dupG
-
POU3F4_000086
ACMG PVS1_Strong, PM2, combination of alleles not reported
PubMed: Jiang 2021
,
PubMed: Wu 2019
-
-
Germline, Germline/De novo (untested)
-
2/1291 cases hearing loss
-
-
-
Johan den Dunnen
+?/.
1
-
c.346_350dup
r.(?)
p.(Ser117ArgfsTer27)
ACMG
likely pathogenic (recessive)
g.82763678_82763682dup
g.83508670_83508674dup
-
-
POU3F4_000087
ACMG PVS1_Strong, PM2
PubMed: Jiang 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.381C>T
r.(?)
p.(Ser127=)
-
likely benign
g.82763713C>T
-
POU3F4(NM_000307.4):c.381C>T (p.S127=)
-
POU3F4_000082
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+, +/.
3
1
c.383del
r.(?)
p.(Gly128Alafs*14)
-
pathogenic
g.82763715del
g.83508707del
-
-
POU3F4_000032, POU3F4_000056
-
MORL Deafness Variation Database
,
PubMed: Lee 2009
,
PubMed: Lee 2009
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
1
-
c.389C>A
r.(?)
p.(Pro130His)
-
VUS
g.82763721C>A
-
POU3F4(NM_000307.4):c.389C>A (p.P130H)
-
POU3F4_000092
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.401_404dup
r.(?)
p.(Gln136LeufsTer58)
ACMG
likely pathogenic (recessive)
g.82763733_82763736dup
g.83508725_83508728dup
-
-
POU3F4_000088
ACMG PVS1_Strong, PM2
PubMed: Jiang 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.406C>T
r.(?)
p.(Gln136*)
-
pathogenic
g.82763738C>T
g.83508730C>T
-
-
POU3F4_000043
-
PubMed: Waryah 2011
-
-
Unknown
-
-
-
-
-
LOVD
+?/.
1
-
c.421_422delinsTA
r.(?)
p.(Val141Ter)
ACMG
likely pathogenic (recessive)
g.82763753_82763754delinsTA
g.83508745_83508746delinsTA
-
-
POU3F4_000089
ACMG PVS1_Strong, PM2
PubMed: Jiang 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.425G>C
r.(?)
p.(Ser142Thr)
-
VUS
g.82763757G>C
g.83508749G>C
POU3F4(NM_000307.3):c.425G>C (p.(Ser142Thr))
-
POU3F4_000045
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
-
c.441del
r.(?)
p.(His147GlnfsTer94)
ACMG
likely pathogenic (recessive)
g.82763773del
g.83508765del
-
-
POU3F4_000090
ACMG PVS1_Strong, PM2
PubMed: Jiang 2021
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.447A>C
r.(?)
p.(Gly149=)
-
likely benign
g.82763779A>C
-
POU3F4(NM_000307.4):c.447A>C (p.G149=)
-
POU3F4_000078
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.468C>T
r.(?)
p.(Ala156=)
-
likely benign
g.82763800C>T
-
POU3F4(NM_000307.4):c.468C>T (p.A156=)
-
POU3F4_000079
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
1
c.471C>T
r.(?)
p.=
-
VUS
g.82763803C>T
g.83508795C>T
A157A
-
POU3F4_000001
found once, nonrecurrent change
PubMed: Tarpey 2009
-
-
Germline
-
-
-
-
-
Lucy Raymond
+/.
1
-
c.478C>T
r.(?)
p.(Gln160Ter)
ACMG
pathogenic (recessive)
g.82763810C>T
g.83508802C>T
-
-
POU3F4_000095
-
PubMed: Richard 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/?
1
1
c.483C>A
r.(?)
p.(Ser161Arg)
-
VUS
g.82763815C>A
g.83508807C>A
-
-
POU3F4_000057
-
MORL Deafness Variation Database
,
PubMed: Duzkale 2013
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
1
-
c.496C>T
r.(?)
p.(Leu166Phe)
-
VUS
g.82763828C>T
-
-
-
POU3F4_000094
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+, +/.
2
1
c.499C>T
r.(?)
p.(Arg167*)
-
pathogenic
g.82763831C>T
g.83508823C>T
-
-
POU3F4_000040
-
PubMed: Stankovic 2010
,
1 more item
-
-
SUMMARY record, Unknown
-
-
-
-
-
Global Variome, with Curator vacancy
+/.
1
-
c.502G>T
r.(?)
p.(Glu168Ter)
-
pathogenic
g.82763834G>T
g.83508826G>T
POU3F4(NM_000307.5):c.502G>T (p.E168*)
-
POU3F4_000050
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
?/?
1
1
c.506C>T
r.(?)
p.(Pro169Leu)
-
VUS
g.82763838C>T
g.83508830C>T
-
-
POU3F4_000058
-
MORL Deafness Variation Database
,
PubMed: Duzkale 2013
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+
1
1
c.540C>A
r.(?)
p.(Cys180*)
-
pathogenic
g.82763872C>A
g.83508864C>A
-
-
POU3F4_000059
-
MORL Deafness Variation Database
,
PubMed: Choi 2015
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
-?/.
1
-
c.549C>T
r.(?)
p.(His183=)
-
likely benign
g.82763881C>T
-
POU3F4(NM_000307.4):c.549C>T (p.H183=)
-
POU3F4_000093
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+, +/.
2
1
c.601_606del
r.(?)
p.(Phe201_Lys202del)
-
pathogenic
g.82763933_82763938del
g.83508925_83508930del
601_606delTTCAAA
-
POU3F4_000026
-
MORL Deafness Variation Database
,
PubMed: Hagiwara 1998
,
PubMed: Hagiwara 1998
-
-
SUMMARY record, Unknown
-
-
-
-
-
Global Variome, with Curator vacancy
+/.
1
1
c.607_610del
r.(?)
p.(Gln203Glufs*37)
-
pathogenic (recessive)
g.82763939_82763942del
-
603-610delCAAA
-
POU3F4_000060
-
PubMed: de Kok 1995
-
-
Germline
yes
-
-
-
-
Global Variome, with Curator vacancy
+/+, +/.
4
1
c.623T>A
r.(?)
p.(Leu208*), p.(Leu208Ter)
-
pathogenic, pathogenic (recessive)
g.82763955T>A
g.83508947T>A
c.623T>A
-
POU3F4_000033
-
PubMed: Choi 2013
,
PubMed: Choi 2013
,
PubMed: Lee 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
3
1
c.632C>T
r.(?)
p.(Thr211Met)
-
likely pathogenic, pathogenic (recessive)
g.82763964C>T
g.83508956C>T
c.632C>T
-
POU3F4_000008
-
PubMed: Choi 2013
,
PubMed: Choi 2013
,
PubMed: Choi 2013
-
-
Germline
-
-
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
Byung Yoon Choi
+/.
1
1
c.647G>A
r.(?)
p.(Gly216Glu)
-
pathogenic
g.82763979G>A
g.83508971G>A
-
-
POU3F4_000039
-
PubMed: Li 2010
-
-
Unknown
-
-
-
-
-
LOVD
+/.
1
1
c.648del
r.(?)
p.(Leu217TrpfsTer24)
-
pathogenic
g.82763980del
g.83508972del
651delG (D215X)
-
POU3F4_000019
not in 100 control chromosomes
PubMed: de Kok 1995
-
-
Germline
-
-
-
-
-
LOVD
+/+
1
1
c.648dup
r.(?)
p.(Leu217Valfs*9)
-
pathogenic
g.82763980dup
g.83508972dup
-
-
POU3F4_000062
-
MORL Deafness Variation Database
,
PubMed: Yang 2013
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/?
1
1
c.652del
r.(?)
p.(Ala218Argfs*23)
-
VUS
g.82763984del
g.83508976del
-
-
POU3F4_000063
-
MORL Deafness Variation Database
,
PubMed: Smith 1993
,
PubMed: Alford 2014
,
PubMed: Shearer 1993
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
-?/.
1
-
c.655C>T
r.(?)
p.(=)
-
likely benign
g.82763987C>T
-
POU3F4(NM_000307.5):c.655C>T (p.(Leu219=))
-
POU3F4_000096
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
1
c.683C>T
r.(?)
p.(Ser228Leu)
-
pathogenic
g.82764015C>T
g.83509007C>T
-
-
POU3F4_000030
-
PubMed: Vore 2005
-
-
Unknown
-
-
-
-
-
LOVD
+/., +?/.
3
1
c.686A>G
r.(?)
p.(Gln229Arg)
-
likely pathogenic, pathogenic (recessive)
g.82764018A>G
g.83509010A>G
c.686A>G
-
POU3F4_000007
-
PubMed: Choi 2013
,
PubMed: Choi 2013
-
-
Germline
-
-
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
Byung Yoon Choi
+/+, +/.
2
1
c.689C>T
r.(?)
p.(Thr230Ile)
-
pathogenic
g.82764021C>T
g.83509013C>T
-
-
POU3F4_000022
-
MORL Deafness Variation Database
,
PubMed: Friedman 1997
,
PubMed: Friedman 1997
-
-
SUMMARY record, Unknown
-
-
-
-
-
Global Variome, with Curator vacancy
+?/+?
1
1
c.695T>C
r.(?)
p.(Ile232Thr)
-
likely pathogenic
g.82764027T>C
g.83509019T>C
-
-
POU3F4_000064
-
1 more item
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+?/., ?/.
3
1
c.706G>A
r.(?)
p.(Glu236Lys)
ACMG
likely pathogenic (recessive), VUS
g.82764038G>A
g.83509030G>A
-
-
POU3F4_000046
VKGL data sharing initiative Nederland
PubMed: Mei 2021
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
-/.
1
-
c.708=
r.(=)
p.(Glu236=)
-
benign
g.82764040A>G
g.83509032=
POU3F4(NM_000307.5):c.708A>G (p.E236=)
-
POU3F4_000003
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/., ?/.
5
1
c.708A>G
r.(?)
p.(=)
-
benign, VUS
g.82764040A>G
g.83509032A>G
A708G
-
POU3F4_000003
1 more item
PubMed: Lee 2009
-
rs5921978
Germline
-
-
-
-
-
LOVD
-/.
2
-
c.710=
r.(=)
p.(Ala237=)
-
benign
g.82764042G>C
g.83509034=
POU3F4(NM_000307.4):c.710G>C (p.G237A), POU3F4(NM_000307.5):c.710G>C (p.G237A)
-
POU3F4_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
-/., ?/.
5
1
c.710G>C
r.(?)
p.(Gly237Ala)
-
benign, VUS
g.82764042G>C
g.83509034G>C
G710C
-
POU3F4_000004
1 more item
PubMed: Lee 2009
-
rs5921979
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.730A>G
r.(?)
p.(Asn244Asp)
-
VUS
g.82764062A>G
g.83509054A>G
POU3F4(NM_000307.3):c.730A>G (p.(Asn244Asp))
-
POU3F4_000053
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
1
1
c.731dup
r.(?)
p.(Asn244Lysfs*27)
-
pathogenic
g.82764063dup
g.83509055dup
-
-
POU3F4_000065
-
MORL Deafness Variation Database
,
PubMed: Moteki 2015
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
1
1
c.733A>G
r.(?)
p.Met245Val
-
VUS
g.82764065A>G
g.83509057A>G
M245V
-
POU3F4_000002
recurrent, found 2 times
PubMed: Tarpey 2009
-
-
Germline
-
-
-
-
-
Lucy Raymond
+?/.
1
-
c.747del
r.(?)
p.(Lys249Asnfs*4)
ACMG
likely pathogenic (recessive)
g.82764079del
g.83509071del
-
-
POU3F4_000100
-
-
-
-
Germline
yes
-
-
-
-
Hina Khan
+/+
1
1
c.772del
r.(?)
p.(Glu258Argfs*30)
-
pathogenic
g.82764104del
g.83509096del
-
-
POU3F4_000066
-
MORL Deafness Variation Database
,
PubMed: Bademci 2015
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
-?/.
1
-
c.844C>A
r.(?)
p.(Arg282=)
-
likely benign
g.82764176C>A
-
POU3F4(NM_000307.4):c.844C>A (p.R282=)
-
POU3F4_000083
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.845G>A
r.(?)
p.(Arg282Gln)
-
likely pathogenic
g.82764177G>A
g.83509169G>A
-
-
POU3F4_000047
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.846G>T
r.(?)
p.(Arg282=)
-
likely benign
g.82764178G>T
g.83509170G>T
POU3F4(NM_000307.4):c.846G>T (p.R282=)
-
POU3F4_000051
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+, +/.
2
1
c.853_854del
r.(?)
p.(Ile285Argfs*43)
-
pathogenic
g.82764185_82764186del
g.83509177_83509178del
-
-
POU3F4_000013
-
1 more item
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Thomas Parzefall
+/+, +/.
3
1
c.862_865del
r.(?)
p.(Val289Argfs*47)
-
pathogenic
g.82764194_82764197del
g.83509186_83509189del
862_865delAGTG
-
POU3F4_000015
not in 80 control chromosomes
PubMed: Bitner-Glindzicz 1995
,
1 more item
-
-
Germline, SUMMARY record
-
-
HphI-
-
-
Global Variome, with Curator vacancy
+/.
1
1
c.896del
r.(?)
p.(Lys299SerfsTer38)
-
pathogenic
g.82764228del
g.83509220del
895delA (L298X)
-
POU3F4_000020
not in 100 control chromosomes
PubMed: de Kok 1995
-
-
Germline
-
-
-
-
-
LOVD
+/+
1
1
c.902C>T
r.(?)
p.(Pro301Leu)
-
pathogenic
g.82764234C>T
g.83509226C>T
-
-
POU3F4_000068
-
MORL Deafness Variation Database
,
PubMed: Bademci 2015
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/.
1
1
c.907C>T
r.(?)
p.(Pro303Ser)
-
pathogenic
g.82764239C>T
g.83509231C>T
-
-
POU3F4_000028
-
PubMed: Cremers 2000
-
-
Unknown
-
-
-
-
-
LOVD
+/+
1
1
c.908C>A
r.(?)
p.(Pro303His)
-
pathogenic
g.82764240C>A
g.83509232C>A
-
-
POU3F4_000069
-
MORL Deafness Variation Database
,
PubMed: Park 2014
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/.
1
-
c.919_921del
r,(?)
p.(Glu307del)
-
pathogenic
g.82764251_82764253del
g.83509243_83509245del
919_921delGAG
-
POU3F4_000098
combination of alleles not reported
PubMed: Wu 2019
-
-
Germline
-
1/1291 cases hearing loss
-
-
-
Johan den Dunnen
+/.
1
1
c.923T>A
r.(?)
p.(Ile308Asn)
-
pathogenic
g.82764255T>A
g.83509247T>A
-
-
POU3F4_000038
-
PubMed: Marlin 2009
-
-
Unknown
-
-
-
-
-
LOVD
+/+
1
1
c.923T>C
r.(?)
p.(Ile308Thr)
-
pathogenic
g.82764255T>C
g.83509247T>C
-
-
POU3F4_000070
-
MORL Deafness Variation Database
,
PubMed: Bademci 2015
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/., ?/.
2
1
c.925T>C
r.(?)
p.(Ser309Pro)
-
pathogenic, VUS
g.82764257T>C
g.83509249T>C
-
-
POU3F4_000031
VKGL data sharing initiative Nederland
PubMed: Wang 2006
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
1
c.927_929del
r.(?)
p.(Ser310del)
-
pathogenic (recessive)
g.82764259_82764261del
g.83509251_83509253del
927_929delCTC
-
POU3F4_000035
-
PubMed: Lee 2009
-
-
Germline
yes
-
-
-
-
LOVD
?/.
1
-
c.931C>G
r.(?)
p.(Leu311Val)
-
VUS
g.82764263C>G
-
POU3F4(NM_000307.4):c.931C>G (p.(Leu311Val))
-
POU3F4_000097
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
-
c.935C>A
r.(?)
p.(Ala312Glu)
-
likely pathogenic
g.82764267C>A
-
-
-
POU3F4_000091
-
-
-
-
Unknown
-
-
-
-
-
Gunnar Schmidt
+/+, +/.
3
1
c.935C>T
r.(?)
p.(Ala312Val)
-
pathogenic
g.82764267C>T
g.83509259C>T
-
-
POU3F4_000016
-
PubMed: Bitner-Glindzicz 1995
,
1 more item
-
-
De novo, SUMMARY record
-
-
AccI+
-
-
Global Variome, with Curator vacancy
+/+, +/., +?/.
4
1
c.950dup
r.(?)
p.(Leu317Phefs*12), p.(Leu317PhefsTer12)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.82764282dup
g.83509274dup
950dupT, c.950dupT
-
POU3F4_000006, POU3F4_000071
-
MORL Deafness Variation Database
,
PubMed: Smith 1993
,
PubMed: Alford 2014
,
PubMed: Shearer 1993
,
2 more items
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
Byung Yoon Choi
+/.
1
-
c.950T>A
r,(?)
p.(Leu317Ter)
-
pathogenic
g.82764282T>A
g.83509274T>A
-
-
POU3F4_000099
combination of alleles not reported
PubMed: Wu 2019
-
-
Germline
-
1/1291 cases hearing loss
-
-
-
Johan den Dunnen
+/.
1
1
c.950T>G
r.(?)
p.(Leu317Trp)
-
pathogenic
g.82764282T>G
g.83509274T>G
-
-
POU3F4_000021
not in 100 control chromosomes
PubMed: de Kok 1995
-
-
Germline
-
-
-
-
-
LOVD
?/?
1
1
c.964G>A
r.(?)
p.(Val322Met)
-
VUS
g.82764296G>A
g.83509288G>A
-
-
POU3F4_000072
-
MORL Deafness Variation Database
,
PubMed: Duzkale 2013
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/.
1
1
c.967C>G
r.(?)
p.(Arg323Gly)
-
pathogenic
g.82764299C>G
g.83509291C>G
-
-
POU3F4_000025
somatic mosaicism 50% in PBL
PubMed: de Kok 1997
-
-
De novo
-
-
-
-
-
LOVD
+/.
1
1
c.973T>A
r.(?)
p.(Trp325Arg)
-
pathogenic
g.82764305T>A
g.83509297T>A
-
-
POU3F4_000041
-
PubMed: Schild 2011
-
-
Unknown
-
-
-
-
-
LOVD
?/.
1
-
c.974G>C
r.(?)
p.(Trp325Ser)
-
VUS
g.82764306G>C
-
-
-
POU3F4_000101
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.975G>T
r.(?)
p.(Trp325Cys)
-
likely pathogenic
g.82764307G>T
-
-
-
POU3F4_000080
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
1
c.983A>C
r.(?)
p.(Asn328Thr)
-
pathogenic
g.82764315A>C
g.83509307A>C
-
-
POU3F4_000029
-
PubMed: Cremers 2000
-
-
Unknown
-
-
-
-
-
LOVD
+/+, +/.
2
1
c.985C>G
r.(?)
p.(Arg329Gly)
-
pathogenic
g.82764317C>G
g.83509309C>G
-
-
POU3F4_000023
-
MORL Deafness Variation Database
,
PubMed: Friedman 1997
,
PubMed: Friedman 1997
-
-
SUMMARY record, Unknown
-
-
-
-
-
Global Variome, with Curator vacancy
+/.
1
1
c.986G>C
r.(?)
p.(Arg329Pro)
-
pathogenic (recessive)
g.82764318G>C
g.83509310G>C
-
-
POU3F4_000036
-
PubMed: Lee 2009
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
1
c.990A>T
r.(?)
p.(Arg330Ser)
-
pathogenic (recessive)
g.82764322A>T
g.83509314A>T
-
-
POU3F4_000024
-
PubMed: de Kok 1997
-
-
Germline/De novo (untested)
-
-
-
-
-
LOVD
?/.
1
-
c.999G>T
r.(?)
p.(Glu333Asp)
-
VUS
g.82764331G>T
g.83509323G>T
POU3F4(NM_000307.4):c.999G>T (p.E333D)
-
POU3F4_000052
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/.
2
1
c.1000A>G
r.(?)
p.(Lys334Glu)
-
likely pathogenic, pathogenic
g.82764332A>G
g.83509324A>G
-
-
POU3F4_000017
not in 100 control chromosomes, VKGL data sharing initiative Nederland
PubMed: de Kok 1995
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
3
1
c.1060del
r.(?)
p.(Thr354Glnfs*115), p.(Thr354GlnfsTer115)
-
pathogenic, pathogenic (recessive)
g.82764392del
g.83509384del
1069delA, c.1060delA
-
POU3F4_000075
-
PubMed: Choi 2013
,
PubMed: Choi 2013
,
PubMed: Choi 2013
,
1 more item
-
-
Germline, SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
+/., +?/.
3
1
c.1084T>C
r,(?), r.(?)
p.(*362Argext*113), p.(Ter362ArgextTer113)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.82764416T>C
g.83509408T>C
-
-
POU3F4_000009
combination of alleles not reported
PubMed: Choi 2013
,
PubMed: Choi 2013
,
PubMed: Wu 2019
-
-
Germline
-
1/1291 cases hearing loss
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
Byung Yoon Choi
+?/.
1
-
c.1086A>G
r.(?)
p.(*362Trpext*113)
-
likely pathogenic
g.82764418A>G
-
-
-
POU3F4_000081
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/+?
1
1
c.1086_*3del
r.(?)
p.(*362Trpext*105)
-
likely pathogenic
g.82764418_82764421del
g.83509410_83509413del
-
-
POU3F4_000076
-
1 more item
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
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