Global Variome shared LOVD
GNE (glucosamine (UDP-N-acetyl)-2-epimerase/N-acety...)
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Curators:
Fleur Garton
,
Tara Voogel
, and
Johan den Dunnen
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Unique variants in the GNE gene
This database is one of the gene variant databases from the
Leiden Muscular Dystrophy pages
The variants shown are described using the NM_001128227.2 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.
219 entries on 3 pages. Showing entries 1 - 100.
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Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
+/.
1
3i
c.709 + 1del
r.spl
p.?
-
pathogenic (recessive)
g.36246028del
g.36246031del
709 + 1delG
-
GNE_000035
-
PubMed: Khadilkar 2022
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/.
1
_1_3i
c.-35077_710-2769del
r.0
p.0
-
pathogenic
g.36239759_36312027del
g.36239762_36312030del
-
-
GNE_000118
-
PubMed: Zhu 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
_1_8i
c.(?_-112)_(1726+1_1727-1)del
r.0?
p.0?
-
pathogenic
g.(36220018_36222773)_(36277053_?)del
-
-
-
GNE_000011
>35.7 Kb deletion (exons 1-9); exon 13 not from GNE gene
PubMed: Del Bo 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
1
c.-65C>A
r.(=)
p.(=)
-
VUS
g.36277006G>T
g.36277009G>T
NM_001190388.1:34C>A (P12T)
-
GNE_000126
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.-11C>G
r.(?)
p.(=)
-
VUS
g.36276952G>C
-
GNE(NM_001128227.2):c.-11C>G (p.(=))
-
GNE_000218
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/., ?/.
2
5' UTR
c.-10G>A
r.(?)
p.(=)
-
likely benign, VUS
g.36276951C>T
g.36276954C>T
GNE(NM_001128227.2):c.-10G>A (p.(=))
-
GNE_000175
no second variant, VKGL data sharing initiative Nederland
PubMed: Nallamilli 2018
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Leiden
?/.
2
1, 5' UTR
c.-4C>T
r.(=), r.(?)
p.(=)
-
VUS
g.36276945G>A
g.36276948G>A
NM_001190388.1:95C>T (S32L)
-
GNE_000129
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
5' UTR
c.-3G>A
r.(?)
p.(=)
-
VUS
g.36276944C>T
g.36276947C>T
-
-
GNE_000174
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
3
c.?
r.?
p.?
-
pathogenic (recessive)
g.?
-
326A>G (His109Arg)
-
PTCH1_000000
-
PubMed: Khadilkar 2022
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.33A>G
r.(?)
p.(=)
-
VUS
g.36276909T>C
-
GNE(NM_001190388.1):c.131A>G (p.(His44Arg))
-
GNE_000216
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
1
c.43G>C
r.(?)
p.(Gly15Arg)
-
VUS
g.36276899C>G
g.36276902C>G
-
-
GNE_000173
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-/.
1
-
c.51+34T>C
r.(=)
p.(=)
-
benign
g.36276857A>G
-
GNE(NM_001128227.3):c.51+34T>C
-
GNE_000202
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
1i
c.51+7981_52-8189del
r.?
p.?
-
pathogenic
g.36257594_36268921del
g.36257597_36268924del
-
-
GNE_000113
mRNA expression reduced to 0.50
PubMed: Garland 2017
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
2
1i
c.51+9423_52-8182del
r.0
p.0
-
pathogenic
g.36257583_36267475del
g.36257586_36267478del
-
-
GNE_000116
-
PubMed: Zhu 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1i
c.51+10408_52-10008del
r.0
p.0
-
pathogenic (recessive)
g.36259402_36266483del
g.36259405_36266486del
-
-
GNE_000179
7.082 kb deletion
PubMed: Chakravorty 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.51+13314T>C
r.(=)
p.(=)
-
benign
g.36263577A>G
-
GNE(NM_001128227.3):c.51+13314T>C
-
GNE_000201
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
1i
c.52-12264_52-8148del
r.0?
p.0?
-
pathogenic
g.36257571_36261687del
g.36257574_36261690del
-
-
GNE_000121
-
PubMed: Zhu 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
4
1i
c.52-11451_52-447del
r.0, r.0?
p.0, p.0?
-
pathogenic
g.36249843_36260847del
g.36249846_36260850del
-
-
GNE_000115
no detectable transcript
PubMed: Zhu 2017
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
1
1i_5i
c.52-8128_1075+531del
r.0
p.0
-
pathogenic
g.36233459_36257595del
g.36233462_36257598del
-
-
GNE_000120
-
PubMed: Zhu 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1i_2i
c.52-4733_257+1241del
r.0?
p.0?
-
pathogenic
g.36247950_36254129del
g.36247953_36254132del
-
-
GNE_000117
-
PubMed: Zhu 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1i_3i
c.52-3652_709+469dup
r.52_709dup
p.?
-
pathogenic
g.36245582_36253069dup
g.36245585_36253072dup
-
-
GNE_000119
two breakpoints found, somatic mosaicism
PubMed: Zhu 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.78A>T
r.(?)
p.(Lys26Asn)
-
VUS
g.36249368T>A
-
GNE(NM_001128227.3):c.78A>T (p.(Lys26Asn))
-
GNE_000219
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
2
c.79C>T
r.(?)
p.(Arg27*)
-
pathogenic
g.36249367G>A
g.36249370G>A
-
-
GNE_000172
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.95T>C
r.(?)
p.(Met32Thr)
ACMG
VUS
g.36249351A>G
g.36249354A>G
-
-
GNE_000193
-
PubMed: Chakravorty 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
2
c.97G>T
r.(?)
p.(Glu33*), p.(Glu33Ter)
ACMG
pathogenic, pathogenic (recessive)
g.36249349C>A
g.36249352C>A
-
-
GNE_000171
-
PubMed: Chakravorty 2020
,
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
+/.
2
2
c.98A>G
r.(?)
p.(Glu33Gly)
-
pathogenic
g.36249348T>C
g.36249351T>C
A>G (E2G)
-
GNE_000020
-
PubMed: Saechao 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.115C>T
r.(?)
p.(Arg39*)
-
pathogenic
g.36249331G>A
g.36249334G>A
C>T (R8X)
-
GNE_000012
-
PubMed: Saechao 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
2
c.116G>A
r.(?)
p.(Arg39Gln)
-
VUS
g.36249330C>T
g.36249333C>T
-
-
GNE_000170
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
3
2
c.124C>T
r.(?)
p.(Arg42Trp)
ACMG
pathogenic, pathogenic (recessive)
g.36249322G>A
g.36249325G>A
(R11W), R11W
-
GNE_000013
not in 100 control chromosomes
PubMed: Chakravorty 2020
,
PubMed: Huizing 2004
,
PubMed: Khadilkar 2022
-
-
Germline, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/., +?/.
11
2
c.131G>C
r.(?), r.131g>c
p.(Cys44Ser), p.Cys44Ser
-
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.36249315C>G
g.36249318C>G
89G>C (C13S), C13S, NM_005476.4:c.38G>C
-
GNE_000014
two genetic etiologies
PubMed: Kim 2006
,
PubMed: Liu 2015
,
PubMed: Park 2017
,
PubMed: Saito 2004
,
PubMed: Tomimitsu 2004
,
1 more item
-
-
Germline
-
1/209 cases
-
-
-
Johan den Dunnen
+?/.
1
2
c.146del
r.(?)
p.(Asn49Thrfs*33)
-
likely pathogenic
g.36249301del
g.36249304del
-
-
GNE_000015
-
-
-
-
Germline
-
-
-
-
-
Tom Winder
+/.
2
2
c.172C>T
r.(?)
p.(Pro58Ser)
-
pathogenic
g.36249274G>A
g.36249277G>A
79C>T (P27S)
-
GNE_000016
-
PubMed: Broccolini 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
2
2
c.173C>T
r.(?)
p.(Pro58Leu)
-
likely pathogenic, pathogenic
g.36249273G>A
g.36249276G>A
80C>T (Pro27Leu), GNE(NM_001128227.2):c.173C>T (p.(Pro58Leu))
-
GNE_000125
unknown variant 2nd chromosome, VKGL data sharing initiative Nederland
PubMed: Nalini 2013
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
+/., +?/.
2
2
c.179T>C
r.(?)
p.(Met60Thr)
-
likely pathogenic (recessive), pathogenic
g.36249267A>G
g.36249270A>G
M29T
-
GNE_000017
-
PubMed: Kim 2006
,
PubMed: Park 2017
-
-
Germline
-
1/209 cases
-
-
-
Johan den Dunnen
+/.
1
2
c.188_197dup
r.(?)
p.(Glu66Aspfs*2)
-
pathogenic
g.36249250_36249259dup
g.36249253_36249262dup
dupTTAAAACCGA (fs*)
-
GNE_000018
-
PubMed: Nishino 2002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.200C>T
r.(?)
p.(Pro67Leu)
-
pathogenic
g.36249246G>A
g.36249249G>A
107C>T (P36L)
-
GNE_000019
-
PubMed: Eisenberg 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2i
c.257+1G>A
r.spl
p.?
-
pathogenic
g.36249188C>T
g.36249191C>T
-
-
GNE_000169
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
-?/.
1
-
c.258-18A>G
r.(=)
p.(=)
-
likely benign
g.36246497T>C
-
GNE(NM_001128227.2):c.258-18A>G (p.(=))
-
GNE_000214
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
2
3
c.268C>T
r.(?)
p.(Arg90*), p.(Arg90Ter)
ACMG
pathogenic, pathogenic (recessive)
g.36246469G>A
g.36246472G>A
-
-
GNE_000168
-
PubMed: Chakravorty 2020
,
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
+?/.
1
3
c.271A>G
r.(?)
p.(Met91Val)
-
likely pathogenic
g.36246466T>C
g.36246469T>C
-
-
GNE_000090
-
PubMed: Celeste 2014
,
Journal: Celeste 2014
-
-
Unknown
?
-
-
-
-
Frank Celeste
?/.
2
2, 3
c.300C>T
r.(=), r.(?)
p.(=)
-
VUS
g.36246437G>A
g.36246440G>A
NM_001190388.1:192C>T (N64N)
-
GNE_000128
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/., ?/.
5
3
c.304A>T
r.(?)
p.(Arg102Trp)
-
pathogenic, VUS
g.36246433T>A
g.36246436T>A
A>T (R71W)
-
GNE_000021
no second variant, variant apparently homozygous; no second variant
PubMed: Nallamilli 2018
,
PubMed: Saechao 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
+?/.
1
-
c.313A>G
r.(?)
p.(Thr105Ala)
-
likely pathogenic
g.36246424T>C
-
-
-
GNE_000205
-
-
-
-
CLASSIFICATION record
-
-
-
-
-
MobiDetails
+?/.
1
-
c.326G>A
r.(?)
p.(Gly109Glu)
-
pathogenic
g.36246411C>T
-
GNE c.218G>A
-
GNE_000208
-
PubMed: Hartnett 2022
,
PubMed: Tavakoli 2023
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.350A>T
r.(?)
p.(Glu117Val)
-
pathogenic (recessive)
g.36246387T>A
g.36246390T>A
-
-
GNE_000183
-
PubMed: Yu 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.358G>C
r.(?)
p.(Gly120Arg)
-
pathogenic
g.36246379C>G
g.36246382C>G
265G>C (G89R)
-
GNE_000022
-
PubMed: Liewluck 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
2
2
c.390T>G
r.(=)
p.(=)
-
VUS
g.36246347A>C
g.36246350A>C
NM_001190388.1:282T>G (L94L)
-
GNE_000127
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
2
-
c.395G>A
r.(?)
p.(Arg132His)
-
VUS
g.36246342C>T
g.36246345C>T
GNE(NM_001128227.2):c.395G>A (p.(Arg132His))
-
GNE_000102
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
?/.
2
3
c.398T>C
r.(?)
p.(Leu133Pro)
-
VUS
g.36246339A>G
g.36246342A>G
-
-
GNE_000167
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
3
c.410T>C
r.(?)
p.(Ile137Thr)
-
VUS
g.36246327A>G
g.36246330A>G
-
-
GNE_000166
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
3
c.422A>G
r.(?)
p.(His141Arg)
-
VUS
g.36246315T>C
g.36246318T>C
-
-
GNE_000165
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/., +?/.
8
3
c.478C>T
r.(?)
p.(Arg160*), p.(Arg160Ter)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.36246259G>A
g.36246262G>A
-
-
GNE_000084
-
PubMed: Celeste 2014
,
Journal: Celeste 2014
,
PubMed: Chakravorty 2020
,
PubMed: Ganapathy 2019
,
1 more item
-
-
Germline, Germline/De novo (untested), Unknown
?
-
-
-
-
Johan den Dunnen
,
Tom Winder
,
Frank Celeste
+/.
2
3
c.479G>A
r.(?), r.479g>a
p.(Arg160Gln), p.Arg160Gln
-
pathogenic
g.36246258C>T
g.36246261C>T
R129Q
-
GNE_000023
-
PubMed: Kim 2006
,
PubMed: Tomimitsu 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.489C>G
r.(?)
p.(His163Gln)
-
pathogenic
g.36246248G>C
g.36246251G>C
447C>G (H132Q)
-
GNE_000024
-
PubMed: Nishino 2002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.490_491dup
r.(?)
p.(Glu165Leufs*18)
ACMG
pathogenic (recessive)
g.36246246_36246247dup
.36246249_36246250dup
c.397_398dupAT (Glu134fs)
-
GNE_000189
-
PubMed: Chakravorty 2020
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.491T>C
r.(?)
p.(Ile164Thr)
-
VUS
g.36246246A>G
-
GNE(NM_001128227.2):c.491T>C (p.(Ile164Thr))
-
GNE_000186
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
3
c.497G>T
r.(?)
p.(Gly166Val)
-
pathogenic
g.36246240C>A
g.36246243C>A
404G>T (G135V)
-
GNE_000025
-
PubMed: Darvish 2002
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/., +?/.
2
3
c.518T>C
r.(?)
p.(Ile173Thr)
-
likely pathogenic (recessive), pathogenic
g.36246219A>G
g.36246222A>G
T>C (I142T)
-
GNE_000026
-
PubMed: Park 2017
,
PubMed: Saechao 2010
-
-
Germline
-
1/209 cases
-
-
-
Johan den Dunnen
?/.
1
3
c.548A>C
r.(?)
p.(Lys183Thr)
-
VUS
g.36246189T>G
g.36246192T>G
-
-
GNE_000164
no second variant
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
-
c.557A>G
r.(?)
p.(His186Arg)
-
pathogenic (recessive)
g.36246180T>C
g.36246183T>C
-
-
GNE_000194
-
PubMed: Grecu 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
3
c.559T>C
r.(?)
p.(Tyr187His)
-
likely pathogenic
g.36246178A>G
g.36246181A>G
-
-
GNE_000092
-
PubMed: Celeste 2014
,
Journal: Celeste 2014
-
-
Unknown
?
-
-
-
-
Frank Celeste
?/.
1
3
c.574A>G
r.(?)
p.(Thr192Ala)
-
VUS
g.36246163T>C
g.36246166T>C
-
-
GNE_000163
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/., +?/.
6
3
c.577C>T
r.(?)
p.(Arg193Cys)
-
likely pathogenic, pathogenic, pathogenic (recessive)
g.36246160G>A
g.36246163G>A
484C>T (Arg162Cys), 535C>T (R162C), NM_005476.5:c.484C>T
-
GNE_000027
not in 200 control chromosomes, VKGL data sharing initiative Nederland
PubMed: Del Bo 2003
,
PubMed: Nalini 2013
,
PubMed: Nallamilli 2018
,
PubMed: Westra 2019
-
-
CLASSIFICATION record, Germline
-
-
PstI+
-
-
Johan den Dunnen
,
Madhuri Hegde
,
VKGL-NL_Nijmegen
?/.
1
3
c.580A>G
r.(?)
p.(Ser194Gly)
-
VUS
g.36246157T>C
g.36246160T>C
-
-
GNE_000162
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
2
3
c.604A>G
r.(?)
p.(Met202Val)
-
pathogenic
g.36246133T>C
g.36246136T>C
562A>G (M171V)
-
GNE_000028
-
PubMed: Broccolini 2002
,
OMIM:var0016
-
-
Germline
-
-
NlaIII-
-
-
Johan den Dunnen
?/.
1
-
c.606G>A
r.(?)
p.(Met202Ile)
-
VUS
g.36246131C>T
-
-
-
GNE_000200
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
26
3
c.620A>T
r.(?), r.620a>u
p.(Asp207Val), p.Asp207Val
-
pathogenic, pathogenic (recessive)
g.36246117T>A
g.36246120T>A
527A>T (Asp176Val), 578A>T (D176V), A>T (D176V), D176V, NM_005476.4:c.527A>T
-
GNE_000029
-
PubMed: Choi 2015
,
PubMed: Motozaki 2007
,
PubMed: Nallamilli 2018
,
PubMed: Nishino 2002
,
5 more items
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
+/., +?/.
2
3
c.622C>T
r.(?)
p.(Arg208Cys)
-
likely pathogenic, pathogenic
g.36246115G>A
g.36246118G>A
580C>T (R177C)
-
GNE_000030
combination of variants not reported
PubMed: Nishino 2002
,
PubMed: Topf 2020
-
-
Germline
-
2/1001 cases
-
-
-
Johan den Dunnen
+/.
2
3
c.628C>T
r.(?)
p.(Leu210Phe)
-
pathogenic
g.36246109G>A
g.36246112G>A
535C>T (L179F), C>T (L179F)
-
GNE_000031
unknown variant 2nd chromosome
PubMed: Bruno 2008
,
PubMed: Grandis 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
3
c.661C>T
r.(?)
p.(Leu221Phe)
-
VUS
g.36246076G>A
g.36246079G>A
-
-
GNE_000161
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
1
3
c.691A>T
r.(?)
p.(Ile231Phe)
-
pathogenic
g.36246046T>A
g.36246049T>A
649A>T (I200F)
-
GNE_000032
-
PubMed: Eisenberg 2003
-
-
Germline
-
-
AluI+
-
-
Johan den Dunnen
-/., -?/.
2
3
c.693C>T
r.(?)
p.(=), p.(Ile231=)
-
benign, likely benign
g.36246044G>A
g.36246047G>A
GNE(NM_001128227.2):c.693C>T (p.(Ile231=)), NM_005476.5:c.600C>T (I200I)
-
GNE_000085
VKGL data sharing initiative Nederland
from website {DBsub-Emory}
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
Madhuri Hegde
,
VKGL-NL_Leiden
+/.
1
3
c.694_697dup
r.(?)
p.(Arg233Hisfs*7)
-
pathogenic
g.36246042_36246045dup
g.36246045_36246048dup
694_697dupATTC
-
GNE_000160
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.697C>T
r.(?)
p.(Arg233Cys)
-
VUS
g.36246040G>A
g.36246043G>A
GNE(NM_005476.6):c.604C>T (p.R202C)
-
GNE_000181
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
3
c.698G>T
r.(?)
p.(Arg233Leu)
-
pathogenic
g.36246039C>A
g.36246042C>A
R202L
-
GNE_000033
not in 100 control chromosomes
PubMed: Huizing 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.705G>A
r.(?)
p.(Trp235*)
-
pathogenic
g.36246032C>T
g.36246035C>T
G>A (W204X)
-
GNE_000034
-
PubMed: Saechao 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.706C>G
r.(?)
p.(Leu236Val)
-
VUS
g.36246031G>C
-
GNE(NM_001128227.2):c.706C>G (p.L236V)
-
GNE_000180
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/., ?/.
3
3
c.709G>A
r.(?)
p.(Gly237Ser)
-
pathogenic, VUS
g.36246028C>T
g.36246031C>T
616G>A (G206S)
-
GNE_000036
no second variant
PubMed: Broccolini 2004
,
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
+/.
2
3i
c.709+1del
r.(spl?)
p.(Gly237fs*4)
-
pathogenic
g.36246028del
g.36246031del
616delG (G206fsX4)
-
GNE_000035
-
PubMed: Broccolini 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.709+5G>A
r.spl?
p.?
-
likely benign
g.36246023C>T
-
GNE(NM_001128227.3):c.709+5G>A
-
GNE_000217
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.709+7A>G
r.(=)
p.(=)
-
likely benign
g.36246021T>C
-
GNE(NM_001128227.2):c.709+7A>G (p.(=))
-
GNE_000206
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
-
c.715G>A
r.(?)
p.(Asp239Asn)
-
likely pathogenic (recessive)
g.36236976C>T
g.36236979C>T
-
-
GNE_000182
-
PubMed: Park 2017
-
-
Germline
-
1/209 cases
-
-
-
Johan den Dunnen
+/., +?/.
2
-
c.717T>G
r.(?)
p.(Asp239Glu)
-
likely pathogenic, pathogenic (recessive)
g.36236974A>C
g.36236977A>C
-
-
GNE_000195
-
PubMed: Grecu 2021
-
rs35224402
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
MobiDetails
+/., ?/.
2
-
c.729dup
r.(?)
p.(Asp244ArgfsTer13)
-
pathogenic, VUS
g.36236964dup
g.36236967dup
GNE(NM_001128227.2):c.729dupA (p.(Asp244ArgfsTer13)), GNE(NM_005476.7):c.636dupA (p.D213Rfs*13)
-
GNE_000178
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_VUmc
+?/., ?/.
2
4
c.731A>T
r.(?)
p.(Asn244Val), p.(Asp244Val)
-
likely pathogenic, VUS
g.36236960T>A
g.36236963T>A
-
-
GNE_000093
-
PubMed: Celeste 2014
,
Journal: Celeste 2014
,
PubMed: Nallamilli 2018
-
-
Germline, Unknown
?
-
-
-
-
Madhuri Hegde
,
Frank Celeste
+/., +?/.
3
4
c.740T>C
r.(?)
p.(Val247Ala)
-
likely pathogenic, pathogenic
g.36236951A>G
g.36236954A>G
698T>C (V216A), V216A
-
GNE_000037
not in 36 control chromosomes
PubMed: Huizing 2004
,
PubMed: Nallamilli 2018
,
PubMed: Vasconcelos 2002
-
-
Germline
yes
-
Fnu4HI+
-
-
Johan den Dunnen
,
Madhuri Hegde
?/.
1
4
c.748C>A
r.(?)
p.(Gln250Lys)
-
VUS
g.36236943G>T
g.36236946G>T
-
-
GNE_000159
no second variant in GNE
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/., ?/.
2
4
c.766G>A
r.(?)
p.(Asp256Asn)
-
pathogenic, VUS
g.36236925C>T
g.36236928C>T
724G>A (D225N)
-
GNE_000010
not in 132 control chromosomes
PubMed: Eisenberg 2001
,
PubMed: Eisenberg 2003
,
OMIM:var0011
,
PubMed: Nallamilli 2018
-
-
Germline
-
-
TspRI-
-
-
Johan den Dunnen
,
Madhuri Hegde
?/.
1
4
c.788T>C
r.(?)
p.(Met263Thr)
-
VUS
g.36236903A>G
g.36236906A>G
-
-
GNE_000158
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
-
c.792T>A
r.(?)
p.(Phe264Leu)
-
VUS
g.36236899A>T
-
-
-
GNE_000199
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
4
c.803T>C
r.(?)
p.(Leu268Ser)
-
VUS
g.36236888A>G
g.36236891A>G
-
-
GNE_000157
-
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
?/.
1
4
c.804G>C
r.(?)
p.(Leu268Phe)
-
VUS
g.36236887C>G
g.36236890C>G
-
-
GNE_000156
variant apparently homozygous
PubMed: Nallamilli 2018
-
-
Germline
-
-
-
-
-
Madhuri Hegde
+/.
2
4
c.815T>G
r.(?)
p.(Ile272Ser)
-
pathogenic
g.36236876A>C
g.36236879A>C
T>G (I241S)
-
GNE_000038
-
PubMed: Ro 2005
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
3
4
c.816_820del
r.(?)
p.(Ser273Ter)
ACMG
likely pathogenic (recessive), pathogenic (recessive)
g.36236873_36236877del
g.36236876_36236880del
816_820delCTCAT, c.816_820delCTCAT
-
GNE_000192
-
PubMed: Chakravorty 2020
,
PubMed: Khadilkar 2022
-
-
Germline, Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
+/.
5
4
c.829C>T
r.(?)
p.(Arg277Trp)
-
pathogenic
g.36236862G>A
g.36236865G>A
736C>T (R246W)
-
GNE_000039
-
PubMed: Darvish 2002
,
PubMed: Nallamilli 2018
,
PubMed: Stober 2010
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
,
Madhuri Hegde
+/., +?/.
6
4
c.830G>A
r.(?)
p.(Arg277Gln)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.36236861C>T
g.36236864C>T
737G>A (R246Q), 788G>A (R246Q), G>A (R246Q)
-
GNE_000009
not in 132 control chromosomes, variant apparently homozygous
PubMed: Broccolini 2004
,
PubMed: Chakravorty 2020
,
PubMed: Nallamilli 2018
,
PubMed: Saechao 2010
,
1 more item
-
-
Germline
-
-
NlaIV-
-
-
Johan den Dunnen
,
Madhuri Hegde
+/.
1
4i
c.862+4A>G
r.710_862del
p.Asp238_Gly288del
-
pathogenic
g.36236825T>C
g.36236828T>C
IVS4+4A>G
-
GNE_000040
-
PubMed: Nishino 2002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
5
c.881G>T
r.881g>u
p.Arg294Leu
-
pathogenic
g.36234111C>A
g.36234114C>A
788G>T (R263L)
-
GNE_000003
no variants 2nd chromosome
PubMed: Seppala
,
OMIM:var0003
-
rs121908623
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.887T>C
r.(?)
p.(Met296Thr)
-
VUS
g.36234105A>G
-
GNE(NM_005476.7):c.794T>C (p.M265T)
-
GNE_000207
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/.
1
5
c.889C>T
r.889c>u
p.Arg297Trp
-
pathogenic
g.36234103G>A
g.36234106G>A
796C>T (R266W)
-
GNE_000001
no variants 2nd chromosome
PubMed: Seppala
,
OMIM:var0001
-
rs121908621
Germline
-
-
-
-
-
Johan den Dunnen
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