Global Variome shared LOVD
GUCY2D (guanylate cyclase 2D, membrane (retina-spe...))
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All variants in the GUCY2D gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_000180.3 transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
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
Allele
: On which allele is the variant located? Does not necessarily imply inheritance! 'Paternal' (confirmed or inferred), 'Maternal' (confirmed or inferred), 'Parent #1' or #2 for compound heterozygosity without having screened the parents, 'Unknown' for heterozygosity without having screened the parents, 'Both' for homozygozity.
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.
1115 entries on 12 pages. Showing entries 1 - 100.
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Effect
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
-?/.
-
c.-20del
r.(?)
p.(=)
-
likely benign
g.7906042del
g.8002724del
-
-
GUCY2D_000076
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
-
c.-18del
r.(?)
p.(?)
-
likely pathogenic
g.7906044del
g.8002726del
GUCY2D -18 5' UTR 1 bp del (G)
-
GUCY2D_000285
no alteration in cyclase activity, but expected to reduce RNA levels, which in this setup could not be replicated (strong promoter); obsolete nucleotide annotation, correct annotation extrapolated from databases; homozygous
PubMed: Tucker 2004
-
-
In vitro (cloned)
yes
-
-
-
-
LOVD
-/.
-
c.-10+5A>G
r.spl?
p.?
-
benign
g.7906057A>G
g.8002739A>G
-
-
GUCY2D_000044
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
-
c.?
r.(?)
p.?
-
likely pathogenic
g.?
-
Pro701Ser
-
MYH2_000008
-
PubMed: Booij 2005
-
-
Germline
-
-
-
-
-
Julia Lopez
+?/.
-
c.?
r.(?)
p.?
-
likely pathogenic
g.?
-
Pro1069ArgfsX37
-
MYH2_000008
-
PubMed: Booij 2005
-
-
Germline
-
-
-
-
-
Julia Lopez
+?/.
-
c.?
r.(?)
p.?
-
likely pathogenic
g.?
-
Pro701Ser
-
MYH2_000008
-
PubMed: Booij 2005
-
-
Germline
-
-
-
-
-
Julia Lopez
+?/.
-
c.?
r.(?)
p.?
-
likely pathogenic
g.?
-
Arg768Trp
-
MYH2_000008
-
PubMed: Booij 2005
-
-
Germline
-
-
-
-
-
Julia Lopez
-/.
-
c.?
r.(?)
p.?
-
benign
g.?
-
2011C>T
-
MYH2_000008
-
PubMed: Booij 2005
-
-
Germline
-
-
-
-
-
Julia Lopez
-/.
-
c.?
r.(?)
p.?
-
benign
g.?
-
2419C>A
-
MYH2_000008
-
PubMed: Yamanoshita 2005
;
PubMed: Booij 2005
-
-
Germline
-
-
-
-
-
Julia Lopez
-/.
-
c.?
r.(?)
p.?
-
benign
g.?
-
3528C>T
-
MYH2_000008
-
PubMed: Yamanoshita 2005
;
PubMed: Booij 2005
-
-
Germline
-
-
-
-
-
Julia Lopez
-/.
-
c.?
r.(?)
p.?
-
benign
g.?
-
814C>T
-
MYH2_000008
-
PubMed: Booij 2005
-
-
Germline
-
-
-
-
-
Julia Lopez
-/.
-
c.?
r.(?)
p.?
-
benign
g.?
-
227G>T
-
MYH2_000008
-
Perrault 1996;
PubMed: Booij 2005
-
-
Germline
-
-
-
-
-
Julia Lopez
-/.
2
c.?
r.(?)
p.?
-
benign
g.7906613C>T
-
248C?T
-
MYH2_000008
-
PubMed: Li-2009
-
-
Germline
-
-
-
-
-
LOVD
+/.
-
c.?
r.?
p.?
-
pathogenic (dominant)
g.?
-
Arg838Cys
-
MYH2_000008
-
PubMed: Daiger 2014
-
-
Germline
-
-
-
-
-
LOVD
?/.
-
c.?
r.(?)
p.(?)
-
VUS
g.?
g.?
DelG hom 5UTR GUCY2D
-
MYH2_000008
homozygous
PubMed: Dharmaraj 2000
-
-
Germline
?
-
-
-
-
LOVD
?/.
-
c.?
r.(?)
p.(?)
-
VUS
g.?
g.?
DelG het 5UTR GUCY2D
-
MYH2_000008
single heterozygous
PubMed: Dharmaraj 2000
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
2
c.3G>A
r.(?)
p.(Met1?)
-
likely pathogenic
g.7906368G>A
g.8003050G>A
GUCY2D G3A, M11
-
GUCY2D_000308
obsolete annotation, actual nucleotide extrapolated from databases; heterozygous
PubMed: Perrault 2000
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
2
c.3G>C
r.(?)
p.(Met1?)
-
likely pathogenic
g.7906368G>C
g.8003050G>C
GUCY2D G3C, M1I
-
GUCY2D_000307
obsolete annotation, actual nucleotide extrapolated from databases; homozygous
PubMed: Perrault 2000
-
-
Unknown
?
-
-
-
-
LOVD
-?/.
-
c.49T>C
r.(?)
p.(Cys17Arg)
-
likely benign
g.7906414T>C
-
GUCY2D(NM_000180.3):c.49T>C (p.C17R)
-
GUCY2D_000116
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
2
c.52_99dup
r.(?)
p.(Gly18_Leu33dup)
-
likely pathogenic
g.7906417_7906464dup
g.8003099_8003146dup
GUCY2D 52-99Dup48bp
-
GUCY2D_000309
obsolete annotation, actual nucleotide extrapolated from databases; homozygous
PubMed: Perrault 2000
-
-
Unknown
?
-
-
-
-
LOVD
-/.
2
c.61T>C
r.(?)
p.(Trp21Arg)
-
benign
g.7906426T>C
g.8003108T>C
-
-
GUCY2D_000007
predicted benign
PubMed: Neveling 2012
-
-
Germline
no
-
-
-
-
Kornelia Neveling
-/.
-
c.61T>C
r.(?)
p.(Trp21Arg)
-
benign
g.7906426T>C
g.8003108T>C
GUCY2D(NM_000180.3):c.61T>C (p.W21R)
-
GUCY2D_000007
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
-
c.74C>T
r.(?)
p.(Ser25Phe)
-
VUS
g.7906439C>T
g.8003121C>T
GUCY2D(NM_000180.3):c.74C>T (p.S25F), GUCY2D(NM_000180.4):c.74C>T (p.S25F)
-
GUCY2D_000025
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
-
c.74C>T
r.(?)
p.(Ser25Phe)
-
likely benign
g.7906439C>T
g.8003121C>T
GUCY2D(NM_000180.3):c.74C>T (p.S25F), GUCY2D(NM_000180.4):c.74C>T (p.S25F)
-
GUCY2D_000025
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
-
c.74C>T
r.(?)
p.(Ser25Phe)
-
VUS
g.7906439C>T
-
-
-
GUCY2D_000025
-
PubMed: Wang 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
-
c.74C>T
r.(?)
p.(Ser25Phe)
ACMG
VUS
g.7906439C>T
g.8003121C>T
GUCY2D c.2513G>A, p.(Arg838His), c.74C>T, p.(Ser25Phe)
-
GUCY2D_000025
-
PubMed: Jespersgaar 2019
-
-
Germline
?
-
-
-
-
LOVD
?/.
-
c.74C>T
r.(?)
p.(Ser25Phe)
ACMG
VUS
g.7906439C>T
g.8003121C>T
GUCY2D c.2513G>A, p.(Arg838His), c.74C>T, p.(Ser25Phe)
-
GUCY2D_000025
-
PubMed: Jespersgaar 2019
-
-
Germline
?
-
-
-
-
LOVD
?/.
-
c.74C>T
r.(?)
p.(Ser25Phe)
-
VUS
g.7906439C>T
-
GUCY2D(NM_000180.3):c.74C>T (p.S25F), GUCY2D(NM_000180.4):c.74C>T (p.S25F)
-
GUCY2D_000025
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
-
c.74C>T
r.(?)
p.(Ser25Phe)
-
benign
g.7906439C>T
-
GUCY2D(NM_000180.3):c.74C>T (p.S25F), GUCY2D(NM_000180.4):c.74C>T (p.S25F)
-
GUCY2D_000025
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
-
c.82dup
r.(?)
p.(Arg28ProfsTer291)
-
pathogenic
g.7906447dup
g.8003129dup
82_83insC
-
GUCY2D_000175
-
PubMed: Wang 2015
-
-
Germline
-
-
-
-
-
LOVD
+?/.
-
c.91dup
r.(?)
p.(Arg31Profs*288)
-
likely pathogenic (recessive)
g.7906456dup
g.8003138dup
-
-
GUCY2D_000140
-
PubMed: Thompson 2017
-
-
Germline
-
-
-
-
-
LOVD
-/.
-
c.111G>A
r.(?)
p.(Pro37=)
-
benign
g.7906476G>A
g.8003158G>A
GUCY2D(NM_000180.4):c.111G>A (p.P37=)
-
GUCY2D_000077
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
-
c.121C>T
r.(?)
p.(Leu41Phe)
-
likely benign
g.7906486C>T
g.8003168C>T
GUCY2D(NM_000180.4):c.121C>T (p.L41F)
-
GUCY2D_000026
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
2
c.121C>T
r.(?)
p.(Leu41Phe)
-
likely benign
g.7906486C>T
-
c.121C>T (p.Leu41Phe)
-
GUCY2D_000026
-
PubMed: Vallespin 2007
-
-
Germline
-
-
-
-
-
LOVD
+?/.
2
c.121C>T
r.(?)
p.(Leu41Phe)
-
likely pathogenic
g.7906486C>T
g.8003168C>T
GUCY2D C121T, L41F
-
GUCY2D_000026
obsolete annotation, actual nucleotide extrapolated from databases; single heterozygous variant, no second allele found
PubMed: Perrault 2000
-
-
Unknown
?
-
-
-
-
LOVD
?/.
-
c.129_134del
r.(?)
p.(Leu44_Leu45del)
-
VUS
g.7906494_7906499del
g.8003176_8003181del
GUCY2D(NM_000180.3):c.129_134delTCTGCT (p.(Leu44_Leu45del)), GUCY2D(NM_000180.3):c.129_134delTCTGCT (p.L44_L45del), GUCY2D(NM_000180.4):c.129_134de...
-
GUCY2D_000027
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
-
c.129_134del
r.(?)
p.(Leu44_Leu45del)
-
VUS
g.7906494_7906499del
g.8003176_8003181del
GUCY2D(NM_000180.3):c.129_134delTCTGCT (p.(Leu44_Leu45del)), GUCY2D(NM_000180.3):c.129_134delTCTGCT (p.L44_L45del), GUCY2D(NM_000180.4):c.129_134de...
-
GUCY2D_000027
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
-
c.129_134del
r.(?)
p.(Leu44_Leu45del)
-
VUS
g.7906494_7906499del
g.8003176_8003181del
-
-
GUCY2D_000027
no genotypes reported
PubMed: Sergouniotis 2016
-
rs552184470
Germline
-
2/486 individuals
-
-
-
LOVD
+/.
-
c.129_134del
r.(?)
p.(Leu44_Leu45del)
-
pathogenic
g.7906494_7906499del
g.8003176_8003181del
c.124_129del, p.(Leu44_Leu45del)
-
GUCY2D_000027
heterozygous
PubMed: Wang 2019
-
-
Germline
?
-
-
-
-
LOVD
+?/.
2
c.129_134del
r.(?)
p.(Leu44_Leu45del)
-
likely pathogenic
g.7906494_7906499del
-
c.129_134del
-
GUCY2D_000027
-
PubMed: Maggi_2021
-
-
Germline
-
-
-
-
-
LOVD
+?/.
-
c.129_134del
r.(?)
p.(Leu44_Leu45del)
-
likely pathogenic
g.7906494_7906499del
g.8003176_8003181del
GUCY2D Leu42del6bp
-
GUCY2D_000027
no nucleotide written, extrapolated from protein and databases; most probably deleted nucleotide was meant to be c.129_134del - 3' rule shifts it from Leu 42 to Leu44; heterozygous
PubMed: Jacobson 2012
-
-
Germline
yes
-
-
-
-
LOVD
?/.
-
c.129_134del
r.(?)
p.(Leu44_Leu45del)
-
VUS
g.7906494_7906499del
-
GUCY2D(NM_000180.3):c.129_134delTCTGCT (p.(Leu44_Leu45del)), GUCY2D(NM_000180.3):c.129_134delTCTGCT (p.L44_L45del), GUCY2D(NM_000180.4):c.129_134de...
-
GUCY2D_000027
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
2
c.134T>C
r.(?)
p.(Leu45Pro)
-
benign
g.7906499T>C
-
134T>C
-
GUCY2D_000152
-
PubMed: Loyer 1998
;
PubMed: Booij 2005
-
-
Germline
-
-
-
-
-
Julia Lopez
+?/.
-
c.139delC
r.(?)
p.(Ala49Profs*36)
-
likely pathogenic
g.7906509del
g.8003191del
GUCY2D c.139delC (p.Ala49Profs*36)
-
GUCY2D_000275
heterozygous
PubMed: Feng 2020
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
-
c.139delC
r.(?)
p.(Ala49Profs*36)
-
likely pathogenic
g.7906509del
g.8003191del
GUCY2D c.139delC (p.Ala49Profs*36)
-
GUCY2D_000275
heterozygous
PubMed: Feng 2020
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
-
c.139_140delinsA
r.(?)
p.(Pro47Thrfs*38)
-
likely pathogenic (recessive)
g.7906504_7906505delinsA
g.8003186_8003187delinsA
-
-
GUCY2D_000356
ACMG PVS1, PM2
PubMed: Rashid 2020
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
-?/.
-
c.142C>T
r.(?)
p.(Pro48Ser)
-
likely benign
g.7906507C>T
g.8003189C>T
GUCY2D(NM_000180.3):c.142C>T (p.P48S)
-
GUCY2D_000112
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
2
c.154G>T
r.(?)
p.(Ala52Ser)
-
likely benign
g.7906519G>T
g.8003201G>T
-
-
GUCY2D_000001
predicted unknown effect on function, present at significant fraction in Exome Variant Server
PubMed: Neveling 2012
-
-
Germline
-
-
-
-
-
Kornelia Neveling
-?/.
2
c.154G>T
r.(?)
p.(Ala52Ser)
-
likely benign
g.7906519G>T
g.8003201G>T
-
-
GUCY2D_000001
predicted unknown effect on function, present at significant fraction in Exome Variant Server
PubMed: Neveling 2012
-
-
Germline
-
-
-
-
-
Kornelia Neveling
?/.
2
c.154G>T
r.(?)
p.(Ala52Ser)
-
VUS
g.7906519G>T
g.8003201G>T
-
-
GUCY2D_000001
predicted unknown effect on function, present at significant fraction in Exome Variant Server
PubMed: Neveling 2012
-
-
Germline
yes
-
-
-
-
Kornelia Neveling
?/.
2
c.154G>T
r.(?)
p.(Ala52Ser)
-
VUS
g.7906519G>T
g.8003201G>T
-
-
GUCY2D_000001
predicted unknown effect on function, present at significant fraction in Exome Variant Server
PubMed: Neveling 2012
-
-
Germline
yes
-
-
-
-
Kornelia Neveling
?/.
2
c.154G>T
r.(?)
p.(Ala52Ser)
-
VUS
g.7906519G>T
g.8003201G>T
-
-
GUCY2D_000001
disease-related variants in other gene; not segregating with disease in other family
PubMed: Neveling 2012
-
-
Germline
-
-
-
-
-
Kornelia Neveling
?/.
2
c.154G>T
r.(?)
p.(Ala52Ser)
-
VUS
g.7906519G>T
g.8003201G>T
-
-
GUCY2D_000001
-
PubMed: Neveling 2012
-
-
Germline
no
-
-
-
-
Kornelia Neveling
?/.
2
c.154G>T
r.(?)
p.(Ala52Ser)
-
VUS
g.7906519G>T
g.8003201G>T
-
-
GUCY2D_000001
not segregating with disease in other family
PubMed: Neveling 2012
-
-
Germline
-
-
-
-
-
Kornelia Neveling
?/.
2
c.154G>T
r.(?)
p.(Ala52Ser)
-
VUS
g.7906519G>T
g.8003201G>T
-
-
GUCY2D_000001
not segregating with disease in other family
PubMed: Neveling 2012
-
-
Germline
-
-
-
-
-
Kornelia Neveling
-/.
-
c.154G>T
r.(?)
p.(Ala52Ser)
-
benign
g.7906519G>T
g.8003201G>T
GUCY2D(NM_000180.3):c.154G>T (p.A52S), GUCY2D(NM_000180.4):c.154G>T (p.A52S)
-
GUCY2D_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
-
c.154G>T
r.(?)
p.(Ala52Ser)
-
benign
g.7906519G>T
g.8003201G>T
GUCY2D(NM_000180.3):c.154G>T (p.A52S), GUCY2D(NM_000180.4):c.154G>T (p.A52S)
-
GUCY2D_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
-
c.154G>T
r.(?)
p.(Ala52Ser)
-
benign
g.7906519G>T
g.8003201G>T
GUCY2D(NM_000180.3):c.154G>T (p.A52S), GUCY2D(NM_000180.4):c.154G>T (p.A52S)
-
GUCY2D_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
-
c.154G>T
r.(?)
p.(Ala52Ser)
-
benign
g.7906519G>T
g.8003201G>T
GUCY2D(NM_000180.3):c.154G>T (p.A52S), GUCY2D(NM_000180.4):c.154G>T (p.A52S)
-
GUCY2D_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
c.159del
r.(?)
p.(Phe54Serfs*31)
-
likely pathogenic
g.7906524del
g.8003206del
-
-
GUCY2D_000018
-
-
-
-
Germline
yes
-
-
-
-
Soumittra Nagasamy
+?/.
-
c.160T>A
r.(?)
p.(Phe54Ile)
-
likely pathogenic
g.7906525T>A
g.8003207T>A
-
-
GUCY2D_000153
-
PubMed: Bravo-Gil 2017
-
-
Germline
-
-
-
-
-
Nereida Bravo Gil
?/.
2
c.162C>G
r.(?)
p.(Phe54Leu)
-
VUS
g.7906527C>G
-
c.162C>G:p.F54L
-
GUCY2D_000272
-
PubMed: Numa-2020
-
-
Unknown
-
-
-
-
-
LOVD
-?/.
-
c.164C>T
r.(?)
p.(Thr55Met)
-
likely benign
g.7906529C>T
g.8003211C>T
GUCY2D(NM_000180.3):c.164C>T (p.T55M), GUCY2D(NM_000180.4):c.164C>T (p.T55M)
-
GUCY2D_000028
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
-
c.164C>T
r.(?)
p.(Thr55Met)
-
benign
g.7906529C>T
g.8003211C>T
GUCY2D(NM_000180.3):c.164C>T (p.T55M), GUCY2D(NM_000180.4):c.164C>T (p.T55M)
-
GUCY2D_000028
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
-
c.164C>T
r.(?)
p.(Thr55Met)
-
VUS
g.7906529C>T
g.8003211C>T
-
-
GUCY2D_000028
4 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs201414567
Germline
-
4/2795 individuals
-
-
-
Mohammed Faruq
-?/.
-
c.164C>T
r.(?)
p.(Thr55Met)
-
likely benign
g.7906529C>T
g.8003211C>T
-
-
GUCY2D_000028
classification based on frequency in 305 unrelated individuals
PubMed: Le 2019
-
-
Germline
-
frequency 0.015
-
-
-
Global Variome, with Curator vacancy
+/.
2
c.164C>T
r.(?)
p.(Thr55Met)
-
pathogenic
g.7906529C>T
-
164C>T
-
GUCY2D_000028
-
PubMed: li 2011
-
-
Germline
-
4/87 cases; 0/96 controls
-
-
-
LOVD
+/.
2
c.164C>T
r.(?)
p.(Thr55Met)
-
pathogenic
g.7906529C>T
-
164C>T
-
GUCY2D_000028
-
PubMed: li 2011
-
-
Germline
-
4/87 cases; 0/96 controls
-
-
-
LOVD
+/.
2
c.164C>T
r.(?)
p.(Thr55Met)
-
pathogenic
g.7906529C>T
-
164C>T
-
GUCY2D_000028
-
PubMed: li 2011
-
-
Germline
-
4/87 cases; 0/96 controls
-
-
-
LOVD
-/.
2
c.164C>T
r.(?)
p.(Thr55Met)
-
benign
g.7906529C>T
-
164C>T
-
GUCY2D_000028
-
PubMed: li 2011
-
-
Germline
-
4/87 cases; 0/96 controls
-
-
-
LOVD
-/.
2
c.164C>T
r.(?)
p.(Thr55Met)
-
benign
g.7906529C>T
-
164C>T
-
GUCY2D_000028
-
PubMed: li 2011
-
-
Germline
-
4/87 cases; 0/96 controls
-
-
-
LOVD
-?/.
2
c.164C>T
r.(?)
p.(Thr55Met)
-
likely benign
g.7906529C>T
-
c.164C>T
-
GUCY2D_000028
-
PubMed: Chen-2013
-
-
Unknown
-
-
-
-
-
LOVD
-?/.
2
c.164C>T
r.(?)
p.(Thr55Met)
-
likely benign
g.7906529C>T
-
c.164C>T
-
GUCY2D_000028
-
PubMed: Chen-2013
-
-
Unknown
-
-
-
-
-
LOVD
-?/.
2
c.164C>T
r.(?)
p.(Thr55Met)
-
likely benign
g.7906529C>T
-
c.164C>T
-
GUCY2D_000028
-
PubMed: Chen-2013
-
-
Unknown
-
-
-
-
-
LOVD
+?/.
2
c.164C>T
r.(?)
p.(Thr55Met)
-
likely pathogenic (recessive)
g.7906529C>T
-
c.164C>T
-
GUCY2D_000028
-
PubMed: Liu-2020
-
-
Germline
-
-
-
-
-
LOVD
+?/.
-
c.167_168del
r.(?)
p.(Val56Glyfs*262)
-
likely pathogenic
g.7906532_7906533del
g.8003214_8003215del
GUCY2D c.167_168delTG, p.Val56GlyfsTer262; c.738G>C, p.Met246Ile
-
GUCY2D_000276
heterozygous
PubMed: Liu 2020
-
-
Unknown
?
-
-
-
-
LOVD
+/.
-
c.174delC
r.(?)
p.(Leu59Trpfs*26)
ACMG
pathogenic
g.7906540del
g.8003222del
GUCY2D NM_000180: g.628delC, c.174delC, p.L59Wfs*26
-
GUCY2D_000246
-
PubMed: Xu 2020
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
-
c.186G>A
r.(?)
p.(Trp62Ter)
-
likely pathogenic
g.7906551G>A
g.8003233G>A
GUCY2D p.W62X (c.186G>A)
-
GUCY2D_000286
heterozygous
PubMed: Preising 2007
-
-
De novo
?
-
-
-
-
LOVD
+?/.
2
c.226_239del
r.(?)
p.(Ala76Argfs*238)
-
likely pathogenic
g.7906591_7906604del
g.8003273_8003286del
GUCY2D 226-239Del14bp
-
GUCY2D_000310
obsolete annotation, actual nucleotide extrapolated from databases; homozygous
PubMed: Perrault 2000
-
-
Unknown
?
-
-
-
-
LOVD
+/.
-
c.238_252del
r.(?)
p.(Ala80_Leu84del)
-
pathogenic (recessive)
g.7906603_7906617del
g.8003285_8003299del
17:7906590GGCCGCCCGCCTGGCC>G ENST00000254854.4:c.238_252delGCCGCCGCCCGCCTG (Ala80_Leu84del)
-
GUCY2D_000129
-
PubMed: Carss 2017
,
PubMed: Turro 2020
-
-
Germline
-
-
-
-
-
LOVD
+?/.
-
c.238_252del
r.(?)
p.(Ala80_Leu84del)
-
likely pathogenic
g.7906603_7906617del
g.8003285_8003299del
GUCY2D c.238_252del, p.Ala80_Leu84del; c.2620G>A, p.Glu874Lys
-
GUCY2D_000129
heterozygous
PubMed: Liu 2020
-
-
Germline
yes
-
-
-
-
LOVD
+/.
2
c.244_250dup
r.(?)
p.(Leu84Argfs*237)
ACMG
pathogenic
g.7906609_7906615dup
g.8003291_8003297dup
NM_000180.3:c.244_250dup, NP_000171.1:p.(Leu84ArgfsTer237), NC_000017.10:g.7906609_7906615dup
-
GUCY2D_000214
-
PubMed: Wang 2018
-
-
Germline
?
-
-
-
-
LOVD
+?/.
2
c.244_250dup
r.(?)
p.(Leu84Argfs*237)
-
likely pathogenic (recessive)
g.7906609_7906615dup
-
c.244_250dupGCCCGCC
-
GUCY2D_000214
-
PubMed: Liu-2020
-
-
Germline
-
-
-
-
-
LOVD
-?/.
-
c.271G>C
r.(?)
p.(Ala91Pro)
-
likely benign
g.7906636G>C
g.8003318G>C
GUCY2D(NM_000180.3):c.271G>C (p.A91P)
-
GUCY2D_000078
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
-
c.307G>A
r.(?)
p.(Glu103Lys)
-
pathogenic (recessive)
g.7906672G>A
g.8003354G>A
17:7906672G>A ENST00000254854.4:c.307G>A (Glu103Lys)
-
GUCY2D_000130
-
PubMed: Carss 2017
,
PubMed: Turro 2020
-
-
Germline
-
-
-
-
-
LOVD
+?/.
-
c.307G>A
r.(?)
p.(Glu103Lys)
-
likely pathogenic (recessive)
g.7906672G>A
g.8003354G>A
-
-
GUCY2D_000130
-
PubMed: Thompson 2017
-
-
Germline
-
-
-
-
-
LOVD
+?/.
-
c.307G>A
r.(?)
p.(Glu103Lys)
-
likely pathogenic (recessive)
g.7906672G>A
g.8003354G>A
-
-
GUCY2D_000130
-
PubMed: Thompson 2017
-
-
Germline
-
-
-
-
-
LOVD
+/.
2
c.308A>T
r.(?)
p.(Glu103Val)
-
pathogenic
g.7906673A>T
-
308A>T
-
GUCY2D_000204
-
PubMed: li 2011
-
-
Germline
-
2/87 cases; 0/96 controls
-
-
-
LOVD
+/.
2
c.308A>T
r.(?)
p.(Glu103Val)
-
pathogenic
g.7906673A>T
-
308A>T
-
GUCY2D_000204
-
PubMed: li 2011
-
-
Germline
-
2/87 cases; 0/96 controls
-
-
-
LOVD
+?/.
2
c.312_313dup
r.(?)
p.(Cys105Phefs*25)
-
likely pathogenic (recessive)
g.7906677_7906678dup
g.8003359_8003360dup
c.312_313dupTT, p.Cys105Phefs*25
-
GUCY2D_000255
Heterozygous
PubMed: Cho 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
-
c.314G>A
r.(?)
p.(Cys105Tyr)
-
likely pathogenic
g.7906679G>A
g.8003361G>A
GUCY2D C105Y
-
GUCY2D_000287
protein: reduced RetGC-1 acivity by 50%; no nucleotide annotation, extrapolated from protein; heterozygous
PubMed: Tucker 2004
reduced RetGC-1 acivity by only 50%
-
In vitro (cloned)
yes
-
-
-
-
LOVD
+?/.
-
c.314G>A
r.(?)
p.(Cys105Tyr)
-
likely pathogenic
g.7906679G>A
g.8003361G>A
C105T Comp het GUCY2D
-
GUCY2D_000287
error in table - threonine; correct tyrosine in text; compound heterozygous
PubMed: Dharmaraj 2000
-
-
Germline
?
-
-
-
-
LOVD
-/.
-
c.369C>T
r.(?)
p.(Gly123=)
-
benign
g.7906734C>T
g.8003416C>T
GUCY2D(NM_000180.3):c.369C>T (p.G123=), GUCY2D(NM_000180.4):c.369C>T (p.G123=)
-
GUCY2D_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
-
c.369C>T
r.(?)
p.(Gly123=)
-
likely benign
g.7906734C>T
g.8003416C>T
GUCY2D(NM_000180.3):c.369C>T (p.G123=), GUCY2D(NM_000180.4):c.369C>T (p.G123=)
-
GUCY2D_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
-
c.369C>T
r.(?)
p.(Gly123=)
-
benign
g.7906734C>T
-
GUCY2D(NM_000180.3):c.369C>T (p.G123=), GUCY2D(NM_000180.4):c.369C>T (p.G123=)
-
GUCY2D_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
4
c.370A>T
r.(?)
p.(Arg124Trp)
-
pathogenic
g.43013853A>T
g.44936485A>T
-
-
GUCY2D_000067
-
Sharon, submitted
-
-
Germline
-
-
-
-
-
Dror Sharon
?/.
-
c.380C>G
r.(?)
p.(Pro127Arg)
ACMG
VUS
g.7906745C>G
g.8003427C>G
-
-
GUCY2D_000337
ACMG PM2, PM5, PP2
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
c.380C>T
r.(?)
p.(Pro127Leu)
-
pathogenic (dominant)
g.7906745C>T
g.8003427C>T
-
-
GUCY2D_000135
-
PubMed: Birtel 2018
-
-
Germline
-
-
-
-
-
LOVD
?/.
-
c.380C>T
r.(?)
p.(Pro127Leu)
-
VUS
g.7906745C>T
g.8003427C>T
-
-
GUCY2D_000135
-
PubMed: Ellingford 2016
-
-
Germline
-
-
-
-
-
LOVD
+?/.
2
c.387C>A
r.(?)
p.(Asn129Lys)
-
likely pathogenic
g.7906752C>A
g.8003434C>A
GUCY2D C387A, N129K
-
GUCY2D_000311
obsolete annotation, actual nucleotide extrapolated from databases; heterozygous
PubMed: Perrault 2000
-
-
Unknown
?
-
-
-
-
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