Global Variome shared LOVD
GUCA1A (guanylate cyclase activator 1A (retina))
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Curator:
Global Variome, with Curator vacancy
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Unique variants in the GUCA1A gene
This database is one of the
"Eye disease"
gene variant databases.
The variants shown are described using the NM_000409.3 transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Reported
: The number of times this variant has been reported in the database.
Exon
: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA)
: description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change
: description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein
: description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Classification method
: The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification
: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19)
: HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38)
: HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as
: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN
: description of the variant according to ISCN nomenclature
DB-ID
: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks
: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference
: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID
: ID of variant in ClinVar database
dbSNP ID
: the dbSNP ID
Origin
: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation
: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency
: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site
: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP
: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator. NOTE: to get VIP status ask the curator.
Methylation
: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
How to query this table
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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.
67 entries on 1 page. Showing entries 1 - 67.
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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
-
c.-84322_*128532dup
r.0?
p.0?
ACMG
likely pathogenic
g.42039467_42275673dup
-
-
-
C6orf132_000007
ACMG PM2, PVS1
PubMed: Weisschuh 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.-458+2T>C
r.spl?
p.?
-
likely benign
g.42123333T>C
g.42155595T>C
GUCA1A(NM_001319061.1):c.-829+2T>C
-
GUCA1A_000012
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
2
-
c.-457-8C>T
r.(=)
p.(=)
-
likely benign
g.42130650C>T
g.42162912C>T
-
-
GUCA1A_000018
1 homozygous;
Clinindb (India)
, 58 heterozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs58894089
Germline
-
1/2795 individuals, 58/2795 individuals
-
-
-
Mohammed Faruq
-/.
1
-
c.9C>T
r.(?)
p.(Asn3=)
-
benign
g.42141360C>T
g.42173622C>T
LOC118142757(NM_000409.5):c.9C>T (p.N3=)
-
GUCA1A_000013
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.42C>T
r.(?)
p.(Ser14=)
-
likely benign
g.42141393C>T
g.42173655C>T
GUCA1A(NM_001319061.1):c.42C>T (p.S14=)
-
GUCA1A_000014
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.50_80del
r.(?)
p.(Glu17Valfs*22)
ACMG
VUS
g.42141401_42141431del
g.42173663_42173693del
GUCA1A c.C50_80del p.E17VfsX22
-
GUCA1A_000050
number of normal cases with GUCA1A variant: 3; heterozygous
PubMed: Mizobuchi 2019
-
-
Unknown
no
-
-
-
-
LOVD
+?/.
1
-
c.55C>T
r.(?)
(p.H19Y)
-
likely pathogenic
g.42141406C>T
g.42173668C>T
GUCA1A c.55C > T (p.H19Y)
-
GUCA1A_000060
heterozygous
PubMed: Abbas 2020
-
-
Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.64T>C
r.(?)
p.(Tyr22His)
-
VUS
g.42141415T>C
g.42173677T>C
GUCA1A(NM_001319061.1):c.64T>C (p.Y22H)
-
GUCA1A_000015
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
2
-
c.124T>A
r.(?)
p.(Phe42Ile)
ACMG
VUS
g.42141475T>A
g.42173737T>A
GUCA1A c.124T>A p.F42I, GUCA1A(NM_001319061.1):c.124T>A (p.F42I)
-
GUCA1A_000016
number of normal cases with GUCA1A variant: 2; heterozygous, VKGL data sharing initiative Nederland
PubMed: Mizobuchi 2019
-
-
CLASSIFICATION record, Unknown
no
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/., -?/., ?/.
12
3
c.149C>T
r.(?)
p.(Pro50Leu)
ACMG
likely benign, likely pathogenic, likely pathogenic (dominant), pathogenic, VUS
g.42141500C>T
g.42173762C>T
GUCA1A c.149C>T, p.(Pro50Leu), GUCA1A P50L,
2 more items
-
GUCA1A_000001
VKGL data sharing initiative Nederland,
2 more items
PubMed: Boulanger-Scemama 2015
,
PubMed: Boulanger-Scemama 2019
,
PubMed: Downes 2001
,
PubMed: Wang 2017
,
3 more items
-
rs104893968
CLASSIFICATION record, Germline
?, no, yes
-
-
-
-
Global Variome, with Curator vacancy
,
Kornelia Neveling
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
1
-
c.152C>T
r.(?)
p.(Ser51Leu)
-
VUS
g.42141503C>T
-
GUCA1A(NM_001319061.1):c.152C>T (p.S51L)
-
GUCA1A_000042
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.166G>T
r.(?)
p.(Val56Leu)
-
likely benign
g.42141517G>T
g.42173779G>T
LOC118142757(NM_000409.5):c.166G>T (p.V56L)
-
GUCA1A_000003
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.193T>G
r.(?)
p.(Phe65Val)
-
likely pathogenic
g.42141544T>G
g.42173806T>G
-
-
GUCA1A_000024
-
PubMed: Stone 2017
-
-
Germline
-
-
-
-
-
LOVD
-?/.
1
-
c.201+11G>T
r.(=)
p.(=)
-
likely benign
g.42141563G>T
-
LOC118142757(NM_001319061.2):c.201+11G>T
-
GUCA1A_000058
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.202-16C>T
r.(=)
p.(=)
-
likely benign
g.42146002C>T
g.42178264C>T
GUCA1A(NM_001384910.1):c.202-16C>T, LOC118142757(NM_000409.5):c.202-16C>T
-
GUCA1A_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.204C>G
r.(?)
p.(Asp68Glu)
ACMG
VUS
g.42146020C>G
g.42178282C>G
GUCA1A c.204C>G p.D68E
-
GUCA1A_000051
number of normal cases with GUCA1A variant: 1; heterozygous
PubMed: Mizobuchi 2019
-
-
Unknown
no
-
-
-
-
LOVD
?/.
1
-
c.212T>C
r.(?)
p.(Ile71Thr)
-
VUS
g.42146028T>C
g.42178290T>C
-
-
GUCA1A_000010
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
-
c.216T>C
r.(?)
p.(Asp72=)
-
benign
g.42146032T>C
g.42178294T>C
LOC118142757(NM_000409.5):c.216T>C (p.D72=)
-
GUCA1A_000005
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.238C>A
r.(?)
p.(Leu80Ile)
ACMG
VUS
g.42146054C>A
g.42178316C>A
GUCA1A c.238C>A p.L80I
-
GUCA1A_000052
number of normal cases with GUCA1A variant: 2; heterozygous
PubMed: Mizobuchi 2019
-
-
Unknown
no
-
-
-
-
LOVD
+?/.
9
4
c.250C>T
r.(?)
p.(Leu84Phe)
ACMG
likely pathogenic, likely pathogenic (dominant)
g.42146066C>T
g.42178328C>T
GUCA1A c.250C>T, p.L84F
-
GUCA1A_000043
ACMG PM2, PP2, PP5_STRONG, heterozygous
PubMed: Hitti-Malin 2022
,
Journal: Hitti-Malin 2022
,
PubMed: Kamenarova_2013
,
PubMed: Weisschuh 2024
,
1 more item
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
,
Rebekkah Hitti-Malin
+/.
3
-
c.256G>C
r.(?)
p.(Gly86Arg)
-
pathogenic (dominant)
g.42146072G>C
g.42178334G>C
GUCA1A c.256G -> C, G86R
-
GUCA1A_000053
1 more item
PubMed: Peshenko 2019
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
3
-
c.265G>A
r.(?)
p.(Glu89Lys)
ACMG
likely pathogenic (dominant)
g.42146081G>A
g.42178343G>A
GUCA1A c.265G>A (p.Glu89Lys), GUCA1A c.265G>A, p.Glu89Lys
-
GUCA1A_000039
ACMG PM2, PM1, PP2, PP5, heterozygous
PubMed: Kitiratschky 2009
,
PubMed: Kohl 2012
,
PubMed: Weisschuh 2024
986883
-
Germline, Germline/De novo (untested), Unknown
?, yes
-
-
-
-
Johan den Dunnen
+/.
4
-
c.295T>A
r.(?)
p.(Tyr99Asn)
ACMG
pathogenic (dominant)
g.42146111T>A
g.42178373T>A
GUCA1A c.295 T>A (p.Y99N)
-
GUCA1A_000054
heterozygous
PubMed: Mizobuchi 2019
-
-
Germline
yes
-
-
-
-
LOVD
+/.
2
-
c.296A>C
r.(?)
p.(Tyr99Ser)
ACMG
pathogenic (dominant)
g.42146112A>C
g.42178374A>C
GUCA1A c.296 A> C (p.Y99S)
-
GUCA1A_000055
heterozygous
PubMed: Mizobuchi 2019
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/.
32
2, 4
c.296A>G
r.(?)
p.(Tyr99Cys)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic, pathogenic (dominant)
g.42146112A>G
g.42178374A>G
6:42146112A>G ENST00000394237.1:c.296A>G (Tyr99Cys), c.296A>G, GUCA1A c.296A>G, p.Tyr99Cys,
1 more item
-
GUCA1A_000019
carries likely causative variants in more than one gene, heterozygous,
2 more items
PubMed: Bryant 2018
,
PubMed: Carss 2017
,
PubMed: Downes 2001
,
PubMed: Liu-2020
,
PubMed: Payne 1998
,
5 more items
-
rs104893967
De novo, Germline, Germline/De novo (untested)
?, yes
-
-
-
-
Rebekkah Hitti-Malin
+?/.
3
-
c.299A>G
r.(?)
p.(Asp100Gly)
-
likely pathogenic (dominant)
g.42146115A>G
g.42178377A>G
GUCA1A c.299 A>G p.Asp100Gly (D100G)
-
GUCA1A_000044
heterozygous
PubMed: Nong 2014
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
2
-
c.300T>A
r.(?)
p.(Asp100Glu)
-
likely pathogenic (dominant)
g.42146116T>A
g.42178378T>A
GUCA1A c.300T>A (p.Asp100Glu), GUCA1A c.300T>A, Asp100Gln
-
GUCA1A_000040
error in annotation, should be p.(Asp100Glu); heterozygous, heterozygous
PubMed: Kitiratschky 2009
,
PubMed: Kohl 2012
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
3
-
c.302_304delTAG
r.(?)
p.(Val101del)
-
likely pathogenic (dominant)
g.42146118_42146120del
g.42178380_42178382del
GUCA1A c.302_304delTAG (p.Val101del)
-
GUCA1A_000045
heterozygous
PubMed: Manes 2019
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.304G>C
r.(?)
p.(Asp102His)
-
likely pathogenic (dominant)
g.42146120G>C
g.42178382G>C
-
-
GUCA1A_000028
-
PubMed: Huang 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.306T>A
r.(?)
p.(Asp102Glu)
-
likely pathogenic
g.42146122T>A
g.42178384T>A
-
-
GUCA1A_000025
-
PubMed: Stone 2017
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
4
4
c.312C>A
r.(?)
p.(Asn104Lys)
-
likely pathogenic (dominant), pathogenic (recessive)
g.42146128C>A
g.42178390C>A
819_826del8, GUCA1A C312A, N104K
-
GUCA1A_000022
obsolete nucleotide annotation, extrapolated from protein and databases; heterozygous
PubMed: Jiang 2008
,
PubMed: Wawrocka 2018
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.320T>C
r.(?)
p.(Ile107Thr)
-
likely pathogenic (dominant)
g.42146136T>C
g.42178398T>C
GUCA1A c.320T>C, p.I107T
-
GUCA1A_000046
heterozygous
PubMed: Kamenarova_2013
-
-
Germline
yes
-
-
-
-
LOVD
+/.
2
4
c.328_337del
r.(?)
p.(Asp110Serfs*18)
ACMG
pathogenic, pathogenic (!)
g.42146144_42146153del
g.42178406_42178415del
328_337delGATGAGCTGC
-
GUCA1A_000011
ACMG PVS1, PM2, PP4, dominant, reduced risk
PubMed: Zenteno 2020
-
-
Germline
-
1/143 cases
-
-
-
Johan den Dunnen
,
Juan Carlos Zenteno
+/.
1
-
c.331G>A
r.(?)
p.(Glu111Lys)
-
pathogenic
g.42146147G>A
g.42178409G>A
LOC118142757(NM_000409.5):c.331G>A (p.E111K)
-
GUCA1A_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/.
3
-
c.332A>C
r.(?)
p.(Glu111Ala)
-
likely pathogenic, pathogenic (dominant)
g.42146148A>C
g.42178410A>C
6:42146148A>C ENST00000394237.1:c.332A>C (Glu111Ala), GUCA1A c.332A>C, p.Glu111Ala
-
GUCA1A_000020
heterozygous
PubMed: Carss 2017
,
PubMed: Turro 2020
-
-
Germline, Germline/De novo (untested)
?
-
-
-
-
LOVD
+/.
3
-
c.332A>T
r.(?)
p.(Glu111Val)
-
pathogenic (dominant)
g.42146148A>T
g.42178410A>T
GUCA1A c.332A>T; p.(Glu111Val) - E111V
-
GUCA1A_000049
1 more item
PubMed: Marino 2018
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
4
c.333G>C
r.(?)
p.(Glu111Asp)
-
likely pathogenic
g.42146149G>C
-
c.333G>C
-
GUCA1A_000035
-
PubMed: Maggi_2021
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/.
2
4
c.341C>T
r.(?)
p.(Thr114Ile)
-
likely pathogenic (dominant), pathogenic
g.42146157C>T
g.42178419C>T
c.341C>T(p.T114I), GUCA1A c.341C>T, T114I
-
GUCA1A_000034
heterozygous
PubMed: Nishiguchi_2004
,
PubMed: Wang 2016
-
-
Germline, Unknown
?
-
-
-
-
LOVD
-?/.
1
-
c.351+11C>T
r.(=)
p.(=)
-
likely benign
g.42146178C>T
-
LOC118142757(NM_001319061.2):c.351+11C>T
-
GUCA1A_000031
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.359G>A
r.(?)
p.(Arg120His)
-
VUS
g.42146547G>A
g.42178809G>A
-
-
GUCA1A_000030
0/1266 control chromosomes
PubMed: Xu 2015
-
-
Germline
-
1/314 case chromosomes
-
-
-
LOVD
+/., +?/.
4
5
c.359G>T
r.(?)
p.(Arg120Leu)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic
g.42146547G>T
g.42178809G>T
GUCA1A, variant 1: c.359G>T/p.R120L
-
GUCA1A_000036
ACMG PM2, PM1_SUPPORTING, PP2, PP5_STRONG, possibly solved, heterozygous
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
,
PubMed: Weisschuh 2020
,
PubMed: Weisschuh 2024
802212
-
Germline, Unknown
?
-
-
-
-
Johan den Dunnen
,
Rebekkah Hitti-Malin
+?/.
18
-
c.359_360delinsTT
r.(?)
p.(Arg120Leu)
-
likely pathogenic (dominant)
g.42146547_42146548delinsTT
g.42178809_42178810delinsTT
GUCA1A c.359_360delinsTT, p.R120L
-
GUCA1A_000047
1 more item
PubMed: Chen 2017
-
-
Germline
yes
-
-
-
-
LOVD
?/.
1
-
c.371C>T
r.(?)
p.(Pro124Leu)
-
VUS
g.42146559C>T
-
GUCA1A(NM_001384910.1):c.371C>T (p.P124L)
-
GUCA1A_000064
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.424A>G
r.(?)
p.(Lys142Glu)
-
likely pathogenic
g.42146612A>G
g.42178874A>G
-
-
GUCA1A_000021
-
PubMed: Taylor 2017
-
-
Germline
-
-
-
-
-
LOVD
+?/.
10
-
c.428delinsACAC
r.(?)
p.(Ile143delinsAsnThr)
-
likely pathogenic, likely pathogenic (dominant)
g.42146616delinsACAC
g.42178878delinsACAC
428delTinsACAC, GUCA1A c.428delTinsACAC
-
GUCA1A_000026
no nucleotide annotation, extrapolated from protein and databases; heterozygous
PubMed: Song 2018
,
PubMed: Stone 2017
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
2
5
c.428delTinsACAC
r.(?)
p.(Ile143delinsAsnThr)
-
likely pathogenic (dominant)
g.42146616delinsACAC
g.42178878delinsACAC
GUCA1A c.428delTinsACAC, I143NT
-
GUCA1A_000026
heterozygous
PubMed: Nishiguchi_2004
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.428T>C
r.(?)
p.(Ile143Thr)
-
likely pathogenic
g.42146616T>C
g.42178878T>C
GUCA1A c.429_430insACA; c.428T>C
-
GUCA1A_000032
no protein change given, compound heterozygous
PubMed: Zanolli 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.429_430insACA
r.(?)
p.(Ile143_Asp144insThr)
-
likely pathogenic
g.42146617_42146618insACA
g.42178879_42178880insACA
GUCA1A c.429_430insACA; c.428T>C
-
GUCA1A_000033
no protein change given, compound heterozygous
PubMed: Zanolli 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
5
c.430G>A
r.(?)
p.(Asp144Asn)
ACMG
likely pathogenic
g.42146618G>A
g.42178880G>A
-
-
GUCA1A_000063
-
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
-
-
Germline
-
-
-
-
-
Rebekkah Hitti-Malin
?/.
1
-
c.431A>C
r.(?)
p.(Asp144Ala)
-
VUS
g.42146619A>C
-
LOC118142757(NM_001319061.2):c.431A>C (p.D144A)
-
GUCA1A_000059
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
7
5
c.431A>G
r.(?)
p.(Asp144Gly)
-
pathogenic (dominant)
g.42146619A>G
g.42178881A>G
GUCA1A c.431A>G, p.D144G
-
GUCA1A_000056
1 more item
PubMed: Tang 2019
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
2
-
c.443A>C
r.(?)
p.(Asp148Ala)
-
likely pathogenic
g.42146631A>C
g.42178893A>C
GUCA1A, variant 1: c.443A>C/p.D148A
-
GUCA1A_000037
possibly solved, heterozygous
PubMed: Weisschuh 2020
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
3
-
c.444T>A
r.(?)
p.(Asp148Glu)
-
likely pathogenic (dominant)
g.42146632T>A
g.42178894T>A
GUCA1A c.444T>A (p.Asp148Glu)
-
GUCA1A_000048
heterozygous
PubMed: Manes 2019
-
-
Germline
yes
-
-
-
-
LOVD
-/.
1
-
c.446-18C>A
r.(=)
p.(=)
-
benign
g.42146963C>A
g.42179225C>A
LOC118142757(NM_001319061.2):c.446-18C>A
-
GUCA1A_000006
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/.
43
5, 6
c.451C>T
r.(?)
p.(Leu151Phe)
ACMG
likely pathogenic, likely pathogenic (dominant), pathogenic (dominant)
g.42146986C>T
g.42179248C>T
GUCA1A C451T, l L151F, GUCA1A c.451 C> T (p.L151F), GUCA1A c.451C>T (p.Leu151Phe), GUCA1A L151F,
2 more items
-
GUCA1A_000023
ACMG PM2, PP2, PP5_STRONG, heteozygous, heterozygous,
1 more item
PubMed: Birtel 2018
,
PubMed: Gliem 2020
,
PubMed: Jiang 2005
,
PubMed: Kitiratschky 2009
,
6 more items
-
-
Germline, Unknown
?, yes
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.464A>C
r.(?)
p.(Glu155Ala)
-
likely pathogenic (dominant)
g.42146999A>C
g.42179261A>C
-
-
GUCA1A_000029
-
PubMed: Huang 2013
,
PubMed: Huang 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
29
6
c.464A>G
r.(?)
p.(Glu155Gly)
-
likely pathogenic, likely pathogenic (dominant)
g.42146999A>G
g.42179261A>G
GUCA1A A464G, E155G
-
GUCA1A_000027
heterozygous
PubMed: Stone 2017
,
PubMed: Wilkie_2001
-
-
Germline
yes
-
-
-
-
LOVD
?/.
1
-
c.465G>T
r.(?)
p.(Glu155Asp)
-
VUS
g.42147000G>T
-
-
-
GUCA1A_000062
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
6
c.475G>T
r.(?)
p.(Gly159Cys)
-
likely pathogenic (dominant)
g.42147010G>T
-
c.475G>T
-
GUCA1A_000038
-
PubMed: Liu-2020
-
-
Germline
-
-
-
-
-
LOVD
+?/.
3
-
c.476G>T
r.(?)
p.(Gly159Val)
-
likely pathogenic (dominant)
g.42147011G>T
g.42179273G>T
GUCA1A c.476G>T (p.Gly159Val), GUCA1A c.476G>T, p.Gly159Val
-
GUCA1A_000041
heterozygous
PubMed: Kitiratschky 2009
,
PubMed: Kohl 2012
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.479T>G
r.(?)
(p.V160G)
-
likely pathogenic
g.42147014T>G
g.42179276T>G
GUCA1A c.479T > G (p.V160G)
-
GUCA1A_000061
heterozygous
PubMed: Abbas 2020
-
-
Unknown
?
-
-
-
-
LOVD
+/., +?/.
20
6
c.526C>T
r.(?)
p.(Leu176Phe)
-
likely pathogenic, likely pathogenic (dominant), pathogenic (dominant)
g.42147061C>T
g.42179323C>T
GUCA1A c.526C> T, p.L176F, GUCA1A c.526C>T, p.Leu176Phe, GUCA1A, variant 1: c.526C>T/p.L176F,
1 more item
-
GUCA1A_000007
heterozygous, solved, heterozygous, VKGL data sharing initiative Nederland
PubMed: Birtel 2018
,
PubMed: Gliem 2020
,
PubMed: Vocke 2017
,
PubMed: Weisschuh 2016
,
1 more item
-
-
CLASSIFICATION record, Germline, Unknown
?, yes
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.551A>G
r.(?)
p.(Gln184Arg)
ACMG
VUS
g.42147086A>G
g.42179348A>G
GUCA1A c.551A>G p.Q184R
-
GUCA1A_000057
number of normal cases with GUCA1A variant: 2; heterozygous
PubMed: Mizobuchi 2019
-
-
Unknown
no
-
-
-
-
LOVD
-?/.
1
-
c.567C>T
r.(?)
p.(Asp189=)
-
likely benign
g.42147102C>T
g.42179364C>T
GUCA1A(NM_000409.4):c.567C>T (p.D189=)
-
GUCA1A_000008
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
6
c.598G>A
r.(?)
p.(Ala200Thr)
-
VUS
g.42147133G>A
g.42179395G>A
-
-
GUCA1A_000002
-
PubMed: Neveling 2012
-
-
Germline
no
-
-
-
-
Kornelia Neveling
-/.
1
-
c.*10C>A
r.(=)
p.(=)
-
benign
g.42147151C>A
g.42179413C>A
LOC118142757(NM_000409.5):c.*10C>A
-
GUCA1A_000009
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.*5445G>A
r.(=)
p.(=)
-
likely benign
g.42152586G>A
g.42184848G>A
GUCA1B(NM_002098.5):c.570C>T (p.L190=)
-
GUCA1B_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
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