Global Variome shared LOVD
CERKL (ceramide kinase-like)
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Curator:
David Baux
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Unique variants in the CERKL gene
This database is one of the
”Retinal and hearing impairment genetic variant databases”
.
The variants shown are described using the
NM_001030311.2
NM_201548.4
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'
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143 entries on 2 pages. Showing entries 1 - 100.
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Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
?/.
1
-
c.-71G>T
r.(?)
p.(=)
-
VUS
g.182521804C>A
g.181657077C>A
-
-
CERKL_000002
-
PubMed: Pfeffer 2012
-
-
Germline
no
-
-
-
-
Johan den Dunnen
+?/.
1
_1_2i
c.(?_-1)_(481+1_482-1){0}
r.0?
p.0?
-
likely pathogenic
g.(182438612_182468563)_(182521734_?)del
g.(181573885_181603836)_(181657007)del
del ex1-2
-
CERKL_000106
-
PubMed: Ellingford 2017
,
PubMed: Ellingsford 2018
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
_1_1i
c.-101_(238+1_239-1){0}
r.0?
p.0?
-
likely pathogenic
g.(182468807_182521495)_(182521738_?)del
-
chr2:182521491–182521738
-
CERKL_000107
-
PubMed: Ellingsford 2018
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/., ?/.
18
12
c.?
r.(?), r.0?, r.?, r.spl?
p.(?), p.(C362*), p.(R257*), p.0?, p.?
-
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.182379157_182765581del, g.182409466delT, g.?
-
1404delA, c.1404delA, chr2:g.182379157_182765581del, NM_201548.4:c.1031G>A (R344H), p.R257X/p.C362X,
10 more items
-
SNRNP200_000007
heterozygous
0,
PubMed: Birtel 2018
,
PubMed: Fu-2013
,
PubMed: Matsui 2015
,
PubMed: Turro 2020
,
PubMed: Wang 2014
,
1 more item
-
rs189638090
,
rs35955809
Germline, Germline/De novo (untested)
?, yes
2/314 case chromosomes
-
-
-
LOVD
+?/.
1
-
c.1A>G
r.(?)
p.?
-
likely pathogenic
g.182521733T>C
-
-
-
CERKL_000142
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
-
c.2T>G
r.(?)
p.(Met1?), p.0?
ACMG
pathogenic
g.182521732A>C
g.181657005A>C
-
-
CERKL_000063
case unsolved
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
,
PubMed: Sharon 2019
-
-
Germline
-
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
?/.
1
-
c.17G>T
r.(?)
p.(Arg6Leu)
-
VUS
g.182521717C>A
g.181656990C>A
CERKL(NM_001030311.2):c.17G>T (p.R6L)
-
CERKL_000025
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/., -?/.
2
-
c.27G>A
r.(?)
p.(Arg9=)
-
benign, likely benign
g.182521707C>T
g.181656980C>T
CERKL(NM_001030311.2):c.27G>A (p.R9=), CERKL(NM_001030311.3):c.27G>A (p.R9=)
-
CERKL_000053
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-?/.
1
-
c.46G>C
r.(?)
p.(Gly16Arg)
-
likely benign
g.182521688C>G
g.181656961C>G
CERKL(NM_001030311.2):c.46G>C (p.G16R)
-
CERKL_000052
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/., -?/.
2
-
c.66C>G
r.(?)
p.(Pro22=)
-
benign, likely benign
g.182521668G>C
g.181656941G>C
CERKL(NM_001030311.2):c.66C>G (p.P22=), CERKL(NM_001030311.3):c.66C>G (p.P22=)
-
CERKL_000024
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-/., -?/.
2
-
c.102G>T
r.(?)
p.(Thr34=)
-
benign, likely benign
g.182521632C>A
g.181656905C>A
CERKL(NM_001030311.2):c.102G>T (p.T34=), CERKL(NM_001030311.3):c.102G>T (p.T34=)
-
CERKL_000023
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
1
-
c.119_121del
r.(?)
p.(Glu40del)
-
VUS
g.182521615_182521617del
-
CERKL(NM_001030311.2):c.119_121delAGG (p.E40del)
-
CERKL_000100
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
1
c.154T>C
r.(?)
p.(Phe52Leu)
ACMG
VUS
g.182521580A>G
g.181656853A>G
c.[154T>C;158A>C]
-
CERKL_000139
-
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
-
-
Germline
-
-
-
-
-
Rebekkah Hitti-Malin
-/.
2
-
c.156C>T
r.(?)
p.(Phe52=)
-
benign
g.182521578G>A
g.181656851G>A
CERKL(NM_001030311.3):c.156C>T (p.F52=)
-
CERKL_000022
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+?/.
1
-
c.156_157insT
r.(?)
p.(Glu53*)
ACMG
pathogenic
g.182521577_182521578insA
-
-
-
CERKL_000087
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Jinu Han
-?/., ?/.
2
-
c.157G>A
r.(?)
p.(Glu53Lys)
-
likely benign, VUS
g.182521577C>T
g.181656850C>T
CERKL(NM_001030311.2):c.157G>A (p.E53K), CERKL(NM_001030311.3):c.157G>A (p.E53K)
-
CERKL_000051
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
1
1
c.158A>C
r.(?)
p.(Glu53Ala)
ACMG
VUS
g.182521576T>G
g.181656849T>G
c.[154T>C;158A>C]
-
CERKL_000138
-
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
-
-
Germline
-
-
-
-
-
Rebekkah Hitti-Malin
+?/.
2
1
c.182T>A
r.(?)
p.(Val61Glu)
-
likely pathogenic
g.182521552A>T
g.181656825A>T
CERKL Ex.1 c.182T>A p.(Val61Glu), Ex.6 c.847C>T p.(Arg283*),
1 more item
-
CERKL_000104
compound heterozygous, homozygous
PubMed: Martin Merida 2019
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
?/.
1
-
c.191G>A
r.(?)
p.(Ser64Asn)
-
VUS
g.182521543C>T
g.181656816C>T
CERKL(NM_001030311.2):c.191G>A (p.S64N)
-
CERKL_000056
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., ?/.
11
-
c.193G>T
r.(?)
p.(Glu65*)
-
pathogenic (recessive), VUS
g.182521541C>A
g.181656814C>A
-
-
CERKL_000073
-
PubMed: Avela 2018
,
PubMed: Ellingford 2016
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
3
1
c.197_200dup
r.(?)
p.(Leu68Serfs*15)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.182521542_182521545dup
g.181656815_181656818dup
c.197_200dupGAGC, p.Leu68Serfs*15,
1 more item
-
CERKL_000070
heterozygous
PubMed: Birtel 2018
,
PubMed: Birtel 2018
,
PubMed: Gliem 2020
-
-
Germline, Unknown
?
-
-
-
-
LOVD
-?/.
1
-
c.207G>A
r.(?)
p.(=)
-
likely benign
g.182521527C>T
-
CERKL(NM_001030311.3):c.207G>A (p.R69=)
-
CERKL_000132
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
2
1
c.237_238+13del
r.?, r.spl?
p.(Gly80*), p.?
-
pathogenic
g.182521483_182521497del, g.182521490_182521504del
-
c.237_238+13del
-
CERKL_000059
VKGL data sharing initiative Nederland
PubMed: Panneman 2023
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Nijmegen
,
Daan Panneman
+/., +?/.
31
11i, 1i
c.238+1G>A
r.spl
p.(?), p.?
ACMG
likely pathogenic, pathogenic
g.182521495C>T
g.181656768C>T
CERKL c.238+1G>A
-
CERKL_000027
founder mutation; homozygous
Sharon, submitted,
PubMed: Auslender 2007
,
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
,
2 more items
-
-
Germline
yes
19/2420 IRD families, 4/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Dror Sharon
,
Rebekkah Hitti-Malin
+?/.
1
1i
c.238+2T>C
r.spl?
p.?
-
likely pathogenic
g.182521494A>G
-
c.238+2T>C
-
CERKL_000108
-
PubMed: Ellingsford 2018
-
-
Germline
-
-
-
-
-
LOVD
-?/.
1
-
c.238+6T>C
r.(=)
p.(=)
-
likely benign
g.182521490A>G
-
CERKL(NM_001030311.2):c.238+6T>C
-
CERKL_000099
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.238+16C>G
r.(=)
p.(=)
-
benign
g.182521480G>C
g.181656753G>C
CERKL(NM_001030311.3):c.238+16C>G
-
CERKL_000021
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.239-14T>A
r.(=)
p.(=)
-
likely benign
g.182468820A>T
-
CERKL(NM_001030311.3):c.239-14T>A
-
CERKL_000098
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
3
-
c.239-12T>A
r.(=)
p.(=)
-
benign
g.182468818A>T
g.181604091A>T
CERKL(NM_001030311.2):c.239-12T>A, CERKL(NM_001030311.3):c.239-12T>A
-
CERKL_000050
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
+?/.
2
1i, 2i
c.239-2A>G
r.spl, r.spl?
p.(?), p.?
-
likely pathogenic, likely pathogenic (recessive)
g.182468808T>C
g.181604081T>C
c.239-2A>G, CERKL c.239-2A>G splice site, c.1628_1632dup, p.Ile545Aspfs*13
-
CERKL_000116
heterozygous
PubMed: Gliem 2020
,
PubMed: Liu-2020
-
-
Germline, Unknown
?
-
-
-
-
LOVD
+?/., ?/.
3
1i_2i, 2
c.(238+1_239-1)_(481+1_482-1)del
r.(?), r.?, r.spl
p.(?), p.(Leu68Serfs*15)
ACMG
likely pathogenic, VUS
g.(182521495_182468807)_(182468563_182438612)del, g.?
g.?
CERKL c.197_200dup whole-exon deletion, p.Leu68Serfs*15 putative loss of function, deletion of exon 2
-
SNRNP200_000007
heterozygous
PubMed: Birtel 2018
,
PubMed: Gliem 2020
,
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
-
-
Germline, Unknown
?
-
-
-
-
Rebekkah Hitti-Malin
-/.
4
-
c.242A>C
r.(?)
p.(Asp81Ala)
-
benign
g.182468803T>G
g.181604076T>G
CERKL(NM_001030311.2):c.242A>C (p.D81A)
-
CERKL_000020
VKGL data sharing initiative Nederland
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs61750041
CLASSIFICATION record, Germline
-
422/1204 cases with retinitis pigmentosa, 60/1204 cases with retinitis pigmentosa
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
Yoshito Koyanagi
+?/.
1
2
c.271G>T
r.(?)
p.(Glu91*)
-
likely pathogenic
g.182468774C>A
-
c.271G>T
-
CERKL_000130
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
+?/.
1
2
c.280G>T
r.(?)
p.(Glu94Ter)
ACMG
likely pathogenic
g.182468765C>A
g.181604038C>A
-
-
CERKL_000137
-
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
-
-
Germline
-
-
-
-
-
Rebekkah Hitti-Malin
-/., ?/.
7
-
c.313C>T
r.(?)
p.(Arg105Trp)
-
benign, VUS
g.182468732G>A
g.181604005G>A
CERKL(NM_001030311.3):c.313C>T (p.R105W)
-
CERKL_000019
VKGL data sharing initiative Nederland
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
,
PubMed: Xu 2014
-
rs149078111
CLASSIFICATION record, Germline
-
2/1204 cases with retinitis pigmentosa, 5/314 case chromosomes
-
-
-
VKGL-NL_AMC
,
Yoshito Koyanagi
+/., +?/., ?/.
6
6
c.316C>A
r.(?)
p.(Arg106Ser)
-
likely pathogenic, pathogenic (recessive), VUS
g.182468729G>T
g.181604002G>T
2:182468729G>T ENST00000339098.5:c.316C>A (Arg106Ser), CERKL p.(Arg106Ser),
1 more item
-
CERKL_000068
homozygous, VKGL data sharing initiative Nederland
PubMed: Ali 2008
,
PubMed: Carss 2017
-
-
CLASSIFICATION record, Germline
yes
-
-
-
-
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
+/., +?/.
4
2
c.316C>T
r.(?)
p.(Arg106Cys)
ACMG
likely pathogenic, pathogenic (recessive)
g.182468729G>A
g.181604002G>A
-
-
CERKL_000072
-
PubMed: Avela 2018
,
PubMed: Ellingford 2016
,
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
,
1 more item
-
-
Germline
-
-
-
-
-
Rebekkah Hitti-Malin
+/.
1
2
c.334C>T
r.(?)
p.(Gln112*)
-
pathogenic (recessive)
g.182468711G>A
-
c.334C>T
-
CERKL_000121
-
PubMed: Colombo-2020
-
rs772748858
Germline
yes
-
-
-
-
LOVD
+?/.
3
-
c.356G>A
r.(?)
p.(Gly119Asp)
ACMG
likely pathogenic, likely pathogenic (recessive)
g.182468689C>T
g.181603962C>T
356C>T, CERKL c.356G>A, p.(Gly119Asp), NM_001030311.2, c.356G>A, p.Gly119Asp
-
CERKL_000080
homozygous
PubMed: Dineiro 2020
,
PubMed: Ezquerra-Inchausti 2018
,
PubMed: Weisschuh 2016
-
-
Germline
?
-
-
-
-
LOVD
+?/., ?/.
7
2
c.365T>G
r.(?)
p.(Leu122Arg)
ACMG
likely pathogenic, likely pathogenic (recessive), VUS
g.182468680A>C
g.181603953A>C
CERKL(NM_001030311.2):c.365T>G (p.L122R), CERKL(NM_001030311.3):c.365T>G (p.L122R)
-
CERKL_000018
VKGL data sharing initiative Nederland
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
,
PubMed: Midgley 2024
,
PubMed: Roberts 2016
-
rs558913945
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
,
Rebekkah Hitti-Malin
+/., +?/., ?/.
26
2
c.375C>G
r.(?)
p.(Cys125Trp)
-
likely pathogenic, pathogenic, pathogenic (recessive), VUS
g.182468670G>C
g.181603943G>C
c.375C>G, CERKL c.375C>G , p.(Cys125Trp)
-
CERKL_000071
Check also: Avela 2018, single heterozygous variant in a recessive disease
PubMed: Avela 2018
,
PubMed: Avela 2019
,
PubMed: Ellingford 2016
,
PubMed: Littink 2010
,
1 more item
-
-
Germline, Unknown
yes
-
-
-
-
LOVD
?/.
1
-
c.384G>T
r.(?)
p.(Lys128Asn)
-
VUS
g.182468661C>A
g.181603934C>A
CERKL(NM_001030311.2):c.384G>T (p.K128N)
-
CERKL_000049
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.398T>C
r.(?)
p.(Leu133Pro)
-
pathogenic (recessive)
g.182468647A>G
g.181603920A>G
-
-
CERKL_000082
-
PubMed: Xu 2014
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
2
c.420del
r.(?)
p.(Ile141Leufs*3)
-
pathogenic
g.182468625del
-
c.420delT
-
CERKL_000094
-
PubMed: Nishiguchi-2013
-
-
Germline
-
-
-
-
-
LOVD
+/.
2
2
c.424_427del
r.(?)
p.(Asn142*)
ACMG
pathogenic, pathogenic (recessive)
g.182468621_182468624del
g.181603894_181603897del
424_427delAATT
-
CERKL_000041
ACMG PVS1, PM2, PM3, PP4
PubMed: Zenteno 2020
-
-
Germline
-
1/143 cases
-
-
-
Johan den Dunnen
,
Juan Carlos Zenteno
+?/.
1
2
c.426_430del
r.(?)
p.(Asn142Lysfs*2)
-
likely pathogenic
g.182468615_182468619del
-
c.426_430del
-
CERKL_000129
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
+/., +?/.
3
2
c.450_451del
r.(?)
p.(Ile150Metfs*3), p.(Ile150MetfsTer3)
ACMG
likely pathogenic, pathogenic
g.182468594_182468595del, g.182468596_182468597del
g.181603869_181603870del
CERKL c.450_451delAT; p.Ile150MetfsTer3
-
CERKL_000062
homozygous
PubMed: Azab 2019
,
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
,
PubMed: Sharon 2019
-
-
Germline
yes
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy
,
Rebekkah Hitti-Malin
+?/.
1
-
c.451T>G
r.(?)
p.(Trp151Gly)
-
likely pathogenic
g.182468594A>C
g.181603867A>C
-
-
CERKL_000085
-
PubMed: Huang 2015
-
-
Germline
yes
-
-
-
-
LOVD
+/.
2
-
c.453G>A
r.(?)
p.(Trp151*)
-
pathogenic
g.182468592C>T
g.181603865C>T
-
-
CERKL_000038
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs746595127
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+/., +?/., ?/.
7
2i
c.481+2T>G
r.spl, r.spl?
p.(?), p.?
ACMG
likely pathogenic, pathogenic, VUS
g.182468562A>C
g.181603835A>C
c.481+2T>G, CERKL c.481+2T>G, p.(?), c.481+2T>G, p.(?), IVS2+2T>G,
2 more items
-
CERKL_000026
homozygous, possibly solved, compound heterozygous, solved, homozygous,
1 more item
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
,
PubMed: Jespersgaar 2019
,
PubMed: Panneman 2023
,
2 more items
-
-
CLASSIFICATION record, Germline, Unknown
?
-
-
-
-
VKGL-NL_Nijmegen
,
Rebekkah Hitti-Malin
,
Daan Panneman
-?/.
1
-
c.482-19C>A
r.(=)
p.(=)
-
likely benign
g.182438630G>T
-
CERKL(NM_001030311.3):c.482-19C>A
-
CERKL_000131
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.497C>T
r.(?)
p.(Pro166Leu)
-
likely pathogenic
g.182438596G>A
g.181573869G>A
c.1483delT, p.(Val495*)
-
CERKL_000103
error in annotation: c.497G>A instead of C>T, compound heterozygous
PubMed: Wang 2019
-
-
Germline
?
-
-
-
-
LOVD
?/.
1
3
c.512T>C
r.(?)
p.(Ile171Thr)
ACMG
VUS
g.182438581A>G
g.181573854A>G
-
-
CERKL_000136
-
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
-
-
Germline
-
-
-
-
-
Rebekkah Hitti-Malin
+?/.
1
-
c.541_542delinsTT
r.(?)
p.(Glu181Leu)
-
likely pathogenic
g.182438551_182438552delinsAA
g.181573824_181573825delinsAA
CERKL, variant 1: c.1151+3_1151+6del/p.?, variant 2: c.541_542delinsTT/p.E181L
-
CERKL_000115
solved, compound heterozygous
PubMed: Weisschuh 2020
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.547A>T
r.(?)
p.(Thr183Ser)
-
likely pathogenic
g.182438546T>A
g.181573819T>A
-
-
CERKL_000084
-
PubMed: Huang 2015
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
-
c.589G>T
r.(?)
p.(Ala197Ser)
-
pathogenic
g.182438504C>A
g.181573777C>A
-
-
CERKL_000077
-
PubMed: Costa 2017
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
3
c.598A>T
r.(?)
p.(Lys200*)
-
pathogenic
g.182438495T>A
-
c.598A>T
-
CERKL_000093
-
PubMed: Nishiguchi-2013
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
3
c.608T>G
r.(?)
p.(Val203Gly)
-
VUS
g.182438485A>C
-
c.608T>G
-
CERKL_000128
-
PubMed: Panneman 2023
-
-
Unknown
-
-
-
-
-
Daan Panneman
+/., +?/.
2
-
c.613+4_613+5del
r.spl?
p.(?), p.?
-
likely pathogenic, pathogenic (recessive)
g.182438475_182438476del
g.181573748_181573749del
2:182438474ACT>A ENST00000339098.5:c.613+4_613+5delAG, CERKL c.613+4_613+5delAG,
-
CERKL_000067
heterozygous
PubMed: Carss 2017
,
PubMed: Turro 2020
-
-
Germline, Germline/De novo (untested)
?
-
-
-
-
LOVD
?/.
1
3i
c.613+5_613+8del
r.spl?
p.?
ACMG
VUS
g.182438478_182438481del
g.181573751_181573754del
-
-
CERKL_000004
-
PubMed: de Castro-Miró 2016
-
-
Germline
-
-
-
-
-
Marta de Castro-Miró
-?/.
1
-
c.613+20T>C
r.(=)
p.(=)
-
likely benign
g.182438460A>G
-
CERKL(NM_001030311.3):c.613+20T>C
-
CERKL_000127
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.617T>C
r.(?)
p.(Met206Thr)
-
VUS
g.182430845A>G
g.181566118A>G
-
-
CERKL_000037
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs768703034
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+/.
1
4
c.638dup
r.(?)
p.(Ser214Valfs*4)
-
pathogenic (recessive)
g.182430824dup
-
c.638dupT:p.L213fs
-
CERKL_000122
-
PubMed: Numa-2020
-
-
Unknown
-
-
-
-
-
LOVD
+/.
1
-
c.640del
r.(?)
p.(Ser214Hisfs*36)
-
pathogenic
g.182430822del
g.181566095del
-
-
CERKL_000036
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
-
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+?/.
1
-
c.664C>T
r.(?)
p.(Gln222Ter)
-
likely pathogenic
g.182430798G>A
g.181566071G>A
-
-
CERKL_000078
-
PubMed: Perez-Carro 2016
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
-
c.677+1G>A
r.spl?
p.?
-
pathogenic
g.182430784C>T
-
-
-
CERKL_000141
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
-
c.696G>T
r.(?)
p.(Leu232Phe)
-
benign
g.182430219C>A
g.181565492C>A
CERKL(NM_001030311.3):c.696G>T (p.L232F)
-
CERKL_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.708T>C
r.(?)
p.(His236=)
-
benign
g.182430207A>G
g.181565480A>G
CERKL(NM_001030311.2):c.708T>C (p.H236=)
-
CERKL_000048
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.715G>A
r.(?)
p.(Val239Ile)
-
likely pathogenic (recessive)
g.182430200C>T
g.181565473C>T
R239*
-
CERKL_000079
-
PubMed: Weisschuh 2016
-
-
Germline
-
-
-
-
-
LOVD
-/.
1
-
c.755+5_755+13dup
r.spl?
p.?
-
benign
g.182430149_182430157dup
g.181565422_181565430dup
CERKL(NM_001030311.3):c.755+5_755+13dupGTGATATAA
-
CERKL_000047
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.759del
r.(?)
p.(Val254SerfsTer12)
-
likely pathogenic
g.182423433del
g.181558706del
758delT
-
CERKL_000083
-
-
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/.
3
6
c.769C>T
r.(?), r.?
p.?, p.R257X
-
likely pathogenic, pathogenic
g.182423422G>A
-
c.769C.T, c.769C>T
-
CERKL_000092
C not found at position 870, found G instead.
PubMed: González-del Pozo-2011
,
PubMed: Nishiguchi-2013
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.772G>A
r.(?)
p.(Gly258Arg)
ACMG
likely pathogenic
g.182423419C>T
g.181558692C>T
CERKL c.772G>A, p.(Gly258Arg), c.847C>T, p.(Arg283*)
-
CERKL_000096
-
PubMed: Jespersgaar 2019
-
-
Germline
?
-
-
-
-
LOVD
+/.
1
6
c.780delT
r.?
p.?
-
pathogenic
g.182423411delA
-
c.780delT
-
CERKL_000091
-
PubMed: Nishiguchi-2013
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
6
c.789C>G
r.(?)
p.(Ser263Arg)
-
likely pathogenic (recessive)
g.182423402G>C
-
c.789C>G
-
CERKL_000120
-
PubMed: Liu-2020
-
-
Germline
-
-
-
-
-
LOVD
+/., +?/.
2
6
c.812T>C
r.(?)
p.(Leu271Pro)
-
likely pathogenic, pathogenic
g.182423379A>G
g.181558652A>G
CERKL c.734T>C; p.L245P, NM_201548.4:c.734T>C (p.Leu245Pro)
-
CERKL_000001
different transcript: NM_201548.5(CERKL):c.769C>T; homozygous
PubMed: Abu-Safieh-2013
,
PubMed: Khan 2015
-
-
Germline
yes
-
-
-
-
Leen Abu Safieh
-?/.
2
-
c.813G>C
r.(?)
p.(Leu271=)
-
likely benign
g.182423378C>G
g.181558651C>G
CERKL(NM_001030311.2):c.813G>C (p.L271=), CERKL(NM_001030311.3):c.813G>C (p.L271=)
-
CERKL_000016
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/., +?/., ?/.
118
3, 6
c.847C>T
r.(?)
p.(Arg283*), p.(Arg283Ter)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.182423344G>A
g.181558617G>A
2:182423344G>A ENST00000339098.5:c.847C>T (Arg283Ter), 769C>T (Arg257*), c.847C>T,
21 more items
-
CERKL_000003
1 heterozygous, no homozygous;
Clinindb (India)
, compound heterozygous, heterozygous, homozygous,
9 more items
PubMed: Aleman 2009
,
PubMed: Avela 2018
,
PubMed: Avila Fernandez 2010
,
PubMed: Avila-Fernandez 2008
,
34 more items
-
rs121909398
,
rs886044730
CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown
?, yes
1/143 cases, 1/2420 IRD families, 1/2794 individuals, not in 200 controls
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
James Hejtmancik
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
VKGL-NL_VUmc
,
VKGL-NL_AMC
,
Nereida Bravo Gil
,
Mohammed Faruq
,
Anna Tracewska
,
Rebekkah Hitti-Malin
,
Daan Panneman
?/.
1
-
c.848G>A
r.(?)
p.(Arg283Gln)
-
VUS
g.182423343C>T
g.181558616C>T
-
-
CERKL_000035
-
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs762961333
Germline
-
1/1204 cases with retinitis pigmentosa
-
-
-
Yoshito Koyanagi
+?/.
1
-
c.858del
r.(?)
p.(Pro287Leufs*10)
-
likely pathogenic (recessive)
g.182423333del
g.181558606del
858delT
-
CERKL_000066
-
PubMed: Taylor 2017
-
-
Germline
-
-
-
-
-
LOVD
-?/.
2
-
c.861T>C
r.(?)
p.(Pro287=)
-
likely benign
g.182423330A>G
g.181558603A>G
CERKL(NM_001030311.2):c.861T>C (p.P287=), CERKL(NM_001030311.3):c.861T>C (p.P287=)
-
CERKL_000046
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+?/.
1
-
c.890T>C
r.(?)
p.(Ile297Thr)
-
likely pathogenic
g.182423301A>G
g.181558574A>G
-
-
CERKL_000081
-
PubMed: Patel 2016
-
-
Germline
-
-
-
-
-
LOVD
-?/.
2
-
c.898+9G>A
r.(=)
p.(=)
-
likely benign
g.182423284C>T
g.181558557C>T
CERKL(NM_001030311.2):c.898+9G>A, CERKL(NM_001030311.3):c.898+9G>A
-
CERKL_000015
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
1
-
c.947T>C
r.(?)
p.(Ile316Thr)
-
VUS
g.182414387A>G
-
CERKL(NM_001030311.2):c.947T>C (p.I316T)
-
CERKL_000124
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.950C>T
r.(?)
p.(Thr317Ile)
-
VUS
g.182414384G>A
-
-
-
CERKL_000140
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
4
-
c.973+3A>G
r.spl?
p.?
-
benign
g.182414358T>C
g.181549631T>C
CERKL(NM_001030311.3):c.973+3A>G
-
CERKL_000014
VKGL data sharing initiative Nederland
PubMed: Koyanagi 2019
,
Journal: Koyanagi 2019
-
rs12623687
CLASSIFICATION record, Germline
-
416/1204 cases with retinitis pigmentosa, 54/1204 cases with retinitis pigmentosa
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
Yoshito Koyanagi
-?/.
1
-
c.973+13C>T
r.(=)
p.(=)
-
likely benign
g.182414348G>A
g.181549621G>A
CERKL(NM_001030311.3):c.973+13C>T
-
CERKL_000045
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
3
-
c.974-18T>A
r.(=)
p.(=)
-
benign
g.182413602A>T
g.181548875A>T
CERKL(NM_001030311.2):c.974-18T>A, CERKL(NM_001030311.3):c.974-18T>A
-
CERKL_000013
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
?/.
1
-
c.974-13T>C
r.(=)
p.(=)
-
VUS
g.182413597A>G
g.181548870A>G
CERKL(NM_001030311.3):c.974-13T>C
-
CERKL_000012
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/., -?/.
2
-
c.978T>C
r.(?)
p.(His326=)
-
benign, likely benign
g.182413580A>G
g.181548853A>G
CERKL(NM_001030311.2):c.978T>C (p.H326=), CERKL(NM_001030311.3):c.978T>C (p.H326=)
-
CERKL_000011
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/., +?/.
2
-
c.999C>A
r.(?)
p.(Cys333*), p.(Cys333Ter)
ACMG
likely pathogenic, pathogenic (recessive)
g.182413559G>T
g.181548832G>T
CERKL c.999C>A; p.C333*, NM_001030311.2:cc.999C>A; p.(Cys333*)
-
CERKL_000090
homozygous
PubMed: Khan 2015
,
PubMed: Patel 2018
-
rs75523
Germline
yes
-
-
-
-
LOVD
?/.
5
8
c.1027C>T
r.(?)
p.(Arg343Cys)
ACMG
VUS
g.182413531G>A
g.181548804G>A
c.1027C>T,
1 more item
-
CERKL_000010
VKGL data sharing initiative Nederland
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
,
PubMed: Panneman 2023
-
-
CLASSIFICATION record, Germline, Unknown
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
Rebekkah Hitti-Malin
,
Daan Panneman
+?/., ?/.
2
8
c.1028G>A
r.(?)
p.(Arg343His)
ACMG
likely pathogenic, VUS
g.182413530C>T
g.181548803C>T
CERKL, variant 1: c.1028G>A/p.R343H, variant 2: c.1028G>A/p.R343H
-
CERKL_000114
possibly solved, homozygous
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
,
PubMed: Weisschuh 2020
-
-
Germline, Unknown
?
-
-
-
-
Rebekkah Hitti-Malin
?/.
3
8
c.1031T>C
r.(?)
p.(Phe344Ser)
ACMG
VUS
g.182413527A>G
g.181548800A>G
c.1031T>C, CERKL(NM_001030311.2):c.1031T>C (p.F344S)
-
CERKL_000044
VKGL data sharing initiative Nederland
PubMed: Hitti-Malin 2024
,
Journal: Hitti-Malin 2024
,
PubMed: Panneman 2023
-
-
CLASSIFICATION record, Germline, Unknown
-
-
-
-
-
VKGL-NL_Rotterdam
,
Rebekkah Hitti-Malin
,
Daan Panneman
+/.
2
8
c.1032_1039dup
r.(?)
p.(Ser347Leufs*77)
ACMG
pathogenic, pathogenic (recessive)
g.182413520_182413527dup
g.181548793_181548800dup
1032_1039dupTGGGTTCT
-
CERKL_000040
ACMG PVS1, PM2, PM3, PP4
PubMed: Zenteno 2020
-
-
Germline
-
1/143 cases
-
-
-
Johan den Dunnen
,
Juan Carlos Zenteno
+/., ?/.
4
-
c.1045_1046del
r.(?)
p.(Met349Valfs*20)
-
pathogenic, pathogenic (recessive), VUS
g.182413512_182413513del
g.181548785_181548786del
1045_1046delAT, NM_201548.4:c.967_968delAT
-
CERKL_000076
-
PubMed: Biswas 2017
,
PubMed: Ellingford 2016
,
PubMed: Wang 2017
-
rs750151209
Germline
-
-
-
-
-
LOVD
+/., +?/.
3
8
c.1090C>T
r.(?)
p.(Arg364*), p.(Arg364Ter)
ACMG
likely pathogenic (recessive), pathogenic
g.182413468G>A
g.181548741G>A
-
-
CERKL_000061
case unsolved
PubMed: Boulanger-Scemama 2015
,
PubMed: Boulanger-Scemama 2019
,
PubMed: Weisschuh 2016
,
1 more item
-
-
Germline
-
-
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
+/., +?/.
4
-
c.1151+3_1151+6del
r.spl?
p.(?), p.?
-
likely pathogenic, pathogenic (recessive)
g.182413401_182413404del, g.182413406_182413409del
g.181548679_181548682del
2:182413400GACTT>G ENST00000339098.5:c.1151+3_1151+6delAAGT, CERKL c.1151+3_1151+6delAAGT,,
2 more items
-
CERKL_000043
homozygous, solved, compound heterozygous, VKGL data sharing initiative Nederland
PubMed: Carss 2017
,
PubMed: Turro 2020
,
PubMed: Weisschuh 2020
-
-
CLASSIFICATION record, Germline, Germline/De novo (untested)
?, yes
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.1151+11T>C
r.(=)
p.(=)
-
benign
g.182413396A>G
g.181548669A>G
CERKL(NM_001030311.3):c.1151+11T>C
-
CERKL_000009
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
8
6, 9
c.1164_1165del
r.(?)
p.(Cys388*)
-
likely pathogenic
g.182413295_182413296del, g.182413320_182413321del
g.181548568_181548569del, g.181548593_181548594del
CERKL c.1187_1188delTG; p.Cys362Ter, CERKL c.60_61delGT, p.C362X,
1 more item
-
CERKL_000113
different transcript: NM_201548.5(CERKL):c.60_61delGT; compound heterozygous,
2 more items
PubMed: Azab 2019
,
PubMed: Weisschuh 2020
-
-
Germline, Unknown
?, yes
-
-
-
-
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