Global Variome shared LOVD
KARS (lysyl-tRNA synthetase)
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Global Variome, with Curator vacancy
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Unique variants in the KARS gene
NOTE: gene name changed from KARS to KARS1
The variants shown are described using the NM_005548.2 transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Reported
: The number of times this variant has been reported in the database.
Exon
: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA)
: description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change
: description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein
: description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Classification method
: The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification
: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19)
: HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38)
: HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as
: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN
: description of the variant according to ISCN nomenclature
DB-ID
: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks
: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference
: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID
: ID of variant in ClinVar database
dbSNP ID
: the dbSNP ID
Origin
: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation
: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency
: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site
: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP
: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator. NOTE: to get VIP status ask the curator.
Methylation
: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
How to query this table
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Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
all entries containing 'Arg' and 'Ser'
|
Text
Arg|Ser
all entries containing 'Arg' or 'Ser'
!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
=""
all entries with this field empty
=""
Text
="p.0"
all entries exactly matching 'p.0'
!=""
Text
!=""
all entries with this field not empty
!=""
Text
!="p.0"
all entries not exactly matching 'p.0?'
combination
Text
*|Ter !fs
all entries containing '*' or 'Ter' but not containing 'fs'
Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
!
Date
!2020-03
all entries not matching March, 2020
<
Date
<2020
all entries before the year 2020
<=
Date
<=2020-06
all entries in or before June, 2020
>
Date
>2020-06
all entries after June, 2020
>=
Date
>=2020-06-15
all entries on or after June 15th, 2020
combination
Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
all entries exactly matching 23
|
Numeric
23|24
all entries exactly matching 23 or 24
!
Numeric
!23
all entries not exactly matching 23
<
Numeric
<23
all entries lower than 23
<=
Numeric
<=23
all entries lower than, or equal to, 23
>
Numeric
>23
all entries higher than 23
>=
Numeric
>=23
all entries higher than, or equal to, 23
combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
Example
Matches
Asian
all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
To sort on a certain column, click on the column header or on the arrows. If that column is already selected to sort on, the sort order will be swapped. The column currently sorted on has a darker blue background color than the other columns. The up and down arrows next to the column name indicate the current sorting direction. When sorting on any field other than the default, LOVD will sort secondarily on the default sort column.
93 entries on 1 page. Showing entries 1 - 93.
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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.-974G>A
r.(?)
p.(=)
-
VUS
g.75682511C>T
-
TERF2IP(NM_018975.3):c.670+61C>T
-
KARS_000096
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
2
-
c.-219G>T
r.(?)
p.(=)
-
benign
g.75681756C>A
g.75647858C>A
TERF2IP(NM_018975.3):c.-25C>A (p.(=))
-
TERF2IP_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
-/.
1
-
c.-206G>C
r.(?)
p.(=)
-
benign
g.75681743C>G
g.75647845C>G
-
-
TERF2IP_000003
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
-
c.-162C>A
r.(?)
p.(=)
-
benign
g.75681699G>T
g.75647801G>T
-
-
TERF2IP_000002
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.5C>T
r.(?)
p.(Ala2Val)
-
likely pathogenic (recessive)
g.75681533G>A
-
-
-
KARS_000072
ACMG PM3, PP3, PP4, BS3
PubMed: Cappuccio 2021
,
Journal: Cappuccio 2021
-
-
Germline
-
-
-
-
-
Gerarda Cappuccio
-?/.
1
-
c.15G>A
r.(?)
p.(Gln5=)
-
likely benign
g.75681523C>T
-
KARS1(NM_005548.3):c.15G>A (p.Q5=)
-
KARS_000071
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.18G>A
r.(?)
p.(Ala6=)
-
likely benign
g.75681520C>T
-
KARS1(NM_005548.3):c.18G>A (p.A6=)
-
KARS_000092
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.63-2684A>T
r.(=)
p.(=)
-
VUS
g.75678305T>A
g.75644407T>A
KARS(NM_001130089.1):c.22A>T (p.R8W)
-
KARS_000015
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.63-2584A>C
r.(=)
p.(=)
-
VUS
g.75678205T>G
g.75644307T>G
KARS(NM_001130089.1):c.122A>C (p.D41A)
-
KARS_000035
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.63-20C>T
r.(=)
p.(=)
-
likely benign
g.75675641G>A
-
KARS1(NM_001130089.2):c.147-20C>T
-
KARS_000070
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
1
-
c.75A>G
r.(?)
p.(Arg25=)
-
benign
g.75675609T>C
g.75641711T>C
KARS1(NM_001130089.2):c.159A>G (p.R53=)
-
KARS_000014
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
2
-
c.85G>C
r.(?)
p.(Ala29Pro)
ACMG
pathogenic (recessive)
g.75675599C>G
g.75641701C>G
-
-
KARS_000073
ACMG PS1, PS3, PM2, PP1
PubMed: Cappuccio 2021
,
Journal: Cappuccio 2021
-
-
Germline
-
-
-
-
-
Gerarda Cappuccio
+?/.
1
-
c.139C>T
r.(?)
p.(Gln47Ter)
-
likely pathogenic
g.75675545G>A
g.75641647G>A
-
-
KARS_000086
no variant 2nd chromosome
PubMed: Ganapathy 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.139del
r.(?)
p.(Gln47Serfs*2)
ACMG
pathogenic (recessive)
g.75675545del
g.75641647del
223delC
-
KARS_000074
ACMG PVS1, PM2, PM3
PubMed: Cappuccio 2021
,
Journal: Cappuccio 2021
-
-
Germline
-
-
-
-
-
Gerarda Cappuccio
-?/.
2
-
c.222+10C>T
r.(=)
p.(=)
-
likely benign
g.75675452G>A
g.75641554G>A
KARS(NM_001130089.1):c.306+10C>T, KARS1(NM_001130089.1):c.306+10C>T
-
KARS_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
-?/.
1
-
c.223-8_223-6del
r.(=)
p.(=)
-
likely benign
g.75674268_75674270del
g.75640370_75640372del
KARS1(NM_001130089.2):c.307-8_307-6delTTT
-
KARS_000026
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
2
-
c.223-7_223-6del
r.(=)
p.(=)
-
benign
g.75674269_75674270del
g.75640371_75640372del
KARS1(NM_001130089.1):c.307-7_307-6delTT, KARS1(NM_001130089.2):c.307-7_307-6delTT
-
KARS_000012
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_AMC
-/.
1
-
c.223-7_223-6dup
r.(=)
p.(=)
-
benign
g.75674269_75674270dup
g.75640371_75640372dup
KARS(NM_001130089.1):c.307-7_307-6dupTT
-
KARS_000027
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/., -?/.
4
-
c.223-6del
r.(=)
p.(=)
-
benign, likely benign
g.75674270del
g.75640372del
1 more item
-
KARS_000011
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
-/., -?/.
3
-
c.223-6dup
r.(=)
p.(=)
-
benign, likely benign
g.75674270dup
g.75640372dup
1 more item
-
KARS_000028
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-?/.
1
-
c.223-6T>A
r.(=)
p.(=)
-
likely benign
g.75674253A>T
-
KARS1(NM_001130089.1):c.307-6T>A
-
KARS_000093
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
-
c.238C>T
r.(?)
p.(Arg80Cys)
-
pathogenic (recessive)
g.75674232G>A
-
-
-
KARS_000075
ACMG PS3, PM2, PM3, PM5
PubMed: Cappuccio 2021
,
Journal: Cappuccio 2021
-
-
Germline
-
-
-
-
-
Gerarda Cappuccio
+?/.
1
-
c.239G>A
r.(?)
p.(Arg80His)
-
likely pathogenic
g.75674231C>T
g.75640333C>T
KARS1(NM_001130089.2):c.323G>A (p.R108H)
-
KARS_000010
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
?/.
1
-
c.274G>C
r.(?)
p.(Glu92Gln)
-
VUS
g.75674196C>G
g.75640298C>G
KARS(NM_001130089.1):c.358G>C (p.E120Q)
-
KARS_000034
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
5
-
c.295T>C
r.(?)
p.(Phe99Leu)
ACMG
likely pathogenic (recessive)
g.75674175A>G
-
-
-
KARS_000053
-
PubMed: Lin 2021
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
,
Barbara Vona
+/.
1
-
c.297C>G
r.(?)
p.(Phe99Leu)
-
pathogenic (recessive)
g.75674173G>C
-
-
-
KARS_000061
-
PubMed: Ardissone 2018
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.314T>A
r.(?)
p.(Leu105His)
-
pathogenic (recessive)
g.75674156A>T
g.75640258A>T
398T>A (Leu133His)
-
KARS_000045
-
PubMed: McLaughlin 2010
-
-
Germline
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
1
-
c.370A>G
r.(?)
p.(Ile124Val)
-
VUS
g.75674100T>C
g.75640202T>C
KARS1(NM_001130089.2):c.454A>G (p.I152V)
-
KARS_000009
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.389-2A>C
r.spl?
p.?
-
likely pathogenic
g.75670447T>G
g.75636549T>G
KARS1(NM_001130089.1):c.473-2A>C
-
KARS_000033
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/+, +/.
3
4
c.433T>C
r.(?)
p.(Tyr145His)
-
pathogenic, pathogenic (recessive)
g.75670401A>G
g.75636503A>G
-
-
KARS_000043
-
MORL Deafness Variation Database
,
PubMed: Basit 2011
,
PubMed: Szafranski 2015
,
1 more item
-
-
Germline, SUMMARY record
yes
-
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
+/.
1
4
c.440_441dup
r.(?)
p.(Tyr145Serfs*9)
-
pathogenic (recessive)
g.75670393_75670394dup
g.75636495_75636496dup
524_525insTT
-
KARS_000042
-
PubMed: McLaughlin 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.482+8G>A
r.(=)
p.(=)
-
likely benign
g.75670344C>T
g.75636446C>T
KARS1(NM_001130089.2):c.566+8G>A
-
KARS_000032
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/., +?/.
3
-
c.529C>T
r.(?)
p.(Arg177Cys)
ACMG
likely pathogenic, pathogenic (recessive)
g.75669950G>A
g.75636052G>A
KARS1(NM_001130089.2):c.613C>T (p.R205C)
-
KARS_000025
ACMG PS3, PM2, PP1, PP2, PP3, PP4, VKGL data sharing initiative Nederland
PubMed: Cappuccio 2021
,
Journal: Cappuccio 2021
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
VKGL-NL_VUmc
,
Gerarda Cappuccio
?/.
1
-
c.572C>T
r.(?)
p.(Thr191Ile)
-
VUS
g.75669907G>A
g.75636009G>A
KARS(NM_001130089.1):c.656C>T (p.(Thr219Ile))
-
KARS_000024
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., +?/.
9
5, 6
c.599C>T
r.(?)
p.(Pro200Leu)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive), VUS
g.75669880G>A
g.75635982G>A
KARS(NM_005548.3):c.599C>T (p.(Pro200Leu)), KARS1(NM_001130089.2):c.683C>T (p.P228L)
-
KARS_000041
VKGL data sharing initiative Nederland
PubMed: Fuchs 2019
,
PubMed: Lieber 2013
,
PubMed: Lin 2021
,
PubMed: Scheidecker 2019
ClinVar-RCV000681462.1
,
ClinVar-RCV000986183.1
-
CLASSIFICATION record, Germline, Unknown
yes
-
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
,
VKGL-NL_Leiden
,
VKGL-NL_AMC
,
Barbara Vona
-?/.
1
-
c.600G>A
r.(?)
p.(Pro200=)
-
likely benign
g.75669879C>T
g.75635981C>T
KARS1(NM_001130089.2):c.684G>A (p.P228=)
-
KARS_000031
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
1
-
c.601T>C
r.(?)
p.(Tyr201His)
-
benign
g.75669878A>G
g.75635980A>G
KARS(NM_001130089.1):c.685T>C (p.Y229H)
-
KARS_000023
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/., -?/.
3
-
c.612A>G
r.(?)
p.(Thr204=)
-
benign, likely benign
g.75669867T>C
g.75635969T>C
1 more item
-
KARS_000008
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
+/.
2
-
c.613C>G
r.(?)
p.(Leu205Val)
ACMG
pathogenic (recessive)
g.75669866G>C
-
-
-
KARS_000052
-
PubMed: Lin 2021
,
PubMed: Sun 2019
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
,
Barbara Vona
?/.
1
-
c.679T>A
r.(?)
p.(Tyr227Asn)
-
VUS
g.75669694A>T
-
KARS1(NM_001130089.2):c.763T>A (p.Y255N)
-
KARS_000088
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.680A>G
r.(?)
p.(Tyr227Cys)
-
VUS
g.75669693T>C
g.75635795T>C
-
-
KARS_000085
-
PubMed: Ganapathy 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
2
7
c.690A>T
r.(?)
p.(Arg230Ser)
ACMG
likely pathogenic (recessive)
g.75669683T>A
-
-
-
KARS_000065
-
PubMed: Lin 2021
ClinVar-RCV000660587.1
-
Germline
yes
-
-
-
-
Barbara Vona
-?/., ?/.
3
-
c.717T>G
r.(?)
p.(Phe239Leu)
-
likely benign, VUS
g.75669656A>C
g.75635758A>C
1 more item
-
KARS_000022
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+/.
1
-
c.731T>G
r.(?)
p.(Phe244Cys)
-
pathogenic (recessive)
g.75669642A>C
-
-
-
KARS_000059
-
PubMed: Ardissone 2018
-
rs138062606
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
3
-
c.787T>G
r.(?)
p.(Phe263Val)
ACMG
pathogenic (recessive), VUS
g.75669586A>C
g.75635688A>C
-
-
KARS_000017
ACMG PS1, PS3, PM2, PM3, PP2, PP3, PP4, VKGL data sharing initiative Nederland
PubMed: Cappuccio 2021
,
Journal: Cappuccio 2021
,
PubMed: Scheidecker 2019
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
,
Gerarda Cappuccio
+/.
1
-
c.797T>C
r.(?)
p.(Ile266Thr)
-
pathogenic (recessive)
g.75668189A>G
-
-
-
KARS_000056
-
PubMed: Sun 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.805C>G
r.(?)
p.(Pro269Ala)
ACMG
likely pathogenic (recessive)
g.75668181G>C
-
-
-
KARS_000051
-
PubMed: Lin 2021
-
-
Germline
yes
-
-
-
-
Barbara Vona
-?/., ?/.
2
7
c.822C>G
r.(?)
p.(Ile274Met)
-
likely benign, VUS
g.75668164G>C
g.75634266G>C
906C>G (Ile302Met), KARS1(NM_001130089.2):c.906C>G (p.I302M)
-
KARS_000021
in vitro analysis shows variant maintains normal aminoacylation catalytic activity,
1 more item
PubMed: McLaughlin 2010
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Global Variome, with Curator vacancy
,
VKGL-NL_AMC
+?/.
1
8
c.904C>T
r.(?)
p.(Leu302Phe)
ACMG
likely pathogenic (recessive)
g.75668082G>A
-
-
-
KARS_000068
-
PubMed: Lin 2021
ClinVar-RCV000489757.1
-
Germline
yes
-
-
-
-
Barbara Vona
+?/.
2
-
c.905T>C
r.(?)
p.(Leu302Pro)
ACMG
likely pathogenic (recessive)
g.75668081A>G
-
-
-
KARS_000050
-
PubMed: Lin 2021
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
,
Barbara Vona
?/.
1
-
c.941G>A
r.(?)
p.(Arg314Gln)
-
VUS
g.75665728C>T
g.75631830C>T
KARS(NM_001130089.1):c.1025G>A (p.R342Q)
-
KARS_000007
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.944T>C
r.(?)
p.(Val315Ala)
-
VUS
g.75665725A>G
g.75631827A>G
-
-
KARS_000016
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.953T>C
r.(?)
p.(Ile318Thr)
ACMG
pathogenic (recessive)
g.75665716A>G
-
-
-
KARS_000076
ACMG PS1, PS3, PM2, PM3, PP2, PP3, PP4
PubMed: Cappuccio 2021
,
Journal: Cappuccio 2021
-
-
Germline
-
-
-
-
-
Gerarda Cappuccio
+/.
1
-
c.958C>T
r.(?)
p.(Arg320Cys)
ACMG
pathogenic (recessive)
g.75665711G>A
-
-
-
KARS_000077
ACMG PS3, PM2, PM3, PM5, PP2, PP3, PP4
PubMed: Cappuccio 2021
,
Journal: Cappuccio 2021
-
-
Germline
-
-
-
-
-
Gerarda Cappuccio
+/.
1
-
c.959G>A
r.(?)
p.(Arg320His)
-
pathogenic (recessive)
g.75665710C>T
-
-
-
KARS_000060
-
PubMed: Ardissone 2018
-
rs527981017
Germline
-
-
-
-
-
Johan den Dunnen
+?/., ?/.
3
9
c.967C>T
r.(?)
p.(Arg323Trp)
ACMG
likely pathogenic, likely pathogenic (recessive), VUS
g.75665702G>A
-
KARS(NM_001130089.1):c.1051C>T (p.R351W)
-
KARS_000030, KARS_000067
VKGL data sharing initiative Nederland
PubMed: Lin 2021
ClinVar-RCV000489266.1
-
CLASSIFICATION record, Germline
yes
-
-
-
-
Carmen Espinós
,
VKGL-NL_Rotterdam
,
Barbara Vona
?/.
1
-
c.979A>G
r.(?)
p.(Ile327Val)
-
VUS
g.75665690T>C
-
KARS(NM_001130089.1):c.1063A>G (p.I355V)
-
KARS_000063
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.989C>T
r.(?)
p.(Thr330Met)
ACMG
VUS
g.75665680G>A
-
-
-
KARS_000049
-
PubMed: Lin 2021
-
-
Germline
yes
-
-
-
-
Barbara Vona
-?/.
1
-
c.990G>C
r.(?)
p.(=)
-
likely benign
g.75665679C>G
-
KARS1(NM_001130089.2):c.1074G>C (p.T358=)
-
KARS_000095
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
2
-
c.1040A>G
r.(?)
p.(Tyr347Cys)
ACMG
pathogenic (recessive)
g.75665629T>C
-
-
-
KARS_000048
-
PubMed: Ardissone 2018
,
PubMed: Lin 2021
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
,
Barbara Vona
+/+, +/.
2
8
c.1045G>A
r.(?)
p.(Asp349Asn)
-
pathogenic, pathogenic (recessive)
g.75665624C>T
g.75631726C>T
-
-
KARS_000040
-
MORL Deafness Variation Database
,
PubMed: Basit 2011
,
PubMed: Szafranski 2015
,
1 more item
-
-
Germline, SUMMARY record
yes
-
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
-/.
1
-
c.1079-7C>T
r.(=)
p.(=)
-
benign
g.75665494G>A
g.75631596G>A
KARS1(NM_001130089.2):c.1163-7C>T
-
KARS_000006
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.1083G>A
r.(?)
p.(Met361Ile)
-
VUS
g.75665483C>T
-
KARS1(NM_001130089.2):c.1167G>A (p.M389I)
-
KARS_000090
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
-
c.1120C>T
r.(?)
p.(His374Tyr)
ACMG
pathogenic (recessive)
g.75665446G>A
-
-
-
KARS_000078
ACMG PS3, PM2, PM3, PP2, PP3, PP4
PubMed: Cappuccio 2021
,
Journal: Cappuccio 2021
-
-
Germline
-
-
-
-
-
Gerarda Cappuccio
-?/., ?/.
3
-
c.1178G>A
r.(?)
p.(Arg393Gln)
-
likely benign, VUS
g.75665388C>T
g.75631490C>T
1 more item
-
KARS_000005
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
+/.
1
-
c.1197_1198insAGA
r.(?)
p.(Glu399_Leu400insArg)
-
pathogenic (recessive)
g.75665368_75665369insTCT
-
-
-
KARS_000055
-
PubMed: Sun 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.1219A>T
r.(?)
p.(Lys407*)
-
likely pathogenic
g.75665347T>A
-
KARS(NM_005548.3):c.1219A>T (p.(Lys407*))
-
KARS_000097
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
-
c.1248_1249del
r.(?)
p.(Glu417Argfs*7)
-
likely pathogenic
g.75665317_75665318del
-
KARS1(NM_001130089.2):c.1332_1333delTG (p.E445Rfs*7)
-
KARS_000087
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/., ?/.
4
-
c.1258C>T
r.(?)
p.(Arg420Cys)
-
likely benign, VUS
g.75665146G>A
g.75631248G>A
1 more item
-
KARS_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
+/+
1
10
c.1312C>T
r.(?)
p.(Arg438Trp)
-
pathogenic
g.75665092G>A
g.75631194G>A
-
-
KARS_000039
-
MORL Deafness Variation Database
,
PubMed: McMillan 2015
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+?/.
1
-
c.1342G>T
r.(?)
p.(Val448Phe)
-
likely pathogenic
g.75664403C>A
g.75630505C>A
KARS1(NM_001130089.2):c.1426G>T (p.V476F)
-
KARS_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
?/.
1
-
c.1346G>C
r.(?)
p.(Gly449Ala)
-
VUS
g.75664399C>G
-
KARS1(NM_001130089.2):c.1430G>C (p.G477A)
-
KARS_000089
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.1368C>T
r.(?)
p.(Cys456=)
-
likely benign
g.75664377G>A
-
KARS(NM_001130089.1):c.1452C>T (p.C484=)
-
KARS_000062
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.1412C>T
r.(?)
p.(Pro471Leu)
ACMG
pathogenic (recessive)
g.75664333G>A
-
-
-
KARS_000084
ACMG PS3, PM2, PM3, PP2, PP3, PP4
PubMed: Cappuccio 2021
,
Journal: Cappuccio 2021
-
-
Germline
-
-
-
-
-
Gerarda Cappuccio
+/.
2
-
c.1430G>A
r.(?)
p.(Arg477His)
-
pathogenic (recessive)
g.75663434C>T
-
-
-
KARS_000057
-
PubMed: Ardissone 2018
,
PubMed: Sun 2019
-
rs778748895
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.1433C>T
r.(?)
p.(Ser478Phe)
-
VUS
g.75663431G>A
-
KARS(NM_005548.3):c.1433C>T (p.(Ser478Phe))
-
KARS_000094
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
2
-
c.1485A>G
r.(?)
p.(Ile495Met)
-
VUS
g.75663379T>C
g.75629481T>C
KARS(NM_005548.3):c.1485A>G (p.(Ile495Met)), KARS1(NM_001130089.2):c.1569A>G (p.I523M)
-
KARS_000046
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_AMC
+?/.
1
-
c.1493C>T
r.(?)
p.(Ala498Val)
ACMG
likely pathogenic (recessive)
g.75663371G>A
-
-
-
KARS_000066
-
PubMed: Lin 2021
ClinVar-RCV000986181.1
-
Germline
yes
-
-
-
-
Barbara Vona
+/.
1
-
c.1513C>T
r.(?)
p.(Pro505Ser)
-
pathogenic (recessive)
g.75663351G>A
-
-
-
KARS_000058
-
PubMed: Sun 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.1514C>G
r.(?)
p.(Pro505Arg)
ACMG
pathogenic (recessive)
g.75663350G>C
-
-
-
KARS_000080
ACMG PS3, PM2, PM3, PM5, PP1, PP2, PP3, PP4
PubMed: Cappuccio 2021
,
Journal: Cappuccio 2021
-
-
Germline
-
-
-
-
-
Gerarda Cappuccio
+?/+?
1
12
c.1525C>T
r.(?)
p.(Arg509Trp)
-
likely pathogenic
g.75663339G>A
g.75629441G>A
-
-
KARS_000038
-
MORL Deafness Variation Database
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
+/+
1
13
c.1573G>A
r.(?)
p.(Glu525Lys)
-
pathogenic
g.75662589C>T
g.75628691C>T
-
-
KARS_000037
-
MORL Deafness Variation Database
,
PubMed: McMillan 2015
-
-
SUMMARY record
-
-
-
-
-
Global Variome, with Curator vacancy
?/.
1
-
c.1634C>T
r.(?)
p.(Ala545Val)
-
VUS
g.75662528G>A
g.75628630G>A
KARS(NM_001130089.1):c.1718C>T (p.A573V)
-
KARS_000003
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.1641G>A
r.(?)
p.(Trp547Ter)
-
likely benign
g.75662521C>T
g.75628623C>T
KARS(NM_001130089.1):c.1725G>A (p.(Trp575Ter))
-
KARS_000019
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.1648_1660del
r.(?)
p.(Gly550Serfs*20)
-
pathogenic (recessive)
g.75662502_75662514del
-
1732_1744delGGCATTGATCGAG
-
KARS_000069
-
PubMed: Fuchs 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.1658G>A
r.(?)
p.(Arg553Gln)
ACMG
VUS
g.75662504C>T
-
-
-
KARS_000064
-
PubMed: Lin 2021
-
-
Germline
yes
-
-
-
-
Barbara Vona
+/.
1
-
c.1670T>G
r.(?)
p.(Phe557Cys)
ACMG
pathogenic (recessive)
g.75662492A>C
-
-
-
KARS_000081
ACMG PS3, PM2, PM3, PP2, PP3, PP4
PubMed: Cappuccio 2021
,
Journal: Cappuccio 2021
-
-
Germline
-
-
-
-
-
Gerarda Cappuccio
-/., -?/.
3
-
c.1674C>T
r.(?)
p.(Leu558=)
-
benign, likely benign
g.75662488G>A
g.75628590G>A
1 more item
-
KARS_000018
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
+/., +?/.
3
13
c.1676C>T
r.(?)
p.(Thr559Met)
ACMG
likely pathogenic (recessive), pathogenic (recessive), VUS
g.75662486G>A
g.75628588G>A
-
-
KARS_000036
-
PubMed: Lieber 2013
,
PubMed: Lin 2021
,
PubMed: Sun 2019
-
-
Germline
-
-
-
-
-
Global Variome, with Curator vacancy
,
Johan den Dunnen
+/.
1
-
c.1685G>C
r.(?)
p.(Cys562Ser)
ACMG
pathogenic (recessive)
g.75662561C>G
g.75628663C>G
-
-
KARS_000091
Father not tested
PubMed: Wonkam 2022
-
rs1156833108
Germline/De novo (untested)
yes
-
-
-
-
Yacouba Dia
+/., +?/.
5
-
c.1688A>T
r.(?)
p.(Asn563Ile)
ACMG
likely pathogenic (recessive)
g.75662474T>A
-
-
-
KARS_000047
-
PubMed: Lin 2021
-
-
Germline, Unknown
?, yes
-
-
-
-
Johan den Dunnen
,
Barbara Vona
+/.
1
-
c.1702C>T
r.(?)
p.(Leu568Phe)
-
pathogenic (recessive)
g.75661885G>A
-
-
-
KARS_000054
-
PubMed: Sun 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/., -?/.
2
-
c.1784C>G
r.(?)
p.(Thr595Ser)
-
benign, likely benign
g.75661803G>C
g.75627905G>C
KARS1(NM_001130089.2):c.1868C>G (p.T623S), Thr623Ser
-
KARS_000002
in vitro analysis shows variant maintains normal aminoacylation catalytic activity,
1 more item
PubMed: McLaughlin 2010
-
rs6834
CLASSIFICATION record, Germline
-
31/710 chromosomes
-
-
-
Johan den Dunnen
,
VKGL-NL_AMC
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