Global Variome shared LOVD
ASS1 (argininosuccinate synthase 1)
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Unique variants in the ASS1 gene
The variants shown are described using the NM_000050.4 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'
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.
211 entries on 3 pages. Showing entries 1 - 100.
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Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
?/.
1
-
c.-6+8C>T
r.(=)
p.(=)
-
VUS
g.133325728C>T
g.130450341C>T
ASS1(NM_000050.4):c.-6+8C>T (p.(=))
-
ASS1_000101
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
1
-
c.-5-13C>T
r.(=)
p.(=)
-
benign
g.133327598C>T
g.130452211C>T
ASS1(NM_000050.4):c.-5-13C>T
-
ASS1_000106
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
2i
c.-5-10C>G
r.(-5_35del)
p.)?
-
pathogenic (recessive)
g.133327601C>G
g.130452214C>G
-
-
ASS1_000154
-
PubMed: Diez-Fernandez 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
2, 3
c.-4C>T
r.(?)
p.?
-
pathogenic, pathogenic (recessive)
g.133327612C>T
g.130452225C>T
int0 -4C>T
-
ASS1_000055
described as nonsense variant; combination of variants not reported
PubMed: Diez-Fernandez 2017
,
PubMed: Engel 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
10i_12i
c.(c.688+1_689-1)_(838+1_839-1)del
r.689_838del
p.?
-
pathogenic
g.(133352349_133355102)_(133355837_133364719)del
-
-
-
ASS1_000008
combination of variants not reported
PubMed: Engel 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
_1_2i
c.-356_(-6+1_-5-1){0}
r.0?
p.0?
-
pathogenic (recessive)
g.(?_133320094)_(133325721_133327610)del
g.(?_130444707)_(130450334_130452223)del
del ex1-2
-
ASS1_000206
-
PubMed: Lee 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
_1_1i
c.-356_-68+75{0}
-, r.(=|red)
p.(=|red), p.=|0.03
-
NA, pathogenic (recessive)
g.133319980_133320457del
g.130444593_130445070del
hg19 133319979-133320456del
-
ASS1_000205
expression cloning (A2058 and HepG2 cells) shows reduced reporter expression (0.033-0.036),
1 more item
PubMed: Imagawa 2020
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.18C>T
r.(?)
p.(Ser6=)
-
VUS
g.133327633C>T
-
ASS1(NM_054012.4):c.18C>T (p.(Ser6=))
-
ASS1_000121
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.19G>A
-
p.Val7Met
-
VUS
g.133327634G>A
g.130452247G>A
-
-
ASS1_000155
<0.80 no residual enzymatic ASS1 activity combined variants
PubMed: Zielonka 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
2
c.34A>G
r.(?)
p.(Ser12Gly)
ACMG
likely pathogenic (recessive)
g.133327649A>G
g.130452262A>G
-
-
ASS1_000136
1 more item
-
-
rs1283372037
Germline
yes
-
-
-
-
Miriam Erandi Reyna-Fabián
+/.
7
2, 3
c.40G>A
r.(?), r.40g>a
p.(Gly14Ser), p.Gly14Ser
ACMG
pathogenic (recessive)
g.133327655G>A
g.130452268G>A
G14S
-
ASS1_000056
plasma citrulline 1100 umol/L, plasma citrulline 3809 μmol/L, plasma citrulline 747 umol/L,
1 more item
Kobayashi ASH1989 A201,
PubMed: Kobayashi 1990
,
OMIM:var0004
,
PubMed: Diez-Fernandez 2016
,
3 more items
ClinVar-6324
rs121908636
Germline
yes
-
-
-
-
Johan den Dunnen
,
Miriam Erandi Reyna-Fabián
+/.
1
3
c.53C>T
r.53c>u
p.Ser18Leu
-
pathogenic (recessive)
g.133327668C>T
g.130452281C>T
-
-
ASS1_000057
plasma citrulline elevated
PubMed: Kobayashi 1991
,
PubMed: Kobayashi 1994
,
OMIM:var0011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.55T>C
r.(?)
p.(Cys19Arg)
-
pathogenic (recessive)
g.133327670T>C
g.130452283T>C
-
-
ASS1_000058
plasma citrulline 3510 umol/L
PubMed: Gao 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
5
3
c.79C>T
r.(?)
p.(Gln27Ter)
-
pathogenic (recessive)
g.133327694C>T
g.130452307C>T
79T>C
-
ASS1_000156
-
PubMed: Diez-Fernandez 2017
,
PubMed: Silvera-Ruiz 2019
-
-
Germline
-
1/24 case chromosomes
-
-
-
Johan den Dunnen
-/.
1
-
c.99C>T
r.(?)
p.(Ala33=)
-
benign
g.133327714C>T
g.130452327C>T
ASS1(NM_000050.4):c.99C>T (p.A33=)
-
ASS1_000107
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
3
c.100dup
r.(?)
p.(Tyr34LeufsTer29)
-
pathogenic (recessive)
g.133327715dup
g.130452328dup
100dupT
-
ASS1_000157
-
PubMed: Diez-Fernandez 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.102T>G
-
p.Tyr34Ter
-
pathogenic (recessive)
g.133327717T>G
g.130452330T>G
-
-
ASS1_000158
<0.20 no residual enzymatic ASS1 activity combined variants
PubMed: Zielonka 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.106-10T>G
r.(=)
p.(=)
-
benign
g.133329682T>G
g.130454295T>G
ASS1(NM_000050.4):c.106-10T>G
-
ASS1_000108
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
2
4
c.119A>T
r.(?)
p.(Gln40Leu)
-
pathogenic (recessive)
g.133329705A>T
g.130454318A>T
-
-
ASS1_000059
citrulline level 100–200 (109) uM, plasma citrulline 2517 umol/L
PubMed: Dimmock 2008
,
PubMed: Haberle 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.132C>T
r.(?)
p.(Phe44=)
-
likely benign
g.133329718C>T
g.130454331C>T
ASS1(NM_000050.4):c.132C>T (p.F44=)
-
ASS1_000113
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.133G>A
r.(?)
p.(Glu45Lys)
-
VUS
g.133329719G>A
g.130454332G>A
-
-
ASS1_000151
-
PubMed: Xiong 2022
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
4i
c.174+1G>A
r.(106_174del)
p.(Ala36_Lys58del)
-
pathogenic (recessive)
g.133329761G>A
g.130454374G>A
-
-
ASS1_000143
plasma citrulline 961 umol/L; effect on splicing predicted from pSPL3 splicing assay
PubMed: Kimani 2015
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
4i
c.174+1G>T
r.spl
p.?
-
pathogenic (recessive)
g.133329761G>T
g.130454374G>T
IVS4+1G>T
-
ASS1_000061
-
PubMed: Kleijer 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
4i
c.175-1119G>A
c.174_175ins175-1180_175-1112, r.(?)
p.(Lys58fs), p.Val59ProfsTer14
-
pathogenic (recessive)
g.133332669G>A
g.130457282G>A
(Lys58_Val59ins12*), g.4967G>A (Lys58ins69bpX13)
-
ASS1_000147
plasma citrulline 1720 umol/L
PubMed: Diez-Fernandez 2017
,
PubMed: Faghfoury 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
4
4i_5i
c.(174+1_175-1)_(363+1_364-1)del
r.175_363del, r.del
p.del, p.Val59_Lys121
-
pathogenic, pathogenic (recessive)
g.(133329761_133333787)_(133333977_133339497)del
g.(130454374_130458400)_(130458590_130464110)del
ex5 del
-
ASS1_000062
3-4 Kb deletion, no variant 2nd chromosome
Kobayashi ASH1989 A201, {Kobayashi 1990},
PubMed: Kobayashi 1990
,
OMIM:var0006
,
PubMed: Kobayashi 1990
,
2 more items
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
5
c.190G>A
r.(?)
p.(Val64Ile)
-
pathogenic (recessive)
g.133333803G>A
g.130458416G>A
-
-
ASS1_000159
slightly elevated
PubMed: Diez-Fernandez 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
4
5
c.206T>C
-, r.(?)
p.(Val69Ala), p.Val69Ala
-
pathogenic (recessive)
g.133333819T>C
g.130458432T>C
-
-
ASS1_000063
<0.30 no residual enzymatic ASS1 activity combined variants, plasma citrulline 2551 umol/l
PubMed: Diez-Fernandez 2017
,
PubMed: Martin-Hernandez 2014
,
PubMed: Vilaseca 2001
,
1 more item
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
2
-
c.216C>G
-, r.(?)
p.(Phe72Leu), p.Phe72Leu
-
pathogenic (recessive), VUS
g.133333829C>G
g.130458442C>G
-
-
ASS1_000138
citrulline level 200–400 uM, near normal enzymatic ASS1 activity
PubMed: Dimmock 2008
,
PubMed: Zielonka 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
5
c.235T>C
r.(?)
p.(Ser79Pro)
-
pathogenic, pathogenic (recessive)
g.133333848T>C
g.130458461T>C
-
-
ASS1_000064
combination of variants not reported, plasma citrulline 1000 umol/L
PubMed: Diez-Fernandez 2017
,
PubMed: Engel 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
14
4, 5
c.256C>T
-, r.(?), r.256c>u
p.(Arg86Cys), p.Arg86Cys
ACMG
pathogenic, pathogenic (recessive)
g.133333869C>T
g.130458482C>T
-
-
ASS1_000065
<0.20 no residual enzymatic ASS1 activity, plasma citrulline 1437 umol/L
PubMed: Diez-Fernandez 2017
,
PubMed: Kobayashi 1991
,
OMIM:var0012
,
PubMed: Martin-Hernandez 2014
,
2 more items
6332,
ClinVar-6332
rs121908644
Germline
yes
-
-
-
-
Johan den Dunnen
,
Miriam Erandi Reyna-Fabián
+/., +?/.
3
5
c.257G>A
r.(?), r.257g>a
p.(Arg86His), p.Arg86His
-
likely pathogenic (recessive), pathogenic (recessive)
g.133333870G>A
g.130458483G>A
-
-
ASS1_000066
plasma citrulline 1383 μmol/L, plasma citrulline 903 μmol/L,
1 more item
PubMed: Diez-Fernandez 2017
,
PubMed: Gao 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.262C>A
r.(?)
p.(Leu88Ile)
-
pathogenic (recessive)
g.133333875C>A
g.130458488C>A
262C>G (L88I)
-
ASS1_000139
citrulline level <1000 uM; citruline enzyme activity 0 nM/min/mg protein
PubMed: Dimmock 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
4
-
c.271A>C
-, r.(?)
p.(Thr91Pro), p.Thr91Pro
-
NA, pathogenic (recessive)
g.133333884A>C
g.130458497A>C
T91P
-
ASS1_000127
<0.05 no residual enzymatic ASS1 activity, plasma citrulline 2417 μmol/L,
1 more item
PubMed: Diez-Fernandez 2016
,
PubMed: Miller 2014
,
PubMed: Zielonka 2019
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.271A>G
-, r.(?)
p.(Thr91Ala), p.Thr91Ala
-
pathogenic (recessive)
g.133333884A>G
g.130458497A>G
-
-
ASS1_000125
<0.50 no residual enzymatic ASS1 activity combined variants
PubMed: Martin-Hernandez 2014
,
PubMed: Zielonka 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.282C>T
r.(?)
p.(Ala94=)
-
likely benign
g.133333895C>T
g.130458508C>T
ASS1(NM_000050.4):c.282C>T (p.A94=)
-
ASS1_000114
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.285G>C
-
p.Arg95Ser
-
NA
g.133333898G>C
g.130458511G>C
R95S
-
ASS1_000140
cDNA expression cloning shows no argininosuccinate synthetase enzyme activity
PubMed: Diez-Fernandez 2016
-
-
In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
1
5
c.285_286delinsTT
r.(?)
p.(Arg95_Pro96delinsSerSer)
-
pathogenic (recessive)
g.133333898_133333899delinsTT
g.130458511_130458512delinsTT
[285G>T;286C>T] [R95S;P96S]
-
ASS1_000128
plasma citrulline 1718 μmol/L (ref<50)
PubMed: Gao 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.286C>T
-
p.Pro96Ser
-
NA
g.133333899C>T
g.130458512C>T
P96S
-
ASS1_000141
cDNA expression cloning shows 0.35 argininosuccinate synthetase enzyme activity
PubMed: Diez-Fernandez 2016
-
-
In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/., +?/.
2
5
c.287C>A
-, r.(?)
p.(Pro96His), p.Pro96His
-
NA, pathogenic (recessive)
g.133333900C>A
g.130458513C>A
P96H
-
ASS1_000069
cDNA expression cloning shows 0.25 argininosuccinate synthetase enzyme activity
PubMed: Diez-Fernandez 2016
,
PubMed: Engel 2009
,
PubMed: Diez-Fernandez 2016
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
3
-
c.287C>T
-, r.(?)
p.(Pro96Leu), p.Pro96Leu
-
NA, pathogenic (recessive)
g.133333900C>T
g.130458513C>T
P96L
-
ASS1_000130
cDNA expression cloning shows no argininosuccinate synthetase enzyme activity,
1 more item
PubMed: Diez-Fernandez 2016
,
PubMed: Faghfoury 2011
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
2
5
c.291C>A
-, r.(?)
p.(Cys97Ter), p.Cys97Ter
-
pathogenic (recessive)
g.133333904C>A
g.130458517C>A
-
-
ASS1_000160
no residual enzymatic ASS1 activity, plasma citrulline >4000 umol/L
PubMed: Diez-Fernandez 2017
,
PubMed: Zielonka 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
5
c.298C>T
r.(?)
p.(Arg100Cys)
-
pathogenic (recessive)
g.133333911C>T
g.130458524C>T
-
-
ASS1_000161
plasma citrulline 200 umol/L
PubMed: Diez-Fernandez 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
5
c.299del
r.(?)
p.(Arg100Profs*40)
-
pathogenic (recessive)
g.133333912del
g.130458525del
-
-
ASS1_000070
plasma citrulline 3248 μmol/L
PubMed: Gao 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
5
c.299G>A
-, r.(?)
p.(Arg100His), p.Arg100His
-
pathogenic (recessive)
g.133333912G>A
g.130458525G>A
-
-
ASS1_000162
<0.80 no residual enzymatic ASS1 activity combined variants
PubMed: Diez-Fernandez 2017
,
PubMed: Zielonka 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
5
c.304del
r.(?)
p.(Gln102LysfsTer38)
-
pathogenic (recessive)
g.133333917del
g.130458530del
304delC
-
ASS1_000163
plasma citrulline 1800 umol/L
PubMed: Diez-Fernandez 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., -/., -?/., ?/.
8
5
c.323G>T
r.(?)
p.(Arg108Leu)
-
benign, likely benign, pathogenic (recessive), VUS
g.133333936G>T
g.130458549G>T
ASS1(NM_000050.4):c.323G>T (p.R108L)
-
ASS1_000071
no variant 2nd chromosome;plasma citrulline 914 umol/l, plasma citrulline 736 umol/L,
2 more items
PubMed: Diez-Fernandez 2017
,
PubMed: Haberle 2002
,
OMIM:var0014
,
1 more item
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Gerard C.P. Schaafsma
,
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
+/.
1
5
c.332C>A
r.(?)
p.(Ala111Asp)
-
pathogenic (recessive)
g.133333945C>A
g.130458558C>A
-
-
ASS1_000164
-
PubMed: Diez-Fernandez 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.333C>T
r.(?)
p.(Ala111=)
-
likely benign
g.133333946C>T
g.130458559C>T
ASS1(NM_000050.4):c.333C>T (p.A111=)
-
ASS1_000115
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/.
6
5
c.349G>A
-, r.(?)
p.(Gly117Ser), p.Gly117Ser
-
likely pathogenic (recessive), NA, pathogenic (recessive)
g.133333962G>A
g.130458575G>A
G117S
-
ASS1_000073
<0.20 no residual enzymatic ASS1 activity, plasma citrulline 1218 μmol/L,
4 more items
PubMed: Diez-Fernandez 2016
,
PubMed: Gao 2003
,
PubMed: Zielonka 2019
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
1
5
c.349G>T
r.(?)
p.(Gly117Cys)
-
pathogenic (recessive)
g.133333962G>T
g.130458575G>T
-
-
ASS1_000165
-
PubMed: Diez-Fernandez 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
4
5
c.350G>A
r.(?)
p.(Gly117Asp)
-
likely pathogenic (recessive), pathogenic (recessive)
g.133333963G>A
g.130458576G>A
-
-
ASS1_000074
plasma citrulline 1504 μmol/L, plasma citrulline 1785 umol/l
PubMed: Gao 2003
,
PubMed: Moarefian 2022
,
PubMed: Vilaseca 2001
,
PubMed: Martin-Hernandez 2014
-
-
Germline
yes
1/10 families
-
-
-
Johan den Dunnen
-/.
1
4
c.351C>T
r.(?)
p.(=)
-
benign
g.133333964C>T
g.130458577C>T
-
-
ASS1_000060
-
PubMed: Engel 2009
-
rs35772983
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/., ?/.
12
5
c.352G>A
-, r.(?), r.352g>a
p.(Ala118Thr), p.Ala118Thr
-
likely pathogenic (recessive), NA, pathogenic (recessive), VUS
g.133333965G>A
g.130458578G>A
A118T, ASS1(NM_000050.4):c.352G>A (p.A118T)
-
ASS1_000075
<0.20 no residual enzymatic ASS1 activity combined variants, plasma citrulline 1000 umol/L,
8 more items
PubMed: Diez-Fernandez 2016
,
PubMed: Diez-Fernandez 2017
,
PubMed: Engel 2009
,
PubMed: Gao 2003
,
4 more items
-
-
CLASSIFICATION record, Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
+/., ?/.
4
5
c.356C>T
-, r.(?)
p.(Thr119Ile), p.Thr119Ile
-
NA, pathogenic (recessive), VUS
g.133333969C>T
g.130458582C>T
T119I
-
ASS1_000076
cDNA expression cloning shows no argininosuccinate synthetase enzyme activity,
3 more items
PubMed: Diez-Fernandez 2016
,
PubMed: Diez-Fernandez 2017
,
1 more item
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
-?/., ?/.
2
-
c.364-6T>C
r.(=)
p.(=)
-
likely benign, VUS
g.133339492T>C
g.130464105T>C
ASS1(NM_000050.4):c.364-6T>C (p.(=))
-
ASS1_000102
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_AMC
+/.
1
5i
c.364-2A>G
r.spl
p.?
-
pathogenic (recessive)
g.133339496A>G
g.130464109A>G
-
-
ASS1_000166
-
PubMed: Diez-Fernandez 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
5i_6i
c.(363+1_364-1)_(420+1_421-1)del
r.del
p.del
-
pathogenic
g.(133333977_133339497)_(133339555_133342111)del
-
ex6 del
-
ASS1_000077
2-3 Kb deletion
Kobayashi ASH1989 A201,
PubMed: Kobayashi 1995
,
OMIM:var0001
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
4
6
c.370G>A
-, r.(?)
p.(Asp124Asn), p.Asp124Asn
-
likely pathogenic (recessive), NA, pathogenic (recessive)
g.133339504G>A
g.130464117G>A
D124N
-
ASS1_000078
<0.30 no residual enzymatic ASS1 activity combined variants,
1 more item
PubMed: Diez-Fernandez 2016
,
PubMed: Kleijer 2006
,
PubMed: Zielonka 2019
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.372T>A
-
p.Asp124Glu
-
pathogenic (recessive)
g.133339506T>A
g.130464119T>A
-
-
ASS1_000167
<0.60 no residual enzymatic ASS1 activity combined variants
PubMed: Zielonka 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
-
c.379C>G
r.(?)
p.(Arg127Gly)
-
pathogenic (recessive)
g.133339513C>G
g.130464126C>G
-
-
ASS1_000168
plasma citrulline 1447 umol/L, plasma citrulline 969 umol/L
PubMed: Lee 2013
,
PubMed: Woo 2013
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
6
c.379C>T
-, r.(?)
p.(Arg127Trp), p.Arg127Trp
-
NA, pathogenic
g.133339513C>T
g.130464126C>T
R127W
-
ASS1_000079
cDNA expression cloning shows no argininosuccinate synthetase enzyme activity,
1 more item
PubMed: Diez-Fernandez 2016
,
PubMed: Engel 2009
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
8
6
c.380G>A
-, r.(?)
p.(Arg127Gln), p.Arg127Gln
-
NA, pathogenic, pathogenic (recessive)
g.133339514G>A
g.130464127G>A
R127Q
-
ASS1_000080
<0.20 no residual enzymatic ASS1 activity, combination of variants not reported,
3 more items
PubMed: Diez-Fernandez 2016
,
PubMed: Diez-Fernandez 2017
,
PubMed: Engel 2009
,
PubMed: Haberle 2003
,
4 more items
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/., ?/.
2
-
c.380G>T
-, r.(?)
p.(Arg127Leu), p.Arg127Leu
-
NA, VUS
g.133339514G>T
g.130464127G>T
R127L
-
ASS1_000134
cDNA expression cloning shows no argininosuccinate synthetase enzyme activity
PubMed: Diez-Fernandez 2016
,
PubMed: Miller 2014
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
2
6
c.412C>T
r.(?)
p.(Gln138*), p.(Gln138Ter)
-
pathogenic, pathogenic (recessive)
g.133339546C>T
g.130464159C>T
ASS1(NM_054012.3):c.412C>T (p.(Gln138*))
-
ASS1_000169
VKGL data sharing initiative Nederland
PubMed: Diez-Fernandez 2017
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Leiden
-/.
1
-
c.421-19C>T
r.(=)
p.(=)
-
benign
g.133342093C>T
g.130466706C>T
ASS1(NM_000050.4):c.421-19C>T
-
ASS1_000109
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
52
6i
c.421-2A>G
r.(421_495del), r.421_495del, r.spl, r.spl?
p.(Val141_Lys165del), p.?, p.del, p.Val141_Lys165del
-
pathogenic, pathogenic (recessive)
g.133342110A>G
g.130466723A>G
ASS1(NM_000050.4):c.421-2A>G, del ex7, IVS6-2A>G
-
ASS1_000081
patient chromosomes, plasma citrulline 1376 umol/L, plasma citrulline 1836 umol/L,
17 more items
Kobayashi ASH1989 A201,
PubMed: Kobayashi 1995
,
OMIM:var0003
,
PubMed: Ban 2001
,
PubMed: Gao 2003
,
11 more items
-
-
CLASSIFICATION record, Germline
-
19/33
MspI-
-
-
Johan den Dunnen
,
VKGL-NL_Groningen
+/.
1
7
c.422T>G
r.(422u>g,421_495del)
p.(Val141Gly,Val141_Lys165del)
-
pathogenic (recessive)
g.133342113T>G
g.130466726T>G
-
-
ASS1_000144
plasma citrulline 961 umol/L; effect on splicing predicted from pSPL3 splicing assay
PubMed: Kimani 2015
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.428C>A
r.(?)
p.(Ala143Asp)
-
VUS
g.133342119C>A
-
-
-
ASS1_000120
-
-
-
-
Germline/De novo (untested)
-
-
-
-
-
Gemeinschaftspraxis für Humangenetik Dresden
+?/.
2
7
c.437G>A
r.(?)
p.(Arg146Lys)
-
likely pathogenic
g.133342128G>A
g.130466741G>A
-
-
ASS1_000104
-
PubMed: Navarrete 2019
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
,
Belen Perez
+/.
1
-
c.440T>C
r.(?)
p.(Met147Thr)
-
pathogenic (recessive)
g.133342131T>C
g.130466744T>C
-
-
ASS1_000209
-
PubMed: Moarefian 2022
-
-
Germline
yes
1/10 families
-
-
-
Johan den Dunnen
+/.
2
7
c.450_451del
r.(?)
p.(Phe150Leufs*9), p.(Phe150LeufsTer9)
-
pathogenic, pathogenic (recessive)
g.133342141_133342142del
g.130466754_130466755del
450_451delCT, c.450_451delCT
-
ASS1_000082
combination of variants not reported, unknown variant 2nd chromosome; plasma citrulline 1778 umol/L
PubMed: Diez-Fernandez 2017
,
PubMed: Engel 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
7
c.460_467del
r.(?)
p.(Phe154Profs*3), p.(Phe154ProfsTer3)
-
pathogenic, pathogenic (recessive)
g.133342151_133342158del
g.130466764_130466771del
459_466del
-
ASS1_000083
combination of variants not reported
PubMed: Diez-Fernandez 2017
,
PubMed: Engel 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.467G>A
-
p.Gly156Asp
-
pathogenic (recessive)
g.133342158G>A
g.130466771G>A
-
-
ASS1_000170
<0.20 no residual enzymatic ASS1 activity
PubMed: Zielonka 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
7
c.469C>A
r.(?)
p.(Arg157Ser)
-
pathogenic (recessive)
g.133342160C>A
g.130466773C>A
-
-
ASS1_000171
plasma citrulline 2877 umol/L
PubMed: Diez-Fernandez 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
8
7
c.469C>T
-, r.(?)
p.(Arg157Cys), p.Arg157Cys
-
NA, pathogenic (recessive)
g.133342160C>T
g.130466773C>T
R157C
-
ASS1_000084
<0.10 no residual enzymatic ASS1 activity combined variants, plasma citrulline 200 umol/L,
3 more items
PubMed: Diez-Fernandez 2016
,
PubMed: Diez-Fernandez 2017
,
PubMed: Gao 2003
,
PubMed: Miller 2014
,
1 more item
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/., ?/.
20
7
c.470G>A
-, r.(?), r.470g>a
p.(Arg157His), p.Arg157His
-
NA, pathogenic (recessive), VUS
g.133342161G>A
g.130466774G>A
R157H
-
ASS1_000085
<0.05 no residual enzymatic ASS1 activity, plasma citrulline 200 umol/L, plasma citrulline high,
6 more items
Kobayashi ASH1989 A201,
PubMed: Kobayashi 1990
,
OMIM:var0005
,
PubMed: Diez-Fernandez 2016
,
5 more items
-
rs121908637
Germline, In vitro (cloned)
yes
1/10 families, 1/2795 individuals
-
-
-
Johan den Dunnen
,
Mohammed Faruq
+/.
4
7
c.479T>C
r.(?)
p.(Leu160Pro)
-
pathogenic, pathogenic (recessive)
g.133342170T>C
g.130466783T>C
-
-
ASS1_000086
combination of variants not reported
PubMed: Diez-Fernandez 2017
,
PubMed: Engel 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
7
c.489C>A
r.(?)
p.(Tyr163Ter)
-
pathogenic (recessive)
g.133342180C>A
g.130466793C>A
-
-
ASS1_000172
-
PubMed: Diez-Fernandez 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.489C>T
r.(?)
p.(Tyr163=)
-
likely benign
g.133342180C>T
-
ASS1(NM_000050.4):c.489C>T (p.Y163=)
-
ASS1_000118
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
4
7
c.490G>C
r.(?)
p.(Ala164Pro)
-
pathogenic (recessive)
g.133342181G>C
g.130466794G>C
-
-
ASS1_000173
plasma citrulline 1789 umol/L, plasma citrulline 1975 umol/L, plasma citrulline 2247 umol/L,
1 more item
PubMed: Diez-Fernandez 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
7i
c.495+1G>T
r.421_495del, r.spl
p.(Val141_Lys165del), p.Val141_Lys165del
-
pathogenic, pathogenic (recessive)
g.133342187G>T
g.130466800G>T
-
-
ASS1_000087
combination of variants not reported
PubMed: Diez-Fernandez 2017
,
PubMed: Engel 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
2
-
c.496-1766_597+732delinsGC
r.(496_597del), r.496_597delinsGC
p.?
-
pathogenic, VUS
g.133344455_133345489delinsGC, g.133344455_133347634delinsGC
g.130469068_130472247delinsGC
-
-
ASS1_000105
1 more item
PubMed: Navarrete 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Belen Perez
+/.
3
7i_8
c.496-21_515del
r.(?), r.spl
p.(Gln166GlyfsTer11), p.del
-
pathogenic, pathogenic (recessive)
g.133346200_133346240del
g.130470813_130470853del
-
-
ASS1_000088
combination of variants not reported
PubMed: Diez-Fernandez 2017
,
PubMed: Engel 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.496-4G>C
r.spl?
p.?
-
likely benign
g.133346217G>C
-
ASS1(NM_054012.4):c.496-4G>C
-
ASS1_000122
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
7i
c.496-2A>G
r.spl
p.?
-
pathogenic (recessive)
g.133346219A>G
g.130470832A>G
-
-
ASS1_000174
-
PubMed: Diez-Fernandez 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.499_500del
-
p.His167ArgfsTer16
-
pathogenic (recessive)
g.133346225_133346226del
g.130470838_130470839del
-
-
ASS1_000175
<0.10 no residual enzymatic ASS1 activity combined variants
PubMed: Zielonka 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
4
8
c.501C>T
r.(?)
p.(=), p.(His167=)
-
benign
g.133346226C>T
g.130470839C>T
ASS1(NM_000050.4):c.501C>T (p.H167=), His167His
-
ASS1_000001
VKGL data sharing initiative Nederland
PubMed: Diez-Fernandez 2017
,
PubMed: Engel 2009
,
PubMed: Mitchell 2009
-
rs10901072
CLASSIFICATION record, Germline
-
15/94
-
-
-
Johan den Dunnen
,
VKGL-NL_AMC
+/., +?/.
33
8
c.535T>C
-, r.(?)
p.(Trp179Arg), p.Trp179Arg
-
likely pathogenic (recessive), pathogenic (recessive)
g.133346260T>C
g.130470873T>C
-
-
ASS1_000089
<0.10 no residual enzymatic ASS1 activity combined variants, citrulline level 300–1000 uM,
19 more items
PubMed: Diez-Fernandez 2017
,
PubMed: Dimmock 2008
,
PubMed: Gao 2003
,
PubMed: Haberle 2003
,
2 more items
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
5
-
c.539G>A
-, r.(?), r.539g>a
p.(Ser180Asn), p.Ser180Asn
-
NA, pathogenic (recessive)
g.133346264G>A
g.130470877G>A
S180N
-
ASS1_000091
<0.30 no residual enzymatic ASS1 activity combined variants, citrulline level 100–200 (109) uM,
3 more items
Kobayashi ASH1989 A201,
PubMed: Kobayashi 1990
,
PubMed: Diez-Fernandez 2016
,
PubMed: Dimmock 2008
,
1 more item
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/., ?/.
2
-
c.539G>T
-, r.(?)
p.(Ser180Ile), p.Ser180Ile
-
NA, VUS
g.133346264G>T
g.130470877G>T
S180I
-
ASS1_000133
cDNA expression cloning shows no argininosuccinate synthetase enzyme activity
PubMed: Diez-Fernandez 2016
,
PubMed: Miller 2014
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
1
8
c.547_561delinsAGAGAACCTCATGCACAT
r.(?)
p.(Glu183_Met186delinsArgGluProHisAla)
-
pathogenic (recessive)
g.133346272_133346286delinsAGAGAACCTCATGCACAT
g.130470885_130470899delinsAGAGAACCTCATGCACAT
[546_547insA;562_563dupAT]
-
ASS1_000176
-
PubMed: Diez-Fernandez 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
8
c.552C>A
r.(?)
p.(Asn184Lys)
-
pathogenic (recessive)
g.133346277C>A
g.130470890C>A
-
-
ASS1_000153
mild citrullinemia
PubMed: Diez-Fernandez 2017
,
PubMed: Xiong 2022
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.556A>G
-
p.Met186Val
-
pathogenic (recessive)
g.133346281A>G
g.130470894A>G
-
-
ASS1_000177
<0.30 no residual enzymatic ASS1 activity combined variants
PubMed: Zielonka 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.557T>G
r.(?)
p.(Met186Arg)
-
pathogenic (recessive)
g.133346282T>G
g.130470895T>G
-
-
ASS1_000126
-
PubMed: Martin-Hernandez 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
8
c.561C>T
r.(?)
p.(=)
-
benign
g.133346286C>T
g.130470899C>T
-
-
ASS1_000100
-
PubMed: Engel 2009
-
rs2297599
Germline
-
-
-
-
-
Johan den Dunnen
-/.
2
8
c.564C>T
r.(?)
p.(=)
-
benign
g.133346289C>T
g.130470902C>T
Ile188Ile
-
ASS1_000002
-
PubMed: Mitchell 2009
-
rs2297599
Germline
-
1/94
-
-
-
Johan den Dunnen
-?/.
1
8i
c.566+80G>A
r.566_567=
p.=
-
benign
g.133346371G>A
g.130470984G>A
-
-
ASS1_000208
-
PubMed: Sun 2018
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.567-4G>A
r.spl?
p.?
-
likely benign
g.133346868G>A
-
ASS1(NM_000050.4):c.567-4G>A
-
ASS1_000116
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.568T>G
r.(?)
p.(Tyr190Asp)
-
pathogenic (recessive)
g.133346873T>G
g.130471486T>G
-
-
ASS1_000092
-
PubMed: Haberle 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
9
9
c.571G>A
-, r.(?), r.579g>a
p.(Glu191Lys), p.Glu191Lys
-
pathogenic (recessive)
g.133346876G>A
g.130471489G>A
E191K
-
ASS1_000093
<0.10 no residual enzymatic ASS1 activity, plasma citrulline 1172 umol/L,
4 more items
PubMed: Diez-Fernandez 2017
,
PubMed: Gao 2003
,
PubMed: Haberle 2004
,
PubMed: Zielonka 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
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