Global Variome shared LOVD
OTC (ornithine carbamoyltransferase)
LOVD v.3.0 Build 30b [
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Curator:
Johan den Dunnen
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Unique variants in the OTC gene
The variants shown are described using the NM_000531.5 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.
442 entries on 5 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
+/.
3
_1_10_
c.-214_*368{0}
r.0
p.0
-
pathogenic
g.(?_g.38211736)_(38280703_?)del
g.(?_38352483)_(38421450_?)del
del ex1-10, delOTC gene >1.6 Mb
-
CYBB_000005
-
PubMed: Climent 1999
,
PubMed: Martin-Hernandez 2014
,
PubMed: Martin-Hernandez 2014
,
1 more item
-
-
Germline
-
5/24 case chromosomes
-
-
-
Johan den Dunnen
+/.
1
_1_10_
c.[-214_(1005+1_1006-1){2};(1005+1_1006-1)*368{0}]
r.?
p.?
-
pathogenic
g.[(?_g.38211736)_(38271253_38280275)dup;(38271253_38280275)_(38280703_?)del]
g.[(?_38352483)_(38412000_38421022)dup;(38412000_38421022)-(38421450_?)del]
dup ex1-9/del ex10
-
OTC_000442
-
PubMed: Silvera-Ruiz 2019
-
-
Germline
-
1/24 case chromosomes
-
-
-
Johan den Dunnen
+/.
1
_1_10_
c.-2802714_*618720del
r.0
p.0
-
pathogenic (recessive)
g.35409236_38899055del
-
hg17 35168893-38655271del
-
CYBB_000481
deletion incl. XK, CYBB, RPGR, OTC, TSPAN7
PubMed: Yamada 2010
-
-
De novo
-
-
-
-
-
Johan den Dunnen
-/., ?/.
12
1
c.-515_-512del
r.(?)
p.(?)
-
benign, VUS
g.38211435_38211438del
g.38352182_38352185del
-512insTCTT
-
OTC_000257
-
PubMed: Azevedo 2003
-
rs57752938
,
rs5917572
Germline
-
1/57, 13/103, 13/57, 16/103, 18/36, 2/36, 21/57, 3/103, 3/57, 38/103, 7/36, 7/57
MboII
-
-
Johan den Dunnen
-/., ?/.
14
1
c.-441A>G
r.(?)
p.(?)
-
benign, VUS
g.38211509A>G
g.38352256A>G
(-441G>A)
-
OTC_000256
-
PubMed: Azevedo 2003
-
rs5917572
Germline
-
1/36, 1/57, 13/103, 13/57, 16/103, 18/36, 2/36, 21/57, 3/103, 3/57, 38/103, 7/36, 7/57
-
-
-
Johan den Dunnen
+/.
1
1
c.-366A>G
r.(=)
p.(=)
-
pathogenic
g.38211584A>G
g.38352331A>G
-
-
OTC_000400
not in 300 control chromosomes
-
-
-
Germline
-
-
-
-
-
Lenka Dvořáková
?/.
4
1
c.-365G>A
r.(?)
p.(?)
-
VUS
g.38211585G>A
g.38352332G>A
-
-
OTC_000255
-
PubMed: Azevedo 2003
-
rs5963030
Germline
-
1/57, 16/103, 2/36, 7/57
TseI;Fnu4HI;BbvI
-
-
Johan den Dunnen
-/.
10
1
c.-359A>G
r.(?)
p.(?)
-
benign
g.38211591A>G
g.38352338A>G
-
-
OTC_000254
1 more item
PubMed: Azevedo 2003
-
rs5917573
Germline
-
1/36, 1/57, 2/36, 2/57, 24/103, 3/57, 4/103, 6/36, 6/57
-
-
-
Johan den Dunnen
+/.
1
_1_3i
c.(?_-1)_(298+1_299-1)del
r.0?
p.0?
-
pathogenic
g.(?_38211949)_(38229131_38240594)del
-
-
-
OTC_000098
deletion exons 1-3
PubMed: Tuchman 1997
-
-
Germline
-
-
-
-
-
Bert Bakker
+/.
1
_1_8i
c.(?_-1)_(867+1_868-1)del
r.0
p.0
-
pathogenic
g.(?_38211949)_(38268279_38271114)del
-
-
-
OTC_000357
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
+/.
1
_1_9i
c.(?_-1)_(1005+1_1006-1)del
r.0
p.0
-
pathogenic
g.(?_38211949)_(38271253_38280275)del
-
-
-
OTC_000355
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
3
_1_10_
c.(?_-1)_(*1_?)del
r.0
p.0
-
VUS
g.(?_38211949)_(38280336_?)del
-
-
-
OTC_000210
deletion entire OTC gene
PubMed: Suess 1992
,
PubMed: Tuchman 2002
,
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
1
c.1A>G
r.(?)
p.0?
-
VUS
g.38211950A>G
g.38352697A>G
-
-
OTC_000099
-
PubMed: Oppliger Leibundgut 1997
-
-
Germline
-
-
MboII;SfaNI
-
-
Bert Bakker
?/.
1
1
c.1A>T
r.(?)
p.(Met1Leu)
-
VUS
g.38211950A>T
g.38352697A>T
-
-
OTC_000361
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
1
c.2T>C
r.(?)
p.(Met1Thr)
-
VUS
g.38211951T>C
g.38352698T>C
-
-
OTC_000365
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
1
c.3G>A
r.(?)
p.0?
-
VUS
g.38211952G>A
g.38352699G>A
-
-
OTC_000211
-
PubMed: Climent and Rubio 2002
-
-
Germline
-
-
SfaNI;HpyCH4III
-
-
Bert Bakker
?/.
1
1
c.26T>G
r.(?)
p.(Leu9X)
-
VUS
g.38211975T>G
g.38352722T>G
c.25T>G
-
OTC_000264
1 more item
PubMed: Kim 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
1
c.29dup
r.(?)
p.(Asn10LysfsTer6)
-
VUS
g.38211978dup
g.38352725dup
-
-
OTC_000375
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
1
c.29_32del
r.(?)
p.(Asn10MetfsTer27)
-
VUS
g.38211978_38211981del
g.38352725_38352728del
-
-
OTC_000370
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
1
c.34G>A
r.(?)
p.(Ala12Thr)
-
VUS
g.38211983G>A
g.38352730G>A
-
-
OTC_000260
-
Tanaka 2005
-
-
Germline
-
-
TseI;CviRI;Fnu4HI;BbvI
-
-
Bert Bakker
?/.
1
1
c.42del
r.(?)
p.(Phe14LeufsTer24)
-
VUS
g.38211991del
g.38352738del
-
-
OTC_000379
Leader sequence; OTC activity 23%
Hwu 2003
-
-
Germline
-
-
-
-
-
Bert Bakker
-/.
2
1
c.46A>G
r.(?)
p.(Asn16Asp ), p.(Asn16Asp)
-
benign
g.38211995A>G
g.38352742A>G
-
-
OTC_000390
100 individuals tested
PubMed: Mitchell 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
1
c.53del
r.(?)
p.(His18ProfsTer20)
-
VUS
g.38212002del
g.38352749del
-
-
OTC_000212
-
PubMed: Tuchman 2002
-
-
Germline
-
-
AvaII
-
-
Bert Bakker
+/., ?/.
2
1
c.67C>T
r.(?)
p.(Arg23Ter), p.(Arg23X)
-
pathogenic, VUS
g.38212016C>T
g.38352763C>T
-
-
OTC_000011
Leader sequence
Grompe 1991,
PubMed: Martin-Hernandez 2014
-
-
Germline
-
-
TaqI;AsuII
-
-
Johan den Dunnen
,
Bert Bakker
-?/.
2
-
c.76C>T
r.(?)
p.(Arg26Trp)
-
likely benign
g.38212025C>T
g.38352772C>T
OTC(NM_000531.5):c.76C>T (p.R26W, p.(Arg26Trp))
-
OTC_000413
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
+/., ?/.
3
1
c.77G>A
r.(?), r.(spl?)
p.(Arg26Gln)
-
pathogenic, VUS
g.38212026G>A
g.38352773G>A
-
-
OTC_000003
1 heterozygous, no homozygous;
Clinindb (India)
, OTC activity 0%,
1 more item
Grompe 1989,
PubMed: Kim 2006
,
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs68031618
Germline
-
1/2795 individuals
-
-
-
Bert Bakker
,
Mohammed Faruq
?/.
2
1
c.77G>C
r.(spl?)
p.(Arg26Pro)
-
VUS
g.38212026G>C
g.38352773G>C
-
-
OTC_000270
Leader sequence, CpG dinucleotide, possible donor splice site error,
1 more item
PubMed: Kim 2006
,
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
1i
c.77+1G>A
r.spl?
p.(?)
-
VUS
g.38212027G>A
g.38352774G>A
-
-
OTC_000298
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
1i
c.77+1G>T
r.spl
p.(?)
-
VUS
g.38212027G>T
g.38352774G>T
c.IVS1+1G>T
-
OTC_000100
-
PubMed: Tuchman 1997
-
-
Germline
-
-
MseI
-
-
Bert Bakker
?/.
1
1i
c.77+2dup
r.spl?
p.?
-
VUS
g.38212028dup
g.38352775dup
-
-
OTC_000302
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
+?/.
1
1i
c.77+3_77+6del
r.spl
p.?
-
likely pathogenic
g.38212029_38212032del
g.38352776_38352779del
c.IVS1+3_6delAAGT
-
OTC_000213
-
PubMed: Tuchman 2002
-
-
Germline
-
-
HpHI
-
-
Bert Bakker
?/.
1
1i
c.77+4A>C
r.spl
p.(?)
-
VUS
g.38212030A>C
g.38352777A>C
c.IVS1+4A>C
-
OTC_000078
OTC activity 0%
Hoshide 1996
-
-
Germline
-
-
TspRI
-
-
Bert Bakker
?/.
1
1i
c.77+5G>A
r.spl
p.(?)
-
VUS
g.38212031G>A
g.38352778G>A
c.IVS1+5G>A
-
OTC_000101
-
PubMed: Tuchman 1997
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
-
c.77+437C>A
r.(=)
p.(=)
-
VUS
g.38212463C>A
g.38353210C>A
-
-
OTC_000401
-
-
-
-
Germline
-
-
-
-
-
Yu Sun
+/.
1
1i
c.77+6024_216+474del
r.[78_298del, 78_386del]
p.del
-
pathogenic
g.38218050_38227156del
g.38358797_38367903del
-
-
OTC_000394
9095 bpdeletion (incl. exon 2)
PubMed: Engel 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
1i
c.78-3C>G
r.77_78insag
p.fsX
-
VUS
g.38226541C>G
g.38367288C>G
-
-
OTC_000312
OTC activity below 5%
Bisanzi 2002
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
1i
c.78-1G>C
r.spl?
p.(?)
-
VUS
g.38226543G>C
g.38367290G>C
-
-
OTC_000314
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
+/.
1
1i_4i
c.(77+1_78-1)_(386+1_387-1)del
r.?
p.?
-
pathogenic
g.(38212027_38226543)_(38240683_38260527)del
-
-
-
OTC_000358
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
-?/.
1
-
c.83G>A
r.(?)
p.(Gly28Glu)
-
likely benign
g.38226549G>A
g.38367296G>A
OTC(NM_000531.5):c.83G>A (p.G28E)
-
OTC_000404
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
2
c.94C>T
r.(?)
p.(Gln32X)
-
VUS
g.38226560C>T
g.38367307C>T
-
-
OTC_000108
-
PubMed: Oppliger Leibundgut 1997
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
2
c.106C>T
r.(?)
p.(Gln36X)
-
VUS
g.38226572C>T
g.38367319C>T
-
-
OTC_000181
-
PubMed: Genet 2000
-
-
Germline
-
-
Fnu4HI;CviRI
-
-
Bert Bakker
?/.
1
2
c.115G>T
r.(?)
p.(Gly39Cys)
-
VUS
g.38226581G>T
g.38367328G>T
-
-
OTC_000140
-
Calvas 1998
-
-
Germline
-
-
HaeIII;Sau96I
-
-
Bert Bakker
?/.
1
2
c.118C>T
r.(?)
p.(Arg40Cys)
-
VUS
g.38226584C>T
g.38367331C>T
-
-
OTC_000060
-
Oppliger Leibundgut 1995
-
-
Germline
-
-
HaeIII
-
-
Bert Bakker
+/., +?/., ?/.
6
2
c.119G>A
r.(?)
p.(Arg40His)
-
likely pathogenic, pathogenic, VUS
g.38226585G>A
g.38367332G>A
-
-
OTC_000046
OTC activity 6%
PubMed: Martin-Hernandez 2014
,
PubMed: Tuchman 1994b
-
-
Germline
-
-
MaeIII;Tsp45I
-
-
Johan den Dunnen
,
Bert Bakker
,
Sha Hong
?/.
1
2
c.122A>G
r.(?)
p.(Asp41Gly)
-
VUS
g.38226588A>G
g.38367335A>G
-
-
OTC_000334
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
2
c.127C>T
r.(?)
p.(Leu43Phe)
-
VUS
g.38226593C>T
g.38367340C>T
-
-
OTC_000102
-
PubMed: Oppliger Leibundgut 1997
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
-
c.130A>G
r.(?)
p.(Thr44Ala)
-
VUS
g.38226596A>G
g.38367343A>G
OTC(NM_000531.5):c.130A>G (p.T44A)
-
OTC_000411
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
2
c.131C>T
r.(?)
p.(Thr44IIe)
-
VUS
g.38226597C>T
g.38367344C>T
-
-
OTC_000079
OTC activity 0, Serum Ammonia 389, Urine orotic acid 238, Serum citrulline 28.0
PubMed: Yoo 1996
;
PubMed: Kim 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
2
c.133C>G
r.(?)
p.(Leu45Val)
-
VUS
g.38226599C>G
g.38367346C>G
-
-
OTC_000141
-
PubMed: Tuchman 1998
-
-
Germline
-
-
HpyCH4III;TspRI
-
-
Bert Bakker
?/.
1
2
c.134T>C
r.(?)
p.(Leu45Pro)
-
VUS
g.38226600T>C
g.38367347T>C
-
-
OTC_000004
-
Grompe 1989
-
-
Germline
-
-
-
-
-
Bert Bakker
-/.
2
2
c.134T>G
r.(?)
p.(Leu45Arg ), p.(Leu45Arg)
-
benign
g.38226600T>G
g.38367347T>G
-
-
OTC_000004
47 individuals tested
PubMed: Mitchell 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
2
2
c.137A>C
r.(?)
p.(Lys46Thr ), p.(Lys46Thr)
-
benign
g.38226603A>C
g.38367350A>C
-
-
OTC_000387
47 individuals tested
PubMed: Mitchell 2009
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/., ?/.
11
2
c.137A>G
r.(?), r.137a>g
p.(Lys46Arg), p.Lys46Arg
-
benign, VUS
g.38226603A>G
g.38367350A>G
OTC(NM_000531.5):c.137A>G (p.K46R), OTC(NM_000531.6):c.137A>G (p.K46R)
-
OTC_000005
100 individuals tested, 78 homozygous;
Clinindb (India)
, 82 heterozygous;
Clinindb (India)
,
2 more items
Grompe 1989,
PubMed: Engel 2008
,
PubMed: Mitchell 2009
,
PubMed: Narang 2020
,
Journal: Narang 2020
,
1 more item
-
rs1800321
CLASSIFICATION record, Germline
-
0.36, 33/208, 78/2790 individuals, 82/2790 individuals
?, DdeI
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
Bert Bakker
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
,
Mohammed Faruq
?/.
1
2
c.140A>C
r.(?)
p.(Asn47Thr)
-
VUS
g.38226606A>C
g.38367353A>C
-
-
OTC_000359
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
2
c.140A>T
r.(?)
p.(Asn47IIe)
-
VUS
g.38226606A>T
g.38367353A>T
-
-
OTC_000103
-
PubMed: Tuchman 1997
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
2
c.140del
r.(?)
p.(Asn47ThrfsTer17)
-
VUS
g.38226606del
g.38367353del
-
-
OTC_000142
OTC activity 0%
Calvas 1998
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
2
c.140dup
r.(?)
p.(Asn47LysfsTer8)
-
VUS
g.38226606dup
g.38367353dup
-
-
OTC_000351
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
2
c.140_141insG
r.(?)
p.fsX
-
VUS
g.38226606_38226607insG
g.38367353_38367354insG
-
-
OTC_000143
-
PubMed: Shimadzu 1998
-
-
Germline
-
-
HindIII;AluI
-
-
Bert Bakker
?/.
1
2
c.143T>C
r.(?)
p.(Phe48Ser)
-
VUS
g.38226609T>C
g.38367356T>C
-
-
OTC_000182
-
PubMed: Genet 2000
-
-
Germline
-
-
-
-
-
Bert Bakker
+/.
1
-
c.144del
r.(?)
p.(Phe48LeufsTer16)
-
pathogenic
g.38226610del
g.38367357del
144delT
-
OTC_000429
-
PubMed: Martin-Hernandez 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
2
c.145A>C
r.(?)
p.(Thr49Pro)
-
VUS
g.38226611A>C
g.38367358A>C
-
-
OTC_000360
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
2
c.148G>A
r.(?)
p.(Gly50Arg)
-
VUS
g.38226614G>A
g.38367361G>A
-
-
OTC_000104
-
PubMed: Tuchman 1997
-
-
Germline
-
-
MspI;HpaII
-
-
Bert Bakker
?/.
1
2
c.149G>T
r.(?)
p.(Gly50X)
-
VUS
g.38226615G>T
g.38367362G>T
-
-
OTC_000020
-
Feldmann 1992
-
-
Germline
-
-
HpaII;Ddel
-
-
Bert Bakker
+/., ?/.
2
2
c.154G>A
r.(?)
p.(Glu52Lys)
-
pathogenic, VUS
g.38226620G>A
g.38367367G>A
-
-
OTC_000183
-
PubMed: Martin-Hernandez 2014
,
PubMed: McCullough 2000
-
-
Germline
-
-
MboII
-
-
Johan den Dunnen
,
Bert Bakker
?/.
1
2
c.154G>T
r.(?)
p.(Glu52X)
-
VUS
g.38226620G>T
g.38367367G>T
-
-
OTC_000184
-
PubMed: McCullough 2000
-
-
Germline
-
-
MboII
-
-
Bert Bakker
?/.
1
2
c.155A>G
r.(?)
p.(Glu52Gly)
-
VUS
g.38226621A>G
g.38367368A>G
-
-
OTC_000362
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
2
c.156A>T
r.(?)
p.(Glu52Asp)
-
VUS
g.38226622A>T
g.38367369A>T
-
-
OTC_000185
OTC activity 4%
PubMed: McCullough 2000
-
-
Germline
-
-
Tsp509I
-
-
Bert Bakker
?/.
1
2
c.158T>C
r.(?)
p.(Ile53Thr)
-
VUS
g.38226624T>C
g.38367371T>C
-
-
OTC_000363
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
2
c.158T>G
r.(?)
p.(IIe53Ser)
-
VUS
g.38226624T>G
g.38367371T>G
-
-
OTC_000214
-
PubMed: Tuchman 2002
-
-
Germline
-
-
MseI;TspEI
-
-
Bert Bakker
?/.
1
2
c.163T>G
r.(?)
p.(Tyr55Asp)
-
VUS
g.38226629T>G
g.38367376T>G
-
-
OTC_000144
OTC activity 28%
Nishiyori 1998
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
-
c.164A>G
r.(?)
p.(Tyr55Cys)
-
VUS
g.38226630A>G
-
OTC(NM_000531.5):c.164A>G (p.Y55C)
-
OTC_000422
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
2
c.167T>C
r.(?)
p.(Met56Thr)
-
VUS
g.38226633T>C
g.38367380T>C
-
-
OTC_000105
OTC activity -54%
PubMed: Tuchman 1997
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
2
c.170T>A
r.(?)
p.(Leu57Gln)
-
VUS
g.38226636T>A
g.38367383T>A
-
-
OTC_000364
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
+?/., ?/.
2
2
c.174G>A
r.(?)
p.(Trp58Ter), p.(Trp58X)
-
likely pathogenic, VUS
g.38226640G>A
g.38367387G>A
OTC(NM_000531.6):c.174G>A (p.W58*)
-
OTC_000366
VKGL data sharing initiative Nederland
PubMed: Yamaguchi 2006
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Bert Bakker
,
VKGL-NL_Groningen
+?/.
1
-
c.176T>C
r.(?)
p.(Leu59Pro)
-
likely pathogenic
g.38226642T>C
g.38367389T>C
-
-
OTC_000420
-
-
-
-
Germline
-
-
-
-
-
Sha Hong
?/.
1
2
c.179C>T
r.(?)
p.(Ser60Leu)
-
VUS
g.38226645C>T
g.38367392C>T
-
-
OTC_000106
-
PubMed: Tuchman 1997
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
2
c.188T>C
r.(?)
p.(Leu63Pro)
-
VUS
g.38226654T>C
g.38367401T>C
-
-
OTC_000107
-
PubMed: Oppliger Leibundgut 1997
-
-
Germline
-
-
BgIII;XhoII
-
-
Bert Bakker
?/.
1
2
c.200T>G
r.(?)
p.(Ile67Arg)
-
VUS
g.38226666T>G
g.38367413T>G
-
-
OTC_000367
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
+/.
1
-
c.205C>T
r.(?)
p.(Gln69Ter)
-
pathogenic
g.38226671C>T
g.38367418C>T
-
-
OTC_000173
-
PubMed: Climent 1999
,
PubMed: Martin-Hernandez 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.211G>T
r.(?)
p.(Gly71Ter)
-
pathogenic
g.38226677G>T
g.38367424G>T
-
-
OTC_000430
-
PubMed: Arranz 2007
,
PubMed: Martin-Hernandez 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
2
2i
c.216+1G>A
r.spl
p.(?), p.?
-
pathogenic, VUS
g.38226683G>A
g.38367430G>A
c.IVS2+1G>A
-
OTC_000080
-
Oppliger Leibundgut 1996a,
PubMed: Silvera-Ruiz 2019
-
-
Germline
-
2/24 case chromosomes
-
-
-
Johan den Dunnen
,
Bert Bakker
?/.
1
2i
c.216+1G>T
r.spl?
p.(?)
-
VUS
g.38226683G>T
g.38367430G>T
-
-
OTC_000368
OTC activity 10%
Azevedo 2002
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
2i
c.217-54dup
r.(=)
p.(=)
-
VUS
g.38228995dup
g.38369742dup
c.IVS2-54insA
-
OTC_000032
-
Matsuura 1993
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
2i
c.217-1G>A
r.spl
p.(?)
-
VUS
g.38229048G>A
g.38369795G>A
c.IVS2-1G>A
-
OTC_000021
-
PubMed: Tuchman 1992
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
2i
c.217-?_298+?del
r.(?)
p.(?)
-
VUS
g.38229049_38229130del
g.38369796_38369877del
-
-
OTC_000145
1 more item
PubMed: Tuchman 1998
-
-
Germline
-
-
PsiI;SspI
-
-
Bert Bakker
?/.
1
3
c.227T>C
r.(?)
p.(Leu76Ser)
-
VUS
g.38229059T>C
g.38369806T>C
-
-
OTC_000186
-
PubMed: Genet 2000
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
3
c.231G>T
r.(?)
p.(Leu77Phe)
-
VUS
g.38229063G>T
g.38369810G>T
-
-
OTC_000187
OTC activity -35%
PubMed: McCullough 2000
-
-
Germline
-
-
CviRI;MwoI
-
-
Bert Bakker
?/.
1
3
c.232C>T
r.(?)
p.(Gln78X)
-
VUS
g.38229064C>T
g.38369811C>T
-
-
OTC_000369
-
PubMed: Yamaguchi 2006
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
3
c.234A>G
r.(?)
p.(Gln78Gln)
-
VUS
g.38229066A>G
g.38369813A>G
-
-
OTC_000215
-
PubMed: Climent and Rubio 2002
-
-
Germline
-
<0.01
-
-
-
Bert Bakker
?/.
1
3
c.236G>A
r.(?)
p.(Gly79Glu)
-
VUS
g.38229068G>A
g.38369815G>A
-
-
OTC_000022
OTC activity 0%
PubMed: Tuchman 1992
-
-
Germline
-
-
-
-
-
Bert Bakker
?/.
1
3
c.238A>G
r.(?)
p.(Lys80Glu)
-
VUS
g.38229070A>G
g.38369817A>G
-
-
OTC_000188
-
Schulz and Salo 2000
-
-
Germline
-
-
HinfI;PleI
-
-
Bert Bakker
?/.
1
3
c.240G>T
r.(?)
p.(Lys80Asn)
-
VUS
g.38229072G>T
g.38369819G>T
-
-
OTC_000189
-
Galloway 2000
-
-
Germline
-
-
EcoRI;Tsp509I
-
-
Bert Bakker
?/.
1
3
c.243_245del
r.(?)
p.(Leu82del)
-
VUS
g.38229075_38229077del
g.38369822_38369824del
-
-
OTC_000061
-
PubMed: Tuchman and Plante 1995
-
-
Germline
-
-
Ddel;Bsu36I
-
-
Bert Bakker
?/.
1
3
c.245T>G
r.(?)
p.(Leu82X)
-
VUS
g.38229077T>G
g.38369824T>G
-
-
OTC_000216
-
PubMed: Tuchman 2002
-
-
Germline
-
-
BseMII;MnII
-
-
Bert Bakker
?/.
1
3
c.247G>C
r.(?)
p.(Gly83Arg)
-
VUS
g.38229079G>C
g.38369826G>C
-
-
OTC_000109
-
PubMed: Tuchman 1997
-
-
Germline
-
-
Bsu36I
-
-
Bert Bakker
?/.
1
3
c.248G>A
r.(?)
p.(Gly83Asp)
-
VUS
g.38229080G>A
g.38369827G>A
-
-
OTC_000146
-
Bartholomew and McClellan1998
-
-
Germline
-
-
CvnI
-
-
Bert Bakker
+/.
1
-
c.254T>G
r.(?)
p.(Ile85Ser)
-
pathogenic
g.38229086T>G
g.38369833T>G
-
-
OTC_000431
-
PubMed: Martin-Hernandez 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
3
c.259G>A
r.(?)
p.(Glu87Lys)
-
VUS
g.38229091G>A
g.38369838G>A
-
-
OTC_000023
-
Feldmann 1992
-
-
Germline
-
-
MseI
-
-
Bert Bakker
+/.
1
-
c.263A>C
r.(?)
p.(Lys88Thr)
-
pathogenic
g.38229095A>C
g.38369842A>C
-
-
OTC_000432
-
PubMed: Martin-Hernandez 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
2
3
c.264A>T
r.(?)
p.(Lys88Asn)
-
pathogenic, VUS
g.38229096A>T
g.38369843A>T
-
-
OTC_000033
OTC activity 3%
Reish 1993,
PubMed: Martin-Hernandez 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Bert Bakker
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