Global Variome shared LOVD
DBT (dihydrolipoamide branched chain transacylase E2)
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Global Variome, with Curator vacancy
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Unique variants in the DBT gene
The variants shown are described using the
NM_001918.2
NM_001918.3
transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Reported
: The number of times this variant has been reported in the database.
Exon
: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA)
: description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change
: description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein
: description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Classification method
: The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification
: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19)
: HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38)
: HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as
: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN
: description of the variant according to ISCN nomenclature
DB-ID
: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks
: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference
: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID
: ID of variant in ClinVar database
dbSNP ID
: the dbSNP ID
Origin
: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation
: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency
: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site
: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP
: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator. NOTE: to get VIP status ask the curator.
Methylation
: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
How to query this table
All list views have search fields which can be used to search data. You can search for a complete word or you can search for a part of a search term. If you enclose two or more words in double quotes, LOVD will search for the combination of those words only exactly in the order you specify. Note that search terms are case-insensitive and that wildcards such as * are treated as normal text! For all options, like "and", "or", and "not" searches, or searching for prefixes or suffixes, see the table below.
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.
57 entries on 1 page. Showing entries 1 - 57.
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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.-17G>C
r.(?)
p.(=)
-
likely benign
g.100715393C>G
g.100249837C>G
DBT(NM_001918.5):c.-17G>C
-
DBT_000018
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.16A>T
r.(?)
p.(Met6Leu)
-
likely benign
g.100715361T>A
g.100249805T>A
DBT(NM_001918.3):c.16A>T (p.M6L)
-
DBT_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
2
c.30G>A
r.(?)
p.(Trp10Ter)
-
pathogenic (recessive)
g.100715347C>T
g.100249791C>T
-
-
DBT_000059
-
PubMed: Khalifa 2020
,
Journal: Khalifa 2020
-
-
Germline
-
2/66 alleles MSUD
-
-
-
Johan den Dunnen
+/.
4
2
c.75_76del
r.(?)
p.(Cys26Trpfs*2), p.(Cys26TrpfsTer2)
ACMG
pathogenic (recessive)
g.100706416_100706417del
g.100240860_100240861del
75_76delAT
-
DBT_000037
Imtiaz 2017:28417071, Hjelm 2010:20639189, Rodríguez-Pombo 2006:16786533,
1 more item
PubMed: Flaschker 2007
,
Journal: Flaschker 2007
,
PubMed: Henneke 2003
,
Journal: Henneke 2003
ClinVar-11950
rs768832921
Germline
yes
-
-
-
-
Johan den Dunnen
,
Miriam Erandi Reyna-Fabián
+/.
1
2i
c.176-1G>A
r.176_189del
p.Ala59GlyfsTer14
-
pathogenic (recessive)
g.100701068C>T
g.100235512C>T
IVS3-1G>A
-
DBT_000048
-
PubMed: Dursun 2002
, {DOI:Dursun 2002:10.1023/a:1015668425004}
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.204del
r.(?)
p.(Lys68Asnfs*12)
-
pathogenic
g.100701039del
-
-
-
DBT_000044
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
3
c.241_242del
r.(?)
p.(Val81Ter)
-
pathogenic (recessive)
g.100701002_100701003del
g.100235446_100235447del
241_242delGT
-
DBT_000058
-
PubMed: Khalifa 2020
,
Journal: Khalifa 2020
-
-
Germline
-
2/66 alleles MSUD
-
-
-
Johan den Dunnen
-?/.
1
-
c.251+19dup
r.(=)
p.(=)
-
likely benign
g.100700978dup
g.100235422dup
DBT(NM_001918.5):c.251+19dupA
-
DBT_000032
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
4
c.263_265del
r.(?)
p.(Glu88del)
ACMG
likely pathogenic (recessive)
g.100696460_100696462del
g.100230904_100230906del
-
-
DBT_000041
1 more item
-
ClinVar-527136
rs1217050849
Germline
yes
-
-
-
-
Miriam Erandi Reyna-Fabián
+/.
1
4
c.294C>G
r.294C>G
p.Ile98Met
-
pathogenic (recessive)
g.100696428G>C
g.100230872G>C
309C>G (I37M)
-
DBT_000047
0.09 decarboxylation activity
PubMed: Tsurata 1998
,
Journal: Tsurata 1998
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/., -?/.
3
-
c.327C>T
r.(?)
p.(=)
-
benign, likely benign
g.100696395G>A
g.100230839G>A
DBT(NM_001918.3):c.327C>T (p.T109=), DBT(NM_001918.5):c.327C>T (p.T109=)
-
DBT_000031
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
+/.
1
-
c.342T>A
r.(?)
p.(Tyr114*)
-
pathogenic
g.100696380A>T
g.100230824A>T
DBT(NM_001918.3):c.342T>A (p.Y114*)
-
DBT_000015
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
2
6
c.434-15_434-4del
r.spl?
p.?
ACMG
pathogenic (recessive)
g.100684307_100684318del
g.100218751_100218762del
-
-
DBT_000036
another patient (same phenotype) showed same variant in homozygous state,
1 more item
PubMed: Vela-Amieva 2024
ClinVar-166983
rs727503895
Germline
yes
2/97 patients
-
-
-
Miriam Erandi Reyna-Fabián
+/.
5
6
c.670G>T
r.(?)
p.(Glu224*), p.(Glu224Ter)
ACMG
pathogenic (recessive)
g.100681641C>A
g.100216085C>A
-
-
DBT_000040
Khalifa 2020:32812330, Rodríguez-Pombo 2006:16786533, Homanics 2006:16579849
PubMed: Khalifa 2020
,
Journal: Khalifa 2020
,
PubMed: Vela-Amieva 2024
ClinVar-94009
rs74103423
Germline
yes
8/66 alleles MSUD
-
-
-
Johan den Dunnen
,
Miriam Erandi Reyna-Fabián
-/.
1
-
c.715A>G
r.(?)
p.(Ile239Val)
-
benign
g.100681596T>C
g.100216040T>C
DBT(NM_001918.5):c.715A>G (p.I239V)
-
DBT_000030
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/., -?/.
3
-
c.724T>C
r.(?)
p.(Ser242Pro)
-
benign, likely benign
g.100681587A>G
g.100216031A>G
DBT(NM_001918.3):c.724T>C (p.S242P), DBT(NM_001918.5):c.724T>C (p.S242P)
-
DBT_000014
21 heterozygous, no homozygous;
Clinindb (India)
, VKGL data sharing initiative Nederland
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs146249007
CLASSIFICATION record, Germline
-
21/2794 individuals
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_AMC
,
Mohammed Faruq
+/.
1
6
c.747_773del
r.(?)
p.(Lys250_Gly258del)
-
pathogenic (recessive)
g.100680543_100681568del
g.100214987_100216012del
-
-
DBT_000057
-
PubMed: Flaschker 2007
,
Journal: Flaschker 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/., -?/.
2
-
c.753C>T
r.(?)
p.(=)
-
benign, likely benign
g.100681558G>A
g.100216002G>A
DBT(NM_001918.3):c.753C>T (p.D251=), DBT(NM_001918.5):c.753C>T (p.D251=)
-
DBT_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
+?/.
1
-
c.772+1G>A
r.spl?
p.?
-
likely pathogenic
g.100681538C>T
-
DBT(NM_001918.3):c.772+1G>A
-
DBT_000035
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
7
c.787A>T
r.(?)
p.(Met263Leu)
-
pathogenic (recessive)
g.100680525T>A
g.100214969T>A
-
-
DBT_000056
-
Journal: Jaradat 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
3
7
c.827T>G
r.(?)
p.(Phe276Cys)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive)
g.100680485A>C
g.100214929A>C
DBT(NM_001918.3):c.827T>G (p.F276C)
-
DBT_000013
VKGL data sharing initiative Nederland
PubMed: Henneke 2003
,
Journal: Henneke 2003
,
PubMed: Soriano-Sexto 2022
,
Journal: Soriano-Sexto 2022
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Utrecht
?/.
1
-
c.872G>A
r.(?)
p.(Arg291Gln)
-
VUS
g.100680440C>T
g.100214884C>T
DBT(NM_001918.5):c.872G>A (p.R291Q)
-
DBT_000012
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.899C>T
r.(?)
p.(Ala300Val)
-
likely benign
g.100680413G>A
-
DBT(NM_001918.3):c.899C>T (p.(Ala300Val))
-
DBT_000043
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
7
c.920T>C
r.(?)
p.(Phe307Ser)
-
pathogenic (recessive)
g.100680392A>G
g.100214836A>G
-
-
DBT_000055
-
PubMed: Flaschker 2007
,
Journal: Flaschker 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
7i
c.940-1G>A
r.940_1017del
p.Ala315_Ala340del
-
pathogenic (recessive)
g.100676328C>T
g.100210772C>T
-
-
DBT_000054
enzyme activity 0.03
PubMed: Henneke 2003
,
Journal: Henneke 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.963G>C
r.(?)
p.(Gln321His)
-
VUS
g.100676304C>G
g.100210748C>G
DBT(NM_001918.3):c.963G>C (p.(Gln321His))
-
DBT_000011
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
1
-
c.972C>T
r.(?)
p.(Phe324=)
-
benign
g.41928652C>T
g.41422747C>T
-
-
BCKDHA_000018
-
Journal: Jaradat 2016
-
rs284652
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.995+26C>T
r.(?)
p.(=)
-
benign
g.41928701C>T
g.41422796C>T
-
-
BCKDHA_000053
-
Journal: Jaradat 2016
-
rs284653
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.995+49G>A
r.(?)
p.(=)
-
benign
g.41928724G>A
g.41422819G>A
-
-
DBT_000001
-
Journal: Jaradat 2016
-
rs284654
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
8i
c.1018-550A>G
r.1017_1018ins1018-676_1018-551
p.Ala340TyrfsTer5, p.fs*
-
pathogenic (recessive)
g.100672742T>C
g.100207186T>C
-
-
DBT_000045
0.09 decarboxylation activity, variant causes allelic expression imbalance
PubMed: Soriano-Sexto 2022
,
Journal: Soriano-Sexto 2022
,
PubMed: Tsurata 1998
,
Journal: Tsurata 1998
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
9
c.1057G>A
r.(?)
p.(Gly353Ser)
-
pathogenic (recessive)
g.100672153C>T
g.100206597C>T
-
-
DBT_000053
-
Journal: Jaradat 2016
-
rs578139656
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.1100T>A
r.(?)
p.(Ile367Lys)
-
VUS
g.100672110A>T
-
DBT(NM_001918.3):c.1100T>A (p.I367K)
-
DBT_000034
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.1132C>T
r.(?)
p.(Gln378Ter)
-
likely pathogenic
g.100672078G>A
g.100206522G>A
-
-
DBT_000042
-
PubMed: Wang 2019
-
-
Germline
-
2/2 case chromosomes
-
-
-
Johan den Dunnen
+/., -/., -?/.
7
9
c.1150A>G
r.(?), r.1150A>G
p.(Ser384Gly), p.Ser384Gly
-
benign, benign (!), likely benign
g.100672060T>C
g.100206504T>C
1150G>A (G384S), 1150G>A (Gly384Ser), 1151G>A (G384S), 1165G>A (G323S),
1 more item
-
DBT_000028
enzyme activity <0.05, variant originally reported as pathogenic,
1 more item
PubMed: Flaschker 2007
,
Journal: Flaschker 2007
,
PubMed: Henneke 2003
,
Journal: Henneke 2003
,
1 more item
-
rs12021720
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
-/., -?/.
2
-
c.1210-10del
r.(=)
p.(=)
-
benign, likely benign
g.100671879del
g.100206323del
DBT(NM_001918.3):c.1210-10delT, DBT(NM_001918.5):c.1210-10delT
-
DBT_000025
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-?/.
1
-
c.1210-10dup
r.(=)
p.(=)
-
likely benign
g.100671879dup
g.100206323dup
DBT(NM_001918.5):c.1210-10dupT
-
DBT_000026
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
2
-
c.1210-10T>A
r.(=)
p.(=)
-
benign
g.100671867A>T
g.100206311A>T
DBT(NM_001918.3):c.1210-10T>A, DBT(NM_001918.5):c.1210-10T>A
-
DBT_000024
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_AMC
-/.
1
-
c.1210-9A>T
r.(=)
p.(=)
-
benign
g.100671866T>A
g.100206310T>A
DBT(NM_001918.5):c.1210-9A>T
-
DBT_000010
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
2
-
c.1210-9_1210-8insT
r.(=)
p.(=)
-
benign
g.100671865_100671866insA
g.100206309_100206310insA
DBT(NM_001918.3):c.1210-9_1210-8insT, DBT(NM_001918.5):c.1210-9_1210-8insT
-
DBT_000023
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_AMC
-?/.
1
-
c.1210-4del
r.spl?
p.?
-
likely benign
g.100671866del
g.100206310del
DBT(NM_001918.5):c.1210-4delA
-
DBT_000008
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.1210-3T>A
r.spl?
p.?
ACMG
VUS
g.100671860A>T
g.100206304A>T
-
-
DBT_000039
1 more item
-
ClinVar-1906704
-
Germline
yes
-
-
-
-
Miriam Erandi Reyna-Fabián
?/.
1
10
c.1261G>T
r.(?)
p.(Gly421Trp)
ACMG
VUS
g.100671806C>A
g.100206250C>A
-
-
DBT_000038
1 more item
-
ClinVar-203669
rs796051945
Germline
yes
-
-
-
-
Miriam Erandi Reyna-Fabián
-?/.
1
-
c.1282-114C>T
r.(=)
p.(=)
-
likely benign
g.100662092G>A
g.100196536G>A
DBT(NM_001918.3):c.1282-114C>T
-
DBT_000007
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
1
-
c.1282-17_1282-10del
r.(=)
p.(=)
-
benign
g.100662008_100662015del
g.100196452_100196459del
DBT(NM_001918.5):c.1282-17_1282-10delTTTTTTTT
-
DBT_000021
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.1282-16_1282-10del
r.(=)
p.(=)
-
likely benign
g.100662009_100662015del
g.100196453_100196459del
DBT(NM_001918.5):c.1282-16_1282-10delTTTTTTT
-
DBT_000003
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
2
-
c.1282-15_1282-10del
r.(=)
p.(=)
-
likely benign
g.100662010_100662015del
g.100196454_100196459del
DBT(NM_001918.3):c.1282-15_1282-10delTTTTTT, DBT(NM_001918.5):c.1282-15_1282-10delTTTTTT
-
DBT_000002, DBT_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_AMC
-/., -?/.
2
-
c.1282-14_1282-10del
r.(=)
p.(=)
-
benign, likely benign
g.100662011_100662015del
g.100196455_100196459del
DBT(NM_001918.3):c.1282-14_1282-10delTTTTT, DBT(NM_001918.5):c.1282-14_1282-10delTTTTT
-
DBT_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-/., -?/.
2
-
c.1282-13_1282-10del
r.(=)
p.(=)
-
benign, likely benign
g.100662012_100662015del
g.100196456_100196459del
DBT(NM_001918.3):c.1282-13_1282-10delTTTT, DBT(NM_001918.5):c.1282-13_1282-10delTTTT
-
DBT_000005
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
-/.
1
-
c.1282-11_1282-10del
r.(=)
p.(=)
-
benign
g.100662014_100662015del
g.100196458_100196459del
DBT(NM_001918.5):c.1282-11_1282-10delTT
-
DBT_000022
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
2
-
c.1282-4C>A
r.spl?
p.?
-
likely benign
g.100661982G>T
g.100196426G>T
DBT(NM_001918.3):c.1282-4C>A
-
DBT_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
+/.
1
11
c.1286T>A
r.(?)
p.(Ile429Asn)
-
pathogenic (recessive)
g.100661974A>T
g.100196418A>T
-
-
DBT_000052
enzyme activity 0.65
PubMed: Henneke 2003
,
Journal: Henneke 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
5
11
c.1291C>T
r.(?)
p.(Arg431Ter)
-
likely pathogenic (recessive)
g.100661969G>A
g.100196413G>A
-
-
DBT_000051
-
PubMed: Khalifa 2020
,
Journal: Khalifa 2020
-
rs398123660
Germline
-
10/66 alleles MSUD
-
-
-
Johan den Dunnen
+/.
1
-
c.1333_1336delAATG
r.(?)
p.(Asn445Ter)
-
pathogenic (recessive)
g.100661928_100661931del
g.100196372_100196375del
1333_1336delAATG
-
DBT_000050
-
PubMed: Jaafar 2013
,
Journal: Jaafar 2013
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
11
c.1336G>A
r.(?)
p.(Val446Met)
-
pathogenic (recessive)
g.100661924C>T
g.100196368C>T
-
-
DBT_000049
enzyme activity 0.65
PubMed: Henneke 2003
,
Journal: Henneke 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.1418A>G
r.(?)
p.(Asn473Ser)
-
likely benign
g.100661842T>C
g.100196286T>C
DBT(NM_001918.3):c.1418A>G (p.N473S)
-
DBT_000019
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
11
c.1448G>T
r.1448G>T
p.Ter483LeuextTer7
-
pathogenic (recessive)
g.100661812C>A
g.100196256C>A
1463G>T (X422L)
-
DBT_000046
0.20 decarboxylation activity
PubMed: Tsurata 1998
,
Journal: Tsurata 1998
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
2
-
c.*4872A>G
r.(=)
p.(=)
-
likely benign
g.100656939T>C
g.100191383T>C
-
-
DBT_000033
1 homozygous;
Clinindb (India)
, 85 heterozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs12044663
Germline
-
1/2787 individuals, 85/2787 individuals
-
-
-
Mohammed Faruq
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