Global Variome shared LOVD
BCKDHB (branched chain keto acid dehydrogenase E1, ...)
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Unique variants in the BCKDHB gene
The variants shown are described using the
NM_000056.3
NM_183050.2
transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Reported
: The number of times this variant has been reported in the database.
Exon
: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA)
: description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change
: description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein
: description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Classification method
: The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification
: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19)
: HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38)
: HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as
: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN
: description of the variant according to ISCN nomenclature
DB-ID
: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks
: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference
: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID
: ID of variant in ClinVar database
dbSNP ID
: the dbSNP ID
Origin
: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation
: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency
: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site
: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP
: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator. NOTE: to get VIP status ask the curator.
Methylation
: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
How to query this table
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Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
all entries containing 'Arg' and 'Ser'
|
Text
Arg|Ser
all entries containing 'Arg' or 'Ser'
!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
=""
all entries with this field empty
=""
Text
="p.0"
all entries exactly matching 'p.0'
!=""
Text
!=""
all entries with this field not empty
!=""
Text
!="p.0"
all entries not exactly matching 'p.0?'
combination
Text
*|Ter !fs
all entries containing '*' or 'Ter' but not containing 'fs'
Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
!
Date
!2020-03
all entries not matching March, 2020
<
Date
<2020
all entries before the year 2020
<=
Date
<=2020-06
all entries in or before June, 2020
>
Date
>2020-06
all entries after June, 2020
>=
Date
>=2020-06-15
all entries on or after June 15th, 2020
combination
Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
all entries exactly matching 23
|
Numeric
23|24
all entries exactly matching 23 or 24
!
Numeric
!23
all entries not exactly matching 23
<
Numeric
<23
all entries lower than 23
<=
Numeric
<=23
all entries lower than, or equal to, 23
>
Numeric
>23
all entries higher than 23
>=
Numeric
>=23
all entries higher than, or equal to, 23
combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
Example
Matches
Asian
all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
To sort on a certain column, click on the column header or on the arrows. If that column is already selected to sort on, the sort order will be swapped. The column currently sorted on has a darker blue background color than the other columns. The up and down arrows next to the column name indicate the current sorting direction. When sorting on any field other than the default, LOVD will sort secondarily on the default sort column.
67 entries on 1 page. Showing entries 1 - 67.
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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.*109G>A
r.(?)
p.(=)
-
benign
g.81055792G>A
g.80346075G>A
-
-
BCKDHB_000064
-
Journal: Jaradat 2016
-
rs15062
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.*120C>T
r.(?)
p.(=)
-
benign
g.81055803C>T
g.80346086C>T
-
-
BCKDHB_000065
-
Journal: Jaradat 2016
-
rs10864
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.10G>A
r.(?)
p.(Val4Ile)
-
likely benign
g.80816420G>A
-
BCKDHB(NM_183050.4):c.10G>A (p.V4I)
-
BCKDHB_000023
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.23C>T
r.(?)
p.(Ala8Val)
-
VUS
g.80816433C>T
g.80106716C>T
-
-
BCKDHB_000021
-
PubMed: Taylor 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/., -?/.
2
-
c.51A>G
r.(?)
p.(Ala17=)
-
benign, likely benign
g.80816461A>G
g.80106744A>G
BCKDHB(NM_183050.4):c.51A>G (p.A17=)
-
BCKDHB_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_AMC
?/.
1
-
c.79C>T
r.(?)
p.(Pro27Ser)
-
VUS
g.80816489C>T
g.80106772C>T
BCKDHB(NM_000056.3):c.79C>T (p.(Pro27Ser))
-
BCKDHB_000002
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
1
c.152del
r.(?)
p.(Val51Glyfs*21)
ACMG
pathogenic (recessive)
g.80816562del
g.80106845del
-
-
BCKDHB_000033
Bashyam 2012:22593002, Rodríguez-Pombo 2006:16786533
-
ClinVar-527129
rs867612284
Germline
yes
-
-
-
-
Miriam Erandi Reyna-Fabián
+/.
1
1
c.163_166dup
r.(?)
p.(Phe56TyrfsTer29)
-
pathogenic (recessive)
g.80816573_80816576dup
g.80106856_80106859dup
163_166dupACTT
-
BCKDHB_000040
no enzyme activity
PubMed: Henneke 2003
,
Journal: Henneke 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.172C>T
r.(?)
p.(Pro58Ser)
-
VUS
g.80816582C>T
-
BCKDHB(NM_183050.4):c.172C>T (p.P58S)
-
BCKDHB_000022
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.(?_197-51)_(742+51_?)dup
r.?
p.?
ACMG
VUS
g.(?_80837213)_(80881158_?)dup
g.(?_80127496)_(80171441_?)dup
197-51_742+51dup
-
BCKDHB_000028
-
-
-
-
Germline
?
-
-
-
-
Zeinab Sayed Abdelkhalek
-/.
1
-
c.197-25A>G
r.(?)
p.(=)
-
benign
g.80837239A>G
g.80127522A>G
-
-
BCKDHB_000041
-
Journal: Jaradat 2016
-
rs9448893
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.197-11del
r.(=)
p.(=)
-
likely benign
g.80837253del
g.80127536del
BCKDHB(NM_183050.4):c.197-11delG
-
BCKDHB_000003
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-/.
4
-
c.197-11G>T
r.(=), r.(?)
p.(=)
-
benign
g.80837253G>T
g.80127536G>T
BCKDHB(NM_000056.4):c.197-11G>T, BCKDHB(NM_183050.4):c.197-11G>T
-
BCKDHB_000004
VKGL data sharing initiative Nederland
Journal: Jaradat 2016
-
rs9448894
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
-?/.
1
-
c.197-10A>T
r.(=)
p.(=)
-
likely benign
g.80837254A>T
g.80127537A>T
BCKDHB(NM_183050.4):c.197-10A>T
-
BCKDHB_000005
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.197-2A>G
r.spl
p.?
-
pathogenic
g.80837262A>G
g.80127545A>G
NM_183050.4:c.197-2A>G
-
BCKDHB_000026
-
PubMed: Chuan 2022
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
2
c.207G>A
r.(?)
p.(Gln69=)
-
benign
g.80837274G>A
g.80127557G>A
-
-
BCKDHB_000039
-
Journal: Jaradat 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.221del
r.(?)
p.(Phe74Serfs*4)
ACMG
pathogenic
g.80837288del
g.80127571del
218delT
-
BCKDHB_000027
ACMG PVS1,PM2,PM3,PM4
PubMed: Chuan 2022
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.238G>A
r.(?)
p.(Ala80Thr)
-
VUS
g.80837305G>A
-
BCKDHB(NM_183050.4):c.238G>A (p.(Ala80Thr))
-
BCKDHB_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
1
2i
c.274+46G>A
r.(?)
p.(=)
-
benign
g.80837387G>A
g.80127670G>A
-
-
BCKDHB_000037
-
Journal: Jaradat 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
2i
c.274+54G>A
r.(?)
p.(=)
-
benign
g.80837395G>A
g.80127678G>A
-
-
BCKDHB_000038
-
Journal: Jaradat 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.281_291del
r.(?)
p.(Phe94CysfsTer8)
-
likely pathogenic
g.80838884_80838894del
-
BCKDHB(NM_183050.4):c.281_291delTTGGTGAAGAT (p.F94Cfs*8)
-
BCKDHB_000010
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/.
2
3
c.288del
r.(?)
p.(Asp97MetfsTer133)
-
pathogenic (recessive)
g.80838891del
g.80129174del
287_287delA
-
BCKDHB_000042
-
PubMed: Khalifa 2020
,
Journal: Khalifa 2020
-
-
Germline
-
4/66 alleles MSUD
-
-
-
Johan den Dunnen
+?/.
1
-
c.302G>A
r.(?)
p.(Gly101Asp)
-
likely pathogenic
g.80838905G>A
g.80129188G>A
-
-
BCKDHB_000017
2 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs398124571
Germline
-
2/2795 individuals
-
-
-
Mohammed Faruq
+/.
1
3
c.331C>T
r.(?)
p.(Arg111Ter)
-
pathogenic (recessive)
g.80838934C>T
g.80129217C>T
-
-
BCKDHB_000043
-
PubMed: Flaschker 2007
,
Journal: Flaschker 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.332G>A
r.(?)
p.(Arg111Gln)
-
VUS
g.80838935G>A
g.80129218G>A
BCKDHB(NM_183050.4):c.332G>A (p.R111Q)
-
BCKDHB_000006
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
4
c.368C>T
r.(?)
p.(Pro123Leu)
-
pathogenic (recessive)
g.80877419C>T
g.80167702C>T
-
-
BCKDHB_000044
-
PubMed: Flaschker 2007
,
Journal: Flaschker 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
4
c.389T>G
r.(?)
p.(Val130Gly)
-
pathogenic (recessive)
g.80877440T>G
g.80167723T>G
-
-
BCKDHB_000045
-
PubMed: Flaschker 2007
,
Journal: Flaschker 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.397G>A
r.(?)
p.(Gly133Arg)
-
likely pathogenic
g.80877448G>A
-
-
-
BCKDHB_000025
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
4
c.403G>A
r.(?)
p.(Gly135Arg)
-
pathogenic (recessive)
g.80877454G>A
g.80167737G>A
-
-
BCKDHB_000046
enzyme activity <0.05
PubMed: Henneke 2003
,
Journal: Henneke 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
4
c.410C>T
r.(?)
p.(Ala137Val)
-
pathogenic (recessive)
g.80877461C>T
g.80167744C>T
-
-
BCKDHB_000047
-
PubMed: Khalifa 2020
,
Journal: Khalifa 2020
-
rs776631396
Germline
-
2/66 alleles MSUD
-
-
-
Johan den Dunnen
+/.
1
4
c.475C>A
r.(?)
p.(Gln159Lys)
-
pathogenic (recessive)
g.80877526C>A
g.80167809C>A
-
-
BCKDHB_000048
no enzyme activity
PubMed: Henneke 2003
,
Journal: Henneke 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
5
c.502C>T
r.(?)
p.(Arg168Cys)
-
pathogenic (recessive)
g.80878616C>T
g.80168899C>T
-
-
BCKDHB_000049
-
PubMed: Flaschker 2007
,
Journal: Flaschker 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
5
c.564T>A
r.(?)
p.(Cys188*)
ACMG
pathogenic (recessive)
g.80878678T>A
g.80168961T>A
-
-
BCKDHB_000032
Bashyam 2012:22593002, Gorzelany 2009:22593002, Rodríguez-Pombo 2006:16786533
-
ClinVar-580585
rs774306610
Germline
yes
-
-
-
-
Miriam Erandi Reyna-Fabián
+/.
2
5
c.595_596del
r.(?)
p.(Pro200Ter)
-
pathogenic (recessive)
g.80878709_80878710del
g.80168992_80168993del
595_596delAG
-
BCKDHB_000009
enzyme activity <0.05
PubMed: Henneke 2003
,
Journal: Henneke 2003
,
1 more item
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
5
c.598C>G
r.(?)
p.(Pro200Ala)
-
pathogenic (recessive)
g.80878712C>G
g.80168995C>G
-
-
BCKDHB_000050
-
PubMed: Flaschker 2007
,
Journal: Flaschker 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.633G>C
r.(?)
p.(Lys211Asn)
-
VUS
g.80878747G>C
g.80169030G>C
BCKDHB(NM_183050.4):c.633G>C (p.K211N)
-
BCKDHB_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
6
c.665A>G
r.(?)
p.(Lys222Arg)
-
pathogenic (recessive)
g.80881030A>G
g.80171313A>G
-
-
BCKDHB_000051
-
PubMed: Henneke 2003
,
Journal: Henneke 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
6
c.667G>C
r.(?)
p.(Gly223Arg)
ACMG
likely pathogenic (recessive)
g.80881032G>C
g.80171315G>C
-
-
BCKDHB_000034
1 more item
-
-
rs1248040495
Germline
yes
-
-
-
-
Miriam Erandi Reyna-Fabián
+/.
2
-
c.716A>G
r.(?)
p.(Glu239Gly)
-
pathogenic (recessive)
g.80881081A>G
g.80171364A>G
-
-
BCKDHB_000052
-
PubMed: Jaafar 2013
,
Journal: Jaafar 2013
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
6
c.721A>T
r.(?)
p.(Lys241Ter)
-
pathogenic (recessive)
g.80881086A>T
g.80171369A>T
-
-
BCKDHB_000053
enzyme activity 0.05, enzyme activity <0.05
PubMed: Henneke 2003
,
Journal: Henneke 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.722_731dup
r.(?)
p.(Tyr244Ter)
-
pathogenic
g.80881087_80881096dup
g.80171370_80171379dup
BCKDHB(NM_183050.4):c.722_731dupAAATACTTTA (p.Y244*)
-
BCKDHB_000011
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
6
c.730T>C
r.(?)
p.(Tyr244His)
-
pathogenic (recessive)
g.80881095T>C
g.80171378T>C
724T>C (Tyr244His)
-
BCKDHB_000054
-
PubMed: Flaschker 2007
,
Journal: Flaschker 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.743-8G>T
r.(=)
p.(=)
-
likely benign
g.80910643G>T
g.80200926G>T
BCKDHB(NM_000056.3):c.743-8G>T (p.(=))
-
BCKDHB_000012
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.743-6A>T
r.(=)
p.(=)
-
likely benign
g.80910645A>T
g.80200928A>T
BCKDHB(NM_000056.3):c.743-6A>T (p.(=))
-
BCKDHB_000013
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.744G>A
r.(?)
p.(=)
-
likely benign
g.80910652G>A
-
BCKDHB(NM_183050.4):c.744G>A (p.(Ala248=))
-
BCKDHB_000035
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
7
c.752T>C
r.(?)
p.(Val251Ala)
-
pathogenic (recessive)
g.80910660T>C
g.80200943T>C
-
-
BCKDHB_000055
enzyme activity <0.01
PubMed: Henneke 2003
,
Journal: Henneke 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.772A>T
r.(?)
p.(Ile258Phe)
-
VUS
g.80910680A>T
-
BCKDHB(NM_183050.4):c.772A>T (p.(Ile258Phe))
-
BCKDHB_000030
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.799C>T
r.(?)
p.(Gln267Ter)
-
pathogenic
g.80910707C>T
g.80200990C>T
BCKDHB(NM_183050.4):c.799C>T (p.Q267*)
-
BCKDHB_000014
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+?/.
1
7
c.806G>A
r.(?)
p.(Gly269Glu)
-
likely pathogenic (recessive)
g.80910714G>A
g.80200997G>A
-
-
BCKDHB_000056
-
PubMed: Khalifa 2020
,
Journal: Khalifa 2020
-
-
Germline
-
2/66 alleles MSUD
-
-
-
Johan den Dunnen
+/.
1
7
c.808_821del
p.Ser270_Leu274del
p.(Asp271CysfsTer25)
-
pathogenic (recessive)
g.80910719_80910732del
g.80201002_80201015del
-
-
BCKDHB_000057
-
PubMed: Flaschker 2007
,
Journal: Flaschker 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
5
7
c.832G>A
r.(?)
p.(Gly278Ser)
-
pathogenic (recessive), VUS
g.80910740G>A
g.80201023G>A
BCKDHB(NM_183050.4):c.832G>A (p.G278S)
-
BCKDHB_000007
VKGL data sharing initiative Nederland
PubMed: Flaschker 2007
,
Journal: Flaschker 2007
,
1 more item
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
-/.
1
-
c.841-73C>T
r.(?)
p.(=)
-
benign
g.80912746C>T
g.80203029C>T
-
-
BCKDHB_000058
-
Journal: Jaradat 2016
-
rs3828753
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
8
c.848T>C
r.(?)
p.(Val283Ala)
-
pathogenic (recessive)
g.80912826T>C
g.80203109T>C
-
-
BCKDHB_000059
-
PubMed: Flaschker 2007
,
Journal: Flaschker 2007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., +?/.
6
8
c.853C>T
r.(?)
p.(Arg285*), p.(Arg285Ter)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.80912831C>T
g.80203114C>T
BCKDHB(NM_183050.4):c.853C>T (p.R285*)
-
BCKDHB_000015
1 heterozygous, no homozygous;
Clinindb (India)
, VKGL data sharing initiative Nederland
{PMID:Fang 2021:34556729, Narang 2020:32906206, Mei 2018:30298494},
3 more items
ClinVar-96615
rs398124598
CLASSIFICATION record, Germline
yes
1/2795 individuals, 2/66 alleles MSUD
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
Mohammed Faruq
,
Miriam Erandi Reyna-Fabián
+/.
2
8
c.908dup
r.(?)
p.(Asp303GlufsTer15)
-
pathogenic (recessive)
g.80912886dup
g.80203169dup
908_909insA
-
BCKDHB_000060
-
PubMed: Khalifa 2020
,
Journal: Khalifa 2020
-
-
Germline
-
4/66 alleles MSUD
-
-
-
Johan den Dunnen
+/.
1
-
c.910del
r.(?)
p.(Leu304Ter)
-
pathogenic
g.80912888del
g.80203171del
BCKDHB(NM_183050.4):c.910delC (p.L304*)
-
BCKDHB_000016
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
8
c.937del
r.(?)
p.(Asp313ThrfsTer7)
-
pathogenic (recessive)
g.80912915del
g.80203198del
937delG
-
BCKDHB_000061
enzyme activity 0.03
PubMed: Henneke 2003
,
Journal: Henneke 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
5
9
c.970C>T
r.(?)
p.(Arg324*), p.(Arg324Ter)
ACMG
pathogenic, pathogenic (recessive)
g.80982870C>T
g.80273153C>T
BCKDHB(NM_183050.4):c.970C>T (p.R324*)
-
BCKDHB_000008
Puckett 2010:20307994, Bashyam 2012:22593002, Couce 2015:26232051,
1 more item
PubMed: Khalifa 2020
,
Journal: Khalifa 2020
96621
rs398124603
CLASSIFICATION record, Germline
yes
4/66 alleles MSUD
-
-
-
Johan den Dunnen
,
VKGL-NL_Groningen
,
VKGL-NL_AMC
,
Miriam Erandi Reyna-Fabián
+?/.
2
9
c.995C>T
r.(?)
p.(Pro332Leu)
-
likely pathogenic, likely pathogenic (recessive)
g.80982895C>T
g.80273178C>T
-
-
BCKDHB_000024
-
PubMed: Khalifa 2020
,
Journal: Khalifa 2020
,
PubMed: Nair 2018
-
rs1554205541
Germline, Germline/De novo (untested)
-
2/66 alleles MSUD
-
-
-
Johan den Dunnen
+?/.
1
-
c.1022T>A
r.(?)
p.(Ile341Asn)
-
likely pathogenic
g.80982922T>A
g.80273205T>A
-
-
BCKDHB_000018
4 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs796051939
Germline
-
4/2777 individuals
-
-
-
Mohammed Faruq
+?/.
1
-
c.1038+2T>C
r.spl?
p.?
-
likely pathogenic
g.80982940T>C
-
BCKDHB(NM_183050.4):c.1038+2T>C
-
BCKDHB_000036
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.1046G>A
r.(?)
p.(Cys349Tyr)
-
VUS
g.81053388G>A
g.80343671G>A
-
-
BCKDHB_000019
conflicting interpretations of pathogenicity; 1 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs398124562
Germline
-
1/2795 individuals
-
-
-
Mohammed Faruq
+/.
2
10
c.1087T>A
r.(?)
p.(Tyr363Asn)
ACMG
likely pathogenic (recessive), pathogenic (recessive)
g.81053429T>A
g.80343712T>A
-
-
BCKDHB_000031
2 more items
-
ClinVar-96568
rs398124565
Germline
yes
2/97 patients
-
-
-
Miriam Erandi Reyna-Fabián
+?/.
4
10
c.1091A>G
r.(?)
p.(Asp364Gly)
-
likely pathogenic (recessive)
g.81053433A>G
g.80343716A>G
-
-
BCKDHB_000062
-
PubMed: Khalifa 2020
,
Journal: Khalifa 2020
-
-
Germline
-
8/66 alleles MSUD
-
-
-
Johan den Dunnen
+/.
2
10
c.1149T>A
r.(?)
p.(Tyr383Ter)
-
pathogenic (recessive)
g.81053491T>A
g.80343774T>A
-
-
BCKDHB_000063
enzyme activity <0.01
PubMed: Henneke 2003
,
Journal: Henneke 2003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.1150A>G
r.(?)
p.(Ser384Gly)
-
benign
g.100672060T>C
g.100206504T>C
-
-
DBT_000028
12 homozygous controls, 10 heterozygous
Journal: Jaradat 2016
-
rs12021720
Germline
-
34/100 control chromosomes
-
-
-
Johan den Dunnen
-/.
1
-
c.1221A>G
r.(?)
p.(Leu407=)
-
benign
g.41930396A>G
g.41424491A>G
-
-
BCKDHA_000021
-
Journal: Jaradat 2016
-
rs4674
Germline
-
-
-
-
-
Johan den Dunnen
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