Global Variome shared LOVD
HADHA (hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA...)
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Global Variome, with Curator vacancy
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Unique variants in the HADHA gene
The variants shown are described using the NM_000182.4 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.
104 entries on 2 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
_1_5i
c.-4259_453+337del
r.0?
p.0?
-
pathogenic (recessive)
g.26456748_26471723del
g.26233880_26248855del
hg38
-
HADHA_000063
-
PubMed: Bo 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.-1G>T
r.(?)
p.(=)
-
likely benign
g.26467465C>A
g.26244597C>A
HADHA(NM_000182.5):c.-1G>T
-
HADHA_000003
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.68-10T>G
r.(=)
p.(=)
-
likely benign
g.26462021A>C
-
HADHA(NM_000182.5):c.68-10T>G
-
HADHA_000031
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
-
c.78C>A
r.(?)
p.(Cys26*)
-
pathogenic (recessive)
g.26462001G>T
g.26239133G>T
Y-13ter
-
HADHA_000055
combination of variants not reported
PubMed: Spiekerkoetter 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.109+4A>C
r.spl?
p.?
-
likely benign
g.26461966T>G
-
HADHA(NM_000182.5):c.109+4A>C
-
HADHA_000037
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.138dup
r.(?)
p.(Gly47ArgfsTer9)
-
pathogenic (recessive)
g.26461846dup
g.26238978dup
-
-
HADHA_000103
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.157C>T
r.(?)
p.(Arg53Ter)
-
pathogenic (recessive)
g.26461825G>A
g.26238957G>A
-
-
HADHA_000102
-
PubMed: Bo 2017
,
PubMed: Sperk 2010
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.180_180+5delinsAT
r,spl, r.spl
(p.(Val61CysfsTer6), p.?
-
pathogenic (recessive)
g.26461797_26461802delinsAT
g.26238929_26238934delinsAT
180_180+5delGGTATCinsAT
-
HADHA_000101
no variant 2nd chromosome
PubMed: Diebold 2019
,
PubMed: Grunert 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
3i
c.180+1G>A
r.110_180del, r.spl
p.?, p.Thr37SerfsTer2
-
pathogenic (recessive)
g.26461801C>T
g.26238933C>T
IVS3G+1A
-
HADHA_000045
-
PubMed: Brackett 1995
,
PubMed: Ushikubo 1996
,
PubMed: Ibdah 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
4
3i
c.180+3A>G
r.110_180del, r.spl
p.?, p.Thr37SerfsTer2
-
pathogenic (recessive)
g.26461799T>C
g.26238931T>C
IVS3A+3G
-
HADHA_000046
-
PubMed: Brackett 1995
,
PubMed: Ushikubo 1996
,
PubMed: Diebold 2019
,
PubMed: Ibdah 1999
,
1 more item
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.180+3_180+6del
r.spl
p.?
-
pathogenic (recessive)
g.26461797_26461800del
g.26238929_26238932del
180+2_180+5delTATC
-
HADHA_000100
-
PubMed: Grunert 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
7
4
c.274_278del
r.(?)
p.(Ser92LysfsTer10)
-
pathogenic (recessive)
g.26459762_26459766del
g.26236894_26236898del
Asp67Ter (del271-275)
-
HADHA_000099
-
PubMed: Boutron 2011
,
PubMed: Grunert 2021
,
PubMed: Ibdah 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
4i
c.315-2A>T
r.spl
p.?
-
pathogenic (recessive)
g.26457225T>A
g.26234357T>A
IVS4A-2T
-
HADHA_000098
-
PubMed: Ibdah 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
2
-
c.316A>G
r.(?)
p.(Met106Val)
-
VUS
g.26457222T>C
g.26234354T>C
HADHA(NM_000182.5):c.316A>G (p.(Met106Val), p.M106V)
-
HADHA_000016
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Groningen
+/.
1
-
c.361C>T
r.(?)
p.(Gln121Ter)
-
pathogenic (recessive)
g.26457177G>A
g.26234309G>A
-
-
HADHA_000097
-
PubMed: Bo 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.389T>C
r.(?)
p.(Leu130Pro)
-
pathogenic (recessive)
g.26457149A>G
g.26234281A>G
L94P
-
HADHA_000056
-
PubMed: Spiekerkoetter 2002
-
-
Germline, Uniparental disomy, maternal allele
-
-
-
-
-
Johan den Dunnen
-?/.
1
4
c.414G>A
r.(=)
p.(=)
-
likely benign
g.26457124C>T
g.26234256C>T
Val138Val
-
HADHA_000002
-
PubMed: Gilissen 2014
-
-
De novo
?
-
-
-
-
Marianne Vos (LOVD-team)
+/.
1
-
c.442G>A
r.(?)
p.(Gly148Arg)
-
pathogenic (recessive)
g.26457096C>T
g.26234228C>T
-
-
HADHA_000096
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.446G>T
r.(?)
p.(Gly149Val)
-
pathogenic (recessive)
g.26457092C>A
g.26234224C>A
-
-
HADHA_000061
-
PubMed: Bursle 2018
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.453+1G>T
r.spl
p.?
-
likely pathogenic (recessive)
g.26457084C>A
g.26234216C>A
-
-
HADHA_000095
-
PubMed: Diebold 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
6
c.479_482delinsAATA
r.(?)
p.(Ile160delinsLysTer)
-
pathogenic (recessive)
g.26455119_26455122delinsTATT
g.26232251_26232254delinsTATT
Ala125Ter (TACC479-482AATA)
-
HADHA_000094
-
PubMed: Ibdah 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.556C>G
r.(?)
p.(Gln186Glu)
-
pathogenic (recessive)
g.26455045G>C
g.26232177G>C
546C>G
-
HADHA_000093
-
PubMed: Diekman 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
6i
c.574-2A>G
r.spl
p.?
-
pathogenic (recessive)
g.26453164T>C
g.26230296T>C
IVS6-2A>G, IVS6A-2G
-
HADHA_000050
-
PubMed: Ibdah 1999
,
PubMed: Spiekerkoetter 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.602T>C
r.(?)
p.(Met201Thr)
-
pathogenic (recessive)
g.26453134A>G
g.26230266A>G
-
-
HADHA_000092
-
PubMed: Grunert 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.652G>C
r.(?)
p.(Val218Leu)
-
VUS
g.26453084C>G
g.26230216C>G
-
-
HADHA_000022
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.676+2T>C
r.[574_676del,=]
p.[Val192AspfsTer4,=]
-
pathogenic (recessive)
g.26453058A>G
g.26230190A>G
-
-
HADHA_000091
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.677G>A
r.(?)
p.(Gly226Glu)
-
VUS
g.26438044C>T
-
HADHA(NM_000182.5):c.677G>A (p.G226E)
-
HADHA_000026
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
6
-
c.703C>T
r.(?), r.703c>u
p.(Arg235Trp), p.Arg235Trp
-
pathogenic (recessive)
g.26438018G>A
g.26215149G>A
-
-
HADHA_000047
-
PubMed: Boutron 2011
,
PubMed: Grunert 2021
,
PubMed: Scheuerman 2009
,
PubMed: Yang 2022
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
1
-
c.703_704dup
r.(?)
p.(Thr236Glyfs*3)
ACMG
pathogenic (recessive)
g.26438017_26438018dup
g.26215148_26215149dup
703_704dupCG
-
HADHA_000049
ACMG PVS1, PM2, PM3
PubMed: Chen 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
9
c.788A>G
r.(?)
p.(Asp263Gly)
-
pathogenic (recessive)
g.26502160A>G
g.26279292A>G
-
-
HADHB_000026
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
9
c.845T>A
r.(?), r.845u>a
p.(Val282Asp), p.Val282Asp
-
pathogenic (recessive)
g.26437385A>T
g.26214516A>T
Val246Asp
-
HADHA_000040
-
PubMed: Ibdah 1998
,
PubMed: Ibdah 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.859del
r.(?)
p.(Glu287LysfsTer16)
-
pathogenic (recessive)
g.26437372del
g.26214503del
-
-
HADHA_000090
no variant 2nd chromosome
PubMed: Diebold 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
9
c.871C>T
r.(?), r.0
p.(Arg291*), p.(Arg291Ter), p.0
-
pathogenic (recessive)
g.26437359G>A
g.26214490G>A
Arg255Ter, R256X
-
HADHA_000042
mRNA r.871c>u (Arg291*) allele not detectable
PubMed: Ibdah 1998
,
PubMed: Ibdah 1999
,
PubMed: Spiekerkoetter 2008
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
4
9
c.914T>A
r.(?), r.914u>a
p.(Ile305Asn), p.Ile305Asn
-
pathogenic (recessive)
g.26437316A>T
g.26214447A>T
Ile269Asn
-
HADHA_000041
-
PubMed: Boutron 2011
,
PubMed: Ibdah 1998
,
PubMed: Ibdah 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.918+1G>A
r.800_918del
p.Lys267SerfsTer7
-
pathogenic (recessive)
g.26437311C>T
g.26214442C>T
-
-
HADHA_000089
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.918+6T>G
r.800_918del
p.Lys267SerfsTer7
-
pathogenic (recessive)
g.26437306A>C
g.26214437A>C
-
-
HADHA_000088
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.919-2A>G
r.spl
p.?
-
pathogenic (recessive)
g.26435497T>C
g.26212628T>C
-
-
HADHA_000087
-
PubMed: Vieira Neto 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/., ?/.
3
-
c.955G>A
r.(?)
p.(Gly319Ser)
-
pathogenic (recessive), VUS
g.26435459C>T
g.26212590C>T
-
-
HADHA_000030
-
PubMed: Diebold 2019
,
PubMed: Khani 2020
-
rs752317877
Germline
yes
-
-
-
-
Johan den Dunnen
,
Yvet den Hartog
+/.
1
-
c.965G>A
r.(?)
p.(Gly319Ser)
-
pathogenic (recessive)
g.26435459C>T
g.26212590C>T
995G>A
-
HADHA_000030
-
PubMed: Grunert 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.1025T>C
r.(?)
p.(Leu342Pro)
-
pathogenic (recessive)
g.26432709A>G
g.26209840A>G
T1025C
-
HADHA_000043
-
PubMed: De Biase 2017
,
PubMed: IJlst 1997
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.1058_1059delinsT
r.(?)
p.(Lys353IlefsTer19)
-
pathogenic (recessive)
g.26432675_26432676delinsA
g.26209806_26209807delinsA
-
-
HADHA_000086
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
4
-
c.1072C>A
r.(?)
p.(Gln358Lys)
-
VUS
g.26432662G>T
g.26209793G>T
-
-
HADHA_000001
-
-
-
-
Germline
-
-
-
-
-
Gerard C.P. Schaafsma
+/.
1
12
c.1086-1_1091del
r.spl
p.(His363LeufsTer7)
-
pathogenic (recessive)
g.26427060_26427066del
g.26204191_26204197del
del-4-091
-
HADHA_000085
-
PubMed: Ibdah 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.1115A>T
r.(?)
p.(Asp372Val)
-
pathogenic (recessive)
g.26507038A>T
g.26284170A>T
-
-
HADHA_000104
-
PubMed: Grunert 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.1129G>A
r.(?)
p.(Ala377Thr)
-
VUS
g.26427022C>T
g.26204153C>T
HADHA(NM_000182.4):c.1129G>A (p.A377T)
-
HADHA_000021
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/., +?/.
3
12
c.1132C>T
r.(?)
p.(Gln378*), p.(Gln378Ter)
-
likely pathogenic, pathogenic (recessive)
g.26427019G>A
g.26204150G>A
(Q342Stop), Gln342Ter
-
HADHA_000027
-
PubMed: Ibdah 1999
,
PubMed: Sims 1995
,
PubMed: Stone 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.1174G>A
r.(?)
p.(Ala392Thr)
-
likely benign
g.26426977C>T
g.26204108C>T
HADHA(NM_000182.4):c.1174G>A (p.(Ala392Thr))
-
HADHA_000015
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
9
c.1191G>A
r.(?)
p.(Trp397Ter)
-
pathogenic (recessive)
g.26507792G>A
g.26284924G>A
-
-
HADHA_000105
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.1195C>T
r.(?)
p.(Arg399Ter)
-
pathogenic (recessive)
g.26426956G>A
g.26204087G>A
R363*
-
HADHA_000084
-
PubMed: Boutron 2011
,
PubMed: Grunert 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
2
-
c.1212G>C
r.(?)
p.(Val404=)
-
likely benign
g.26426939C>G
g.26204070C>G
HADHA(NM_000182.4):c.1212G>C (p.V404=)
-
HADHA_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
+/.
1
-
c.1234G>Y
r.(?)
p.(Val412Leu)
-
pathogenic (recessive)
g.26424176C>R
g.26201307C>R
V376L
-
HADHA_000054
combination of variants not reported
PubMed: Spiekerkoetter 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
3
-
c.1325A>T
r.(?)
p.(Asp442Val)
-
VUS
g.26424085T>A
-
HADHA(NM_000182.4):c.1325A>T (p.D442V), HADHA(NM_000182.5):c.1325A>T (p.(Asp442Val), p.D442V)
-
HADHA_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
+/.
2
-
c.1336_1393del
r.(?)
p.(Glu446Ter)
-
pathogenic (recessive)
g.26420646_26424074del
g.26197777_26201205del
1336-1393del57nt
-
HADHA_000081
-
PubMed: Diekman 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.1343T>C
r.(?)
p.(Val448Ala)
-
VUS
g.26424067A>G
-
HADHA(NM_000182.5):c.1343T>C (p.V448A)
-
HADHA_000025
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
-
c.1381del
r.(?)
p.(Glu461LysfsTer2)
-
pathogenic (recessive)
g.26424030del
g.26201161del
-
-
HADHA_000083
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.1392+1G>A
r.spl
p.?
-
pathogenic (recessive)
g.26424017C>T
g.26201148C>T
-
-
HADHA_000082
-
PubMed: Bo 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.1392+8A>C
r.(=)
p.(=)
-
likely benign
g.26424010T>G
g.26201141T>G
-
-
HADHA_000019
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.1432G>C
r.(?)
p.(Ala478Pro)
-
pathogenic (recessive)
g.26420607C>G
g.26197738C>G
-
-
HADHA_000080
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.1433C>T
r.(?)
p.(Ala478Val)
-
pathogenic (recessive)
g.26420606G>A
g.26197737G>A
-
-
HADHA_000079
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.1465A>G
r.(?)
p.(Lys489Glu)
-
VUS
g.26420574T>C
g.26197705T>C
-
-
HADHA_000024
5 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs145422395
Germline
-
5/2795 individuals
-
-
-
Mohammed Faruq
+/.
2
-
c.1493A>G
r.(?)
p.(His498Arg)
-
pathogenic (recessive)
g.26418088T>C
g.26195219T>C
-
-
HADHA_000059
-
PubMed: De Biase 2017
,
PubMed: Grunert 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.1528G>A
r.(?)
p.(Glu510Lys)
-
pathogenic (recessive)
g.26418053C>T
g.26195184C>T
-
-
HADHA_000038
-
PubMed: Randon 2020
-
-
Germline
-
2/1000 control individuals
-
-
-
Johan den Dunnen
+/., +?/.
132
15, 9
c.1528G>C
r.(?), r.1528g>c
p.(Glu510Gln), p.Glu510Gln
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.26418053C>G
g.26191522C>T, g.26195184C>G
E474Q, G1528C, G1528C (E474Q), Glu474Gln, HADHA, c.1528G>C, p.Glu510Gln, homozygous
-
HADHA_000005
ACMG PP3, PM2, PP5_STRONG, no variant 2nd chromosome, unknown variant 2nd chromosome
PubMed: Boutron 2011
,
PubMed: Chen 2024
,
PubMed: De Biase 2017
,
PubMed: Diebold 2019
,
20 more items
100085
-
Germline, Germline/De novo (untested), Unknown
?
-
-
-
-
Johan den Dunnen
,
Belen Perez
+/.
1
-
c.1533dup
r.(?)
p.(Ile512TyrfsTer29)
-
pathogenic (recessive)
g.26418049dup
g.26195180dup
-
-
HADHA_000078
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.1561_1562del
r.(?)
p.(Thr521GlnfsTer19)
-
pathogenic (recessive)
g.26418021_26418022del
g.26195152_26195153del
-
-
HADHA_000077
no variant 2nd chromosome
PubMed: Diebold 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
15i
c.1620+2_1620+6del
r.spl
p.?
-
pathogenic (recessive)
g.26417960_26417964del
g.26195091_26195095del
del1617-+1
-
HADHA_000062
-
PubMed: Ibdah 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.1620+9C>G
r.(=)
p.(=)
-
likely benign
g.26417952G>C
g.26195083G>C
HADHA(NM_000182.4):c.1620+9C>G
-
HADHA_000018
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
-
c.1620+11G>C
r.(=)
p.(=)
-
benign
g.26417950C>G
-
HADHA(NM_000182.5):c.1620+11G>C
-
HADHA_000035
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
-
c.1663_1665del
r.(?)
p.(Met555del)
-
pathogenic (recessive)
g.26417466_26417468del
g.26194597_26194599del
-
-
HADHA_000076
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
5
16
c.1678C>T
r.(?), r.1678c>u
p.(Arg560*), p.(Arg560Ter), p.Arg560*
-
pathogenic, pathogenic (recessive)
g.26417450G>A
g.26194581G>A
Arg524Ter, C1678T (R524Stop)
-
HADHA_000032
significantly reduced RNA expression of this allele, VKGL data sharing initiative Nederland
PubMed: Boutron 2011
,
PubMed: Ibdah 1999
,
PubMed: Isaacs 1996
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
+/.
5
-
c.1689+2T>G
r.(1621_1689del), r.1621_1689del, r.spl
p.(Asp541_Gln563del), p.?, p.Asp541_Gln563del
-
pathogenic (recessive)
g.26417437A>C
g.26194568A>C
-
-
HADHA_000058
-
PubMed: Bo 2017
,
PubMed: Choi 2007
,
PubMed: Kang 2018
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.1690-6G>A
r.(=)
p.(=)
-
likely benign
g.26416647C>T
-
HADHA(NM_000182.5):c.1690-6G>A
-
HADHA_000034
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
2
-
c.1712T>C
r.(?)
p.(Leu571Pro)
-
pathogenic (recessive)
g.26416619A>G
g.26193750A>G
-
-
HADHA_000060
-
PubMed: Bursle 2018
,
PubMed: Diekman 2014
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
4
-
c.1793_1794del
r.(?)
p.(His598Argfs*33), p.(His598ArgfsTer33)
-
pathogenic (recessive)
g.26416537_26416538del
g.26193668_26193669del
1793-94delAT, 1793_1974del (H598Rfs*33)
-
HADHA_000057
-
PubMed: Hintz 2002
,
PubMed: Kang 2018
,
PubMed: Spiekerkoetter 2002
-
-
Germline, Uniparental disomy, maternal allele
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.1795G>A
r.(?)
p.(Val599Met)
-
pathogenic (recessive)
g.26416536C>T
g.26193667C>T
V563M
-
HADHA_000052
combination of variants not reported
PubMed: Spiekerkoetter 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
-
c.1828C>G
r.(?)
p.(Arg610Gly)
-
pathogenic (recessive)
g.26416503G>C
g.26193634G>C
-
-
HADHA_000075
-
PubMed: Grunert 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.1912A>G
r.(?)
p.(Ile638Val)
-
likely benign
g.26415267T>C
g.26192398T>C
-
-
HADHA_000017
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
18
c.1915_1918del
r.(?)
p.(Tyr639Argfs*4), p.(Tyr639ArgfsTer4)
-
pathogenic
g.26415264_26415267del
g.26192395_26192398del
1915_1918delTATC
-
HADHA_000004
-
PubMed: Navarrete 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
,
Belen Perez
-?/.
1
-
c.1961G>A
r.(?)
p.(Ser654Asn)
-
likely benign
g.26415218C>T
g.26192349C>T
HADHA(NM_000182.4):c.1961G>A (p.(Ser654Asn))
-
HADHA_000014
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
3
18
c.1967del
r.(?)
p.(Leu656Ter)
-
pathogenic (recessive)
g.26415215del
g.26192346del
Leu620Ter(delT1964)
-
HADHA_000074
-
PubMed: Boutron 2011
,
PubMed: Ibdah 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.1981C>T
r.(?)
p.(Leu661=)
-
likely benign
g.26415198G>A
g.26192329G>A
HADHA(NM_000182.4):c.1981C>T (p.L661=)
-
HADHA_000013
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.1981_1999del
r.(?)
p.(Leu661SerfsTer12)
-
pathogenic (recessive)
g.26415189_26415207del
g.26192320_26192338del
-
-
HADHA_000073
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.1990_1991del
r.(?)
p.(Lys664ValfsTer2)
-
pathogenic (recessive)
g.26415188_26415189del
g.26192319_26192320del
-
-
HADHA_000072
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
18i
c.2000+1G>A
r.spl
p.?
-
pathogenic (recessive)
g.26415178C>T
g.26192309C>T
IVS18+1G>A
-
HADHA_000051
-
PubMed: Spiekerkoetter 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
19
c.2026C>T
r.(?)
p.(Arg676Cys)
-
pathogenic (recessive)
g.26414472G>A
g.26191603G>A
Arg640Cys
-
HADHA_000071
-
PubMed: Ibdah 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
19, 9
c.2027G>A
r.(?)
p.(Arg676His)
-
pathogenic (recessive)
g.26414471C>T
g.26191602C>T
Arg640His
-
HADHA_000069
-
PubMed: Boutron 2011
,
PubMed: Ibdah 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.2027G>T
r.(?)
p.(Arg676Leu)
-
pathogenic (recessive)
g.26414471C>A
g.26191602C>A
-
-
HADHA_000070
-
PubMed: Diekman 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.2033T>C
r.(?)
p.(Val678Ala)
-
VUS
g.26414465A>G
-
HADHA(NM_000182.5):c.2033T>C (p.(Val678Ala))
-
HADHA_000036
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.2059del
r.(?)
p.(Met687CysfsTer43)
-
pathogenic (recessive)
g.26414439del
g.26191570del
-
-
HADHA_000068
no variant 2nd chromosome
PubMed: Diebold 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.2063G>A
r.(?)
p.(Cys688Tyr)
-
pathogenic (recessive)
g.26414435C>T
g.26191566C>T
-
-
HADHA_000067
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.2102A>G
r.(?)
p.(Asp701Gly)
-
pathogenic (recessive)
g.26414396T>C
g.26191527T>C
D665G
-
HADHA_000053
combination of variants not reported
PubMed: Spiekerkoetter 2004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.2107G>A
r.(?)
p.(Gly703Arg)
-
pathogenic (recessive)
g.26414391C>T
g.26191522C>T
-
-
HADHA_000048
-
PubMed: Yang 2022
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.2112C>T
r.(?)
p.(Ala704=)
-
likely benign
g.26414386G>A
g.26191517G>A
HADHA(NM_000182.5):c.2112C>T (p.A704=)
-
HADHA_000012
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/.
1
-
c.2113G>A
r.(?)
p.(Val705Ile)
-
VUS
g.26414385C>T
g.26191516C>T
-
-
HADHA_000023
1 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs146406360
Germline
-
1/2794 individuals
-
-
-
Mohammed Faruq
?/.
1
-
c.2120G>T
r.(?)
p.(Gly707Val)
-
VUS
g.26414378C>A
g.26191509C>A
HADHA(NM_000182.4):c.2120G>T (p.(Gly707Val))
-
HADHA_000011
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.2128_2129insC
r.(?)
p.(Phe710Serfs*28)
-
pathogenic (recessive)
g.26414369_26414370insG
g.26191500_26191501insG
C2129ins
-
HADHA_000044
variant might also be c.2132dup
PubMed: IJlst 1997
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.2146+1G>A
r.spl
p.?
-
pathogenic (recessive)
g.26414351C>T
g.26191482C>T
-
-
HADHA_000066
-
PubMed: Vieira Neto 2024
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.2146+6_2146+18del
r.2146_2147ins[guugg;2146+19_2147-1]
p.Pro717TrpfsTer7
-
pathogenic (recessive)
g.26414341_26414353del
g.26191472_26191484del
-
-
HADHA_000065
-
PubMed: Boutron 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.2146+16T>C
r.(=)
p.(=)
-
benign
g.26414336A>G
-
HADHA(NM_000182.5):c.2146+16T>C
-
HADHA_000033
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
-
c.2176G>A
r.(?)
p.(Ala726Thr)
-
VUS
g.26414235C>T
g.26191366C>T
HADHA(NM_000182.5):c.2176G>A (p.A726T)
-
HADHA_000010
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
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