Global Variome shared LOVD
TAZ (tafazzin)
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Global Variome, with Curator vacancy
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Unique variants in the TAZ gene
The variants collected by the
Barth Syndrome Foundation
were used to initiate this database
The variants shown are described using the NM_000116.3 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
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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'
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281 entries on 3 pages. Showing entries 1 - 100.
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Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
./.
1
-
c.-74340_*1073086dup
-
-
-
pathogenic
g.153565841_154722429dup
g.154337626_155494214dup
-
-
DKC1_000000
increased gene dosage
PubMed: DDDS 2015
,
Journal: DDDS 2015
-
-
Germline
-
-
-
-
-
Johan den Dunnen
./.
1
-
c.-69773_*211765dup
r.0?
p.0?
-
pathogenic
g.153570408_153861108dup
g.154342193_154632893dup
GDI1
-
FLNA_000307
-
PubMed: Hu 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.-12436G>C
r.(?)
p.(=)
-
likely benign
g.153627745G>C
-
RPL10(NM_001303625.1):c.82+8G>C
-
RPL10_000035
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.-12339A>G
r.(?)
p.(=)
-
VUS
g.153627842A>G
g.154399501A>G
RPL10(NM_001303625.1):c.97A>G (p.I33V)
-
RPL10_000013
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
-
c.-12010A>G
r.(?)
p.(=)
-
pathogenic
g.153628171A>G
g.154399830A>G
RPL10(NM_001303625.1):c.218A>G (p.N73S)
-
RPL10_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
-?/.
1
-
c.-11384C>T
r.(?)
p.(=)
-
likely benign
g.153628797C>T
g.154400456C>T
RPL10(NM_001303625.1):c.330-8C>T
-
RPL10_000005
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
1
-
c.-11248G>A
r.(?)
p.(=)
-
likely pathogenic
g.153628933G>A
g.154400592G>A
-
-
RPL10_000011
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.-11066C>T
r.(?)
p.(=)
-
VUS
g.153629115C>T
g.154400774C>T
RPL10(NM_006013.3):c.565C>T (p.(Arg189Trp))
-
RPL10_000014
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
1
-
c.-11026A>G
r.(?)
p.(=)
-
benign
g.153629155A>G
g.154400814=
RPL10(NM_006013.5):c.605A>G (p.N202S)
-
RPL10_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-?/.
1
-
c.-11023G>A
r.(?)
p.(=)
-
likely benign
g.153629158G>A
-
RPL10(NM_001303625.1):c.608G>A (p.R203H)
-
RPL10_000034
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.-10998C>T
r.(?)
p.(=)
-
likely benign
g.153629183C>T
g.154400842C>T
RPL10(NM_001256577.1):c.470C>T (p.(Pro157Leu))
-
RPL10_000006
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.-10937C>G
r.(?)
p.(=)
-
VUS
g.153629244C>G
g.154400903C>G
RPL10(NM_001256577.2):c.531C>G (p.Y177*)
-
RPL10_000015
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.-10933C>T
r.(?)
p.(=)
-
likely benign
g.153629248C>T
g.154400907C>T
RPL10(NM_001256577.2):c.535C>T (p.L179=)
-
RPL10_000008
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
-
c.-8890C>T
r.(?)
p.(=)
-
VUS
g.153631291C>T
g.154402950C>T
DNASE1L1(NM_001009932.1):c.766G>A (p.(Glu256Lys))
-
DNASE1L1_000023
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.-7020G>C
r.(?)
p.(=)
-
VUS
g.153633161G>C
g.154404820G>C
DNASE1L1(NM_001009932.1):c.311+8C>G (p.(=))
-
DNASE1L1_000024
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.-6845C>T
r.(?)
p.(=)
-
likely benign
g.153633336C>T
g.154404995C>T
DNASE1L1(NM_006730.3):c.224G>A (p.R75Q)
-
DNASE1L1_000025
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.-6832G>A
r.(?)
p.(=)
-
VUS
g.153633349G>A
g.154405008G>A
DNASE1L1(NM_006730.3):c.211C>T (p.R71*)
-
RPL10_000025
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.-6399A>G
r.(?)
p.(=)
-
VUS
g.153633782A>G
g.154405441A>G
DNASE1L1(NM_006730.3):c.128T>C (p.L43S)
-
RPL10_000016
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.-6285G>A
r.(?)
p.(=)
-
VUS
g.153633896G>A
g.154405555G>A
DNASE1L1(NM_001009932.1):c.14C>T (p.(Thr5Ile))
-
DNASE1L1_000026
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
_1_11_
c.-1390_*4002del
r.0?
p.0?
-
pathogenic
g.153638793_153653347del
g.154410451_154425005del
TAZ deletion
-
TAZ_000005
complete deletion, Alu mediated, 15 kb
PubMed: Singh 2009
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
-?/.
2
1
c.-121del
r.(?)
p.(=)
-
likely benign
g.153640060del
g.154411724del
-
-
TAZ_000203
-
-
-
-
Germline
-
-
-
-
-
Yu Sun
-/.
3
1
c.-120del
r.(?)
p.(=)
-
benign
g.153640061del
-
-119ins/del T
-
RPL10_000017, TAZ_000003
VKGL data sharing initiative Nederland
DB Barth Syndrome Foundation
Kiriwn/Gonzalez (Wilmington, USA),
1 more item
-
rs11388353
CLASSIFICATION record, Unknown
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
-/.
1
-
c.-120dup
r.(?)
p.(=)
-
benign
g.153640061dup
-
TAZ(NM_000116.3):c.-120dupT
-
RPL10_000018
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
4
1
c.-88G>C
r.(?)
p.(=)
-
benign
g.153640093G>C
g.154411756G>C
-
-
TAZ_000004
VKGL data sharing initiative Nederland
DB Barth Syndrome Foundation
Kiriwn/Gonzalez (Wilmington, USA),
2 more items
-
rs113130344
CLASSIFICATION record, Germline, Unknown
-
3/380 chromosomes
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
+?/.
1
1
c.-17C>T
r.(?)
p.(=)
-
likely pathogenic
g.153640164C>T
g.154411827C>T
-
-
TAZ_000006
-
DB Barth Syndrome Foundation
Harvard Partners (USA)
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
2
_1_4i
c.(?_-1)_(370+1_371-1)del
r.0?
p.0?
-
pathogenic
g.153640180_153641905del, g.153640181_153641905del
-
Ex1_Ex4del
-
TAZ_000000
1 more item
DB Barth Syndrome Foundation
- reported by family,
DB Barth Syndrome Foundation
Harvard Partners (USA)
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
5
_1_5i
c.(?_-1)_(460+1_461-1)del
r.0?
p.0?
-
pathogenic
g.153640180_153642528del
-
Ex1_Ex5 del
-
TAZ_000000
3 more items
DB Barth Syndrome Foundation
- reported by family,
PubMed: Rigaud 2013
-
-
Germline, Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
_1_11_
c.(?_-1)_(*1_?)del
r.0?
p.0?
-
pathogenic
g.153640180_153649344del
-
complete deletion
-
TAZ_000000
1 more item
DB Barth Syndrome Foundation
Kiriwn/Gonzalez (Wilmington, USA)
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.1A>G
r.(?)
p.(Met1?)
-
likely pathogenic
g.153640181A>G
-
-
-
RPL10_000036
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
1
c.9dup
r.(?)
p.(His4Alafs*130)
-
pathogenic
g.153640189dup
g.154411852dup
insG297
-
TAZ_000027
de novo from mother
PubMed: Cantlay 1999
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+?/.
1
1
c.13G>T
r.(?)
p.(Val5Leu)
-
likely pathogenic
g.153640193G>T
g.154411856G>T
-
-
TAZ_000038
-
DB Barth Syndrome Foundation
Harvard Partners (USA)
-
rs397515737
Unknown
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.30C>A
r.(?)
p.(Pro10=)
-
VUS
g.153640210C>A
g.154411873C>A
-
-
RPL10_000019
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.44T>G
r.(?)
p.(Leu15Arg)
-
VUS
g.153640224T>G
g.154411887T>G
-
-
RPL10_000026
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.45C>T
r.(?)
p.(Leu15=)
-
likely benign
g.153640225C>T
g.154411888C>T
-
-
RPL10_000020
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.45_46insT
r.(?)
p.(Thr16TyrfsTer118)
-
VUS
g.153640225_153640226insT
g.154411888_154411889insT
-
-
RPL10_000027
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
1
c.48C>G
r.(?)
p.(Thr16=)
-
benign
g.153640228C>G
g.154411891C>G
-
-
TAZ_000126
MYBPC3 .Arg1226Leu likely path VUS
DB Barth Syndrome Foundation
Harvard Partners (USA)
-
rs397515743
Unknown
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.50G>T
r.(?)
p.(Trp17Leu)
-
VUS
g.153640230G>T
g.154411893G>T
-
-
RPL10_000028
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
1
c.51G>A
r.(?)
p.(Trp17*)
-
pathogenic
g.153640231G>A
g.154411894G>A
-
-
TAZ_000131
-
DB Barth Syndrome Foundation
MGZ (Munchen, Germany), {PMID:Steward 2011:
DB Barth Syndrome Foundation
}
-
rs104894941
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.51G>C
r.(?)
p.(Trp17Cys)
-
benign
g.153640231G>C
g.154411894G>C
-
-
RPL10_000029
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.52A>G
r.(?)
p.(Thr18Ala)
-
VUS
g.153640232A>G
g.154411895A>G
-
-
RPL10_000030
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.53C>G
r.(?)
p.(Thr18Ser)
-
likely benign
g.153640233C>G
g.154411896C>G
-
-
RPL10_000031
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.53C>T
r.(?)
p.(Thr18Ile)
-
VUS
g.153640233C>T
g.154411896C>T
-
-
RPL10_000032
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
1
c.54_55del
r.54_55del
p.Leu19Glyfs*114
-
pathogenic
g.153640234_153640235del
g.154411897_154411898del
53_54delCC
-
TAZ_000001
CL6/MLCL5
PubMed: Johnston 1997
,
PubMed: Schlame 2003
,
PubMed: Gonzalez 2005
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.62G>T
r.(?)
p.(Ser21Ile)
-
pathogenic
g.153640242G>T
g.154411905G>T
-
-
TAZ_000161
-
-
-
rs112148852
Unknown
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.69C>A
r.(?)
p.(=)
-
likely benign
g.153640249C>A
-
TAFAZZIN(NM_000116.5):c.69C>A (p.V23=)
-
RPL10_000040
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+/.
1
1
c.83T>A
r.(?)
p.(Val28Glu)
-
pathogenic
g.153640263T>A
g.154411926T>A
-
-
TAZ_000199
-
DB Barth Syndrome Foundation
- BZapała (Krakow, Poland)
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.83_85del
r.(?)
p.(Val28del)
-
pathogenic
g.153640263_153640265del
g.154411926_154411928del
82_84delGTG
-
TAZ_000197
de novo from mother; CL6/MLCL5
{PMID:Steward 2011:
DB Barth Syndrome Foundation
},
PubMed: Kirwin 2014
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
1
c.86G>A
r.(?)
p.(Gly29Asp)
-
pathogenic
g.153640266G>A
g.154411929G>A
-
-
TAZ_000200
-
PubMed: Mazurova 2013
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.89C>T
r.(?)
p.(Thr30Ile)
-
VUS
g.153640269C>T
-
TAFAZZIN(NM_000116.5):c.89C>T (p.T30I)
-
RPL10_000038
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
1i
c.109+1G>C
r.[109_110ins109+1_109+54]
p.Lys37Ser_Tyr38ins18
-
pathogenic
g.153640290G>C
g.154411953G>C
-
-
TAZ_000050
de novo, maternal gonadal mosaicism
PubMed: Chang 2010
,
PubMed: Momoi 2012
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
1i
c.109+5G>A
r.[109_110ins109+1_109+54]
p.Lys37Ser_Tyr38ins18
-
pathogenic
g.153640294G>A
g.154411957G>A
-
-
TAZ_000002
CL 6
PubMed: Johnston 1997
,
PubMed: Schlame 2003
,
PubMed: Gonzalez 2005
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
1i
c.109+5G>C
r.[109_110ins109+1_109+54]
p.Lys37Ser_Tyr38ins18
-
pathogenic
g.153640294G>C
g.154411957G>C
-
-
TAZ_000010
CL 6
PubMed: Johnston 1997
,
PubMed: Schlame 2003
,
PubMed: Kelley 1991
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
1i
c.109+6T>C
r.spl
p.(=)
-
pathogenic
g.153640295T>C
g.154411958T>C
-
-
TAZ_000020
mRNA analysed but effect on splicing not described
PubMed: Mazurova 2013
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
1i
c.109+28C>T
r.(=)
p.(=)
-
benign
g.153640317C>T
g.154411980C>T
-
-
TAZ_000015
-
DB Barth Syndrome Foundation
Harvard Partners (USA)
-
rs397515736
Unknown
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.110-29G>T
r.(=)
p.(=)
-
likely benign
g.153640394G>T
g.154412057G>T
-
-
RPL10_000021
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
6
1i
c.110-17C>T
r.(=), r.=
p.(=), p.=
-
benign
g.153640406C>T
g.154412069=
1 more item
-
TAZ_000021
no effect on splicing, VKGL data sharing initiative Nederland
DB Barth Syndrome Foundation
Kiriwn/Gonzalez (Wilmington, USA),
PubMed: Gonzalez 2005
,
1 more item
-
rs62617809
CLASSIFICATION record, Unknown
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
,
VKGL-NL_AMC
-/.
1
-
c.110-13C>T
r.(=)
p.(=)
-
benign
g.153640410C>T
-
TAFAZZIN(NM_000116.5):c.110-13C>T
-
RPL10_000039
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
-?/.
1
-
c.110-11T>C
r.(=)
p.(=)
-
likely benign
g.153640412T>C
g.154412075T>C
-
-
RPL10_000022
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/., -?/.
2
-
c.110-6C>T
r.(=)
p.(=)
-
benign, likely benign
g.153640417C>T
g.154412080C>T
TAZ(NM_000116.3):c.110-6C>T (p.(=))
-
DNASE1L1_000028
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
-?/.
1
-
c.110-3del
r.spl?
p.?
-
likely benign
g.153640420del
g.154412083del
TAZ(NM_001303465.1):c.165-3delC
-
RPL10_000033
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
3
1i
c.110-2A>G
r.spl, r.[110_238del, (110_111del)]
p.?, p.[Lys37_Gly80del, Lys37fs]
-
pathogenic
g.153640421A>G
g.154412084A>G
398-2A>G
-
TAZ_000054
-
DB Barth Syndrome Foundation
Kiriwn/Gonzalez (Wilmington, USA),
PubMed: Ichida 2001
,
1 more item
-
-
Germline, Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
1i
c.110-1G>C
r.spl
p.?
-
pathogenic
g.153640422G>C
g.154412085G>C
IVS1-1G>C
-
TAZ_000023
CL 6
PubMed: Valianpour 2002
,
PubMed: Kuijpers 2004
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
3
2
c.118A>G
r.(?)
p.(Asn40Asp), p.Asn40Asp
-
NA, pathogenic
g.153640431A>G
g.154412094A>G
-
-
TAZ_000024
functional analysis yeast; y.Asn48Asp, MLCL normal; 37oC expression 0.30,
1 more item
PubMed: Bowron 2014
,
PubMed: Claypool 2011
,
PubMed: Whited 2013
,
1 more item
-
-
In vitro (cloned), Unknown
-
-
-
-
-
Johan den Dunnen
-/.
1
2
c.123C>T
r.(?)
p.(His41=)
-
benign
g.153640436C>T
g.154412099C>T
-
-
TAZ_000033
-
-
-
rs368329470
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
2
2
c.124del
r.(?)
p.(Leu42*)
-
pathogenic
g.153640437del
g.154412100del
123delC
-
TAZ_000032
CL 6
PubMed: Ichida 2001
,
1 more item
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.127A>C
r.(?)
p.(Thr43Pro)
-
pathogenic
g.153640440A>C
g.154412103A>C
-
-
TAZ_000049
-
PubMed: Bachou 2009
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
3
2
c.140_152del
r.(?), r.140_152del
p.Arg47Thrfs*32
-
pathogenic
g.153640453_153640465del
g.154412116_154412128del
140_152del13 [GGGAGGTGCTGTA], 428del13
-
TAZ_000055
CL 6, de novo from grandfather; CL 6
PubMed: D'Adamo 1997
,
PubMed: Vreeken 2000
,
PubMed: Schlame 2003
,
PubMed: Gonzalez 2005
,
1 more item
-
-
De novo, Unknown
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.142G>A
r.(?)
p.(Glu48Lys)
-
VUS
g.153640455G>A
g.154412118G>A
TAZ(NM_000116.3):c.142G>A (p.E48K)
-
TAZ_000207
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.142G>C
r.(?)
p.(Glu48Gln)
-
VUS
g.153640455G>C
g.154412118G>C
TAFAZZIN(NM_000116.5):c.142G>C (p.E48Q)
-
RPL10_000023
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
2
c.143delinsGG
r.(?)
p.(Glu48Glyfs*86)
-
pathogenic
g.153640456delinsGG
g.154412119delinsGG
-
-
TAZ_000043
CL6/MLCL5
PubMed: Rigaud 2013
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
3
2
c.149T>C
r.(?)
p.(Leu50Pro)
-
pathogenic
g.153640462T>C
g.154412125T>C
-
-
TAZ_000051
CL6/MLCL5
DB Barth Syndrome Foundation
Bowles (Houston, USA),
2 more items
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
3
2
c.153C>G
r.(?)
p.(Tyr51*)
-
pathogenic
g.153640466C>G
g.154412129C>G
C441G
-
TAZ_000017
CL 6
DB Barth Syndrome Foundation
Kiriwn/Gonzalez (Wilmington, USA),
PubMed: Yen 2008
,
2 more items
-
rs104894941
Germline, Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.157dup
r.(?)
p.(Leu53Profs*81)
-
pathogenic
g.153640470dup
g.154412133dup
-
-
TAZ_000052
-
PubMed: Xing 2006
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.161T>A
r.(?)
p.(Ile54Asn)
-
pathogenic
g.153640474T>A
g.154412137T>A
-
-
TAZ_000064
-
DB Barth Syndrome Foundation
Bowles (Houston, USA)
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.163G>T
r.(?)
p.(Glu55*)
-
pathogenic
g.153640476G>T
g.154412139G>T
-
-
TAZ_000066
-
DB Barth Syndrome Foundation
Kiriwn/Gonzalez (Wilmington, USA)
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
3
2
c.170G>T
r.(?)
p.(Arg57Leu), p.Arg57Leu
-
NA, pathogenic
g.153640483G>T
g.154412146G>T
-
-
TAZ_000067
CL 6, functional analysis yeast; y.Lys65Leu, CL reduced, MLCL raised, 37oC expression 0.30,
1 more item
PubMed: Claypool 2011
,
PubMed: Whited 2013
,
PubMed: Kuijpers 2004
,
1 more item
-
-
Germline, In vitro (cloned), Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.171del
r.171del
p.Gly58Alafs*25
-
pathogenic
g.153640484del
g.154412147del
-
-
TAZ_000068
de novo from grandfather; CL6/MLCL5
PubMed: Johnston 1997
,
PubMed: Schlame 2003
,
PubMed: Gonzalez 2005
,
PubMed: Kirwin 2014
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.185C>T
r.(?)
p.(Pro62Leu)
-
pathogenic
g.153640498C>T
g.154412161C>T
-
-
TAZ_000069
-
DB Barth Syndrome Foundation
MGZ (Munchen, Germany)
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.197T>A
r.(?)
p.(Val66Glu)
-
pathogenic
g.153640510T>A
-
-
-
RPL10_000037
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
3
2
c.207C>G
r.(?)
p.(His69Gln), p.His69Gln
-
NA, pathogenic
g.153640520C>G
g.154412183C>G
-
-
TAZ_000019
CL6/MLCL5, functional analysis yeast; y.His77Gln, MLC raised, catalytically null
DB Barth Syndrome Foundation
- reported by family,
PubMed: Claypool 2011
,
PubMed: Whited 2013
,
1 more item
-
-
Germline, In vitro (cloned), Unknown
-
-
-
-
-
Johan den Dunnen
+/., +?/.
2
2
c.208C>T
r.(?)
p.(Gln70*)
-
likely pathogenic, pathogenic
g.153640521C>T
g.154412184C>T
-
-
TAZ_000070
TTN Arg1012X likely path
DB Barth Syndrome Foundation
Harvard Partners (USA),
PubMed: Walsh 2017
-
-
Germline, Unknown
-
1/740 cases
-
-
-
Johan den Dunnen
+/.
3
2
c.211T>C
r.(?)
p.(Ser71Pro), p.Ser71Pro
-
NA, pathogenic
g.153640524T>C
g.154412187T>C
-
-
TAZ_000071
functional analysis yeast; y.Ser79Pro, MLCL raised, catalytically null
DB Barth Syndrome Foundation
Kiriwn/Gonzalez (Wilmington, USA),
2 more items
-
-
In vitro (cloned), Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.216C>A
r.(?)
p.(Cys72*)
-
pathogenic
g.153640529C>A
g.154412192C>A
-
-
TAZ_000072
CL6/MLCL5
DB Barth Syndrome Foundation
Honeychurch/Williams (Bristol, UK)
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.222C>G
r.(?)
p.(Asp74Glu)
-
pathogenic
g.153640535C>G
g.154412198C>G
-
-
TAZ_000073
-
DB Barth Syndrome Foundation
- reported by family
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.223G>C
r.(?)
p.(Asp75His)
-
pathogenic
g.153640536G>C
g.154412199G>C
-
-
TAZ_000074
de novo from mother
DB Barth Syndrome Foundation
- reported by family
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.227del
r.(?)
p.(Pro76Leufs*7)
-
pathogenic
g.153640540del
g.154412203del
-
-
TAZ_000075
-
PubMed: Kim 2013
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
2
c.236G>A
r.236g>a
p.Trp79*
-
pathogenic
g.153640549G>A
g.154412212G>A
-
-
TAZ_000076
de novo gr'grandfather; CL6/MLCL5
PubMed: Schlame 2003
,
PubMed: Gonzalez 2005
,
PubMed: Kirwin 2014
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
5
2
c.238G>A
r.(?), r.spl, r.[238_239ins238+1_238+36, =, 110_238del, ..]
p.(Gly80Arg), p.Gly80Arg, p.[Gly80Ser_Ile81ins(12), =, Lys37_Gly80delinsArg]
-
NA, pathogenic
g.153640551G>A
g.154412214G>A
-
-
TAZ_000077, TAZ_000078
CL6/MLCL5, RNA 0.81/0.05/0.08 and some other; CL6/MLCL5, RNA 0.82/0.07/0.04 and some other; CL6/MLCL5,
1 more item
Miller 2005 (ACC2005 Poster1142-245),
DB Barth Syndrome Foundation
Pennock/Williams (Bristol, UK),
2 more items
-
-
Germline, In vitro (cloned), Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.238G>C
r.[110_238del,238_239ins[238+1_238+36]]
p.[Lys37_Gly80delinsArg,Trp79_Gly80insArgThrArgAlaSerValLeuGlyArgGlyArgLys]
-
pathogenic (recessive)
g.153640551G>C
g.154412214G>C
-
-
TAZ_000226
exon skipping, cryptic splice donor; mother germline mosaicism
PubMed: Bournazos 2022
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/.
2
2i
c.238+2T>G
r.[110_238del, 238_239ins238+1_238+36, ..], r.[110_238del]
p.Lys37_Gly80del, p.[Lys37_Gly80del, Gly80Ser_Ile81ins(12)]
-
pathogenic
g.153640553T>G
g.154412216T>G
-
-
TAZ_000053
CL6/MLCL5, RNA 0.52/0.15 and some other; CL6/MLCL5
PubMed: Johnston 1997
,
PubMed: Schlame 2003
,
PubMed: Gonzalez 2005
,,
PubMed: Kirwin 2014
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.238+28G>A
r.(=)
p.(=)
-
benign
g.153640579G>A
g.154412242G>A
TAZ(NM_000116.3):c.238+28G>A
-
TAZ_000208
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-/.
1
2i
c.239-26C>A
r.(=)
p.(=)
-
benign
g.153641518C>A
g.154413181C>A
-
-
TAZ_000079
1 more item
Taylor 2011 (Cardiogenetics 1:9-12)
-
rs199809512
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
2i
c.239-1G>A
r.spl
p.?
-
pathogenic
g.153641543G>A
g.154413206G>A
-
-
TAZ_000056
CL 6
PubMed: Bione 1996
,
PubMed: D'Adamo 1997
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
3
2i
c.239-1G>C
r.spl
p.?
-
pathogenic
g.153641543G>C
g.154413206G>C
527-1C
-
TAZ_000057
CL6/MLCL5
PubMed: Bione 1996
,
PubMed: Vreeken 2000
,
PubMed: D'Adamo 1997
,
PubMed: van Werkhoven 2006
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
1
2i_5i
c.(238+1_239-1)_(460+1_461-1)del
r.(del)
p.?
-
pathogenic
g.153641543_153642528del
-
Ex3_Ex5del
-
TAZ_000000
1 more item
DB Barth Syndrome Foundation
- reported by family
-
-
De novo
-
-
-
-
-
Johan den Dunnen
+/.
2
3
c.239G>A
r.(?), r.spl?
p.(Gly80Glu), p.Gly80Glu
-
NA, pathogenic
g.153641544G>A
g.154413207G>A
-
-
TAZ_000080, TAZ_000081
1 more item
PubMed: Claypool 2011
,
PubMed: Whited 2013
,
1 more item
-
-
Germline, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.239_240insC
r.(?)
p.(Ile81Aspfs*53)
-
pathogenic
g.153641544_153641545insC
g.154413207_154413208insC
G527insC
-
TAZ_000061
CL 6
PubMed: Valianpour 2002
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
+/.
2
3
c.245T>C
r.(?)
p.(Leu82Pro), p.Leu82Pro
-
NA, pathogenic
g.153641550T>C
g.154413213T>C
-
-
TAZ_000082
de novo from grandmother, functional analysis yeast; y.Leu90Pro, MLCL raised; functional hypomorphic
PubMed: Claypool 2011
,
PubMed: Whited 2013
,
PubMed: Gonzalez 2005
-
-
De novo, In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
1
3
c.253C>T
r.(?)
p.(Arg85Cys)
-
pathogenic
g.153641558C>T
g.154413221C>T
-
-
TAZ_000083
-
Exome Variant Server
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
?/.
4
-
c.254G>A
r.(?)
p.(Arg85His)
-
VUS
g.153641559G>A
g.154413222G>A
1 more item
-
TAZ_000209
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_AMC
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