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.
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
Allele : On which allele is the variant located? Does not necessarily imply inheritance! 'Paternal' (confirmed or inferred), 'Maternal' (confirmed or inferred), 'Parent #1' or #2 for compound heterozygosity without having screened the parents, 'Unknown' for heterozygosity without having screened the parents, 'Both' for homozygozity.
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%)
Effect
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.1A>G
r.(?)
p.(Met1?)
-
pathogenic (recessive)
g.39969287A>G
g.41813035A>G
-
-
FKBP10_000112
-
PubMed: Li 2020
-
-
Germline
-
-
-
-
-
Xiuli Zhao
-?/.
-
c.12G>T
r.(?)
p.(=)
-
likely benign
g.39969298G>T
-
FKBP10(NM_021939.4):c.12G>T (p.(Ala4=))
-
FKBP10_000106
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
1
c.14del
r.(?)
p.(Gly5Alafs*154)
-
pathogenic
g.39969300del
g.41813048del
14delG
-
FKBP10_000020
-
PubMed: Schwarze 2013
-
-
Germline
-
-
-
-
-
Peter Byers
-?/.
-
c.21C>T
r.(?)
p.(=)
-
likely benign
g.39969307C>T
-
FKBP10(NM_021939.4):c.21C>T (p.P7=)
-
FKBP10_000107
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/+
1
c.21dup
r.(?)
p.(Ser8Glnfs*67)
-
pathogenic
g.39969307dup
-
21dupC
-
FKBP10_000015
-
PubMed: Caparrós-Martin 2013
-
-
Germline
-
-
-
-
-
Victor L Ruiz-Perez
+/+
1
c.21dup
r.(?)
p.(Ser8Glnfs*67)
-
pathogenic
g.39969307dup
-
21dupC
-
FKBP10_000015
-
PubMed: Trancozo 2019
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
?/.
-
c.106C>A
r.(?)
p.(Pro36Thr)
-
VUS
g.39969392C>A
g.41813140C>A
FKBP10(NM_021939.3):c.106C>A (p.P36T)
-
FKBP10_000081
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+
1
c.122_156del
r.(?)
p.(Leu41Glnfs*22)
-
pathogenic
g.39969408_39969442del
-
-
-
FKBP10_000007
-
PubMed: Kelley 2011
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
?/.
-
c.172G>A
r.(?)
p.(Glu58Lys)
-
VUS
g.39969458G>A
g.41813206G>A
FKBP10(NM_021939.3):c.172G>A (p.(Glu58Lys))
-
FKBP10_000073
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
1
c.179A>C
r.(?)
p.(Gln60Pro)
-
pathogenic
g.39969465A>C
-
-
-
FKBP10_000054
-
PubMed: Trancozo 2019
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
1
c.204delinsAAA
r.(?)
p.(His68Glnfs*92)
-
pathogenic
g.39969490delinsAAA
-
-
-
FKBP10_000028
-
PubMed: Moravej 2015
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
?/.
-
c.209A>G
r.(?)
p.(Asn70Ser)
-
VUS
g.39969495A>G
-
FKBP10(NM_021939.4):c.209A>G (p.(Asn70Ser))
-
FKBP10_000101
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
-
c.210C>G
r.(?)
p.(Asn70Lys)
-
VUS
g.39969496C>G
g.41813244C>G
FKBP10(NM_021939.4):c.210C>G (p.N70K)
-
FKBP10_000074
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
-
c.245+18C>T
r.(=)
p.(=)
-
benign
g.39969549C>T
g.41813297C>T
FKBP10(NM_021939.4):c.245+18C>T
-
FKBP10_000061
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
-?/.
-
c.246-283T>A
r.(=)
p.(=)
-
likely benign
g.39973027T>A
-
FKBP10(NM_021939.4):c.246-283T>A
-
FKBP10_000087
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
-?/.
-
c.246-5C>A
r.spl?
p.?
-
likely benign
g.39973305C>A
-
FKBP10(NM_021939.4):c.246-5C>A
-
FKBP10_000108
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
-
c.246-5C>G
r.spl?
p.?
-
likely benign
g.39973305C>G
g.41817053C>G
FKBP10(NM_021939.3):c.246-5C>G (p.?), FKBP10(NM_021939.4):c.246-5C>G
-
FKBP10_000062
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
-?/.
-
c.246-5C>G
r.spl?
p.?
-
likely benign
g.39973305C>G
-
FKBP10(NM_021939.3):c.246-5C>G (p.?), FKBP10(NM_021939.4):c.246-5C>G
-
FKBP10_000062
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
-
c.246-3dup
r.spl?
p.?
-
likely benign
g.39973307dup
-
FKBP10(NM_021939.3):c.246-3dupC (p.?)
-
FKBP10_000102
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
2
c.288dup
r.(?)
p.(Arg97Alafs*101)
-
pathogenic
g.39973352dup
-
288dupG
-
FKBP10_000021
-
PubMed: Schwarze 2013
-
-
Germline
-
-
-
-
-
Peter Byers
+?/.
-
c.310C>T
r.(?)
p.(Arg104Ter)
-
likely pathogenic
g.39973374C>T
-
FKBP10(NM_021939.3):c.310C>T (p.(Arg104Ter))
-
FKBP10_000037
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
2
c.310C>T
r.(?)
p.(Arg104*)
-
pathogenic
g.39973374C>T
-
-
-
FKBP10_000037
-
PubMed: Caparros-Martin 2016
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
2
c.321_353del
r.(?)
p.(Met107_Leu117del)
-
pathogenic
g.39973385_39973417del
-
-
-
FKBP10_000001
-
PubMed: Alanay 2010
-
-
Germline
-
-
-
-
-
Peter Byers
+/.
-
c.321_353del
r.(?)
p.(Met107_Leu117del)
-
pathogenic
g.39973385_39973417del
g.41817133_41817165del
-
-
FKBP10_000001
-
PubMed: Tuysuz 2022
VCV000631496.2
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
-
c.321_353del
r.(?)
p.(Met107_Leu117del)
-
pathogenic
g.39973385_39973417del
g.41817133_41817165del
-
-
FKBP10_000001
-
PubMed: Tuysuz 2022
VCV000631496.2
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
-
c.321_353del
r.(?)
p.(Met107_Leu117del)
-
pathogenic
g.39973385_39973417del
g.41817133_41817165del
-
-
FKBP10_000001
-
PubMed: Tuysuz 2022
VCV000631496.2
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
-
c.321_353del
r.(?)
p.(Met107_Leu117del)
-
pathogenic
g.39973385_39973417del
g.41817133_41817165del
-
-
FKBP10_000001
-
PubMed: Tuysuz 2022
VCV000631496.2
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
-
c.321_353del
r.(?)
p.(Met107_Leu117del)
-
pathogenic
g.39973385_39973417del
g.41817133_41817165del
-
-
FKBP10_000001
-
PubMed: Tuysuz 2022
VCV000631496.2
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
-
c.321_353del
r.(?)
p.(Met107_Leu117del)
-
pathogenic
g.39973385_39973417del
g.41817133_41817165del
-
-
FKBP10_000001
ACMG
PubMed: Tuysuz 2022
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
c.322_355del
r.(?)
p.(Gly108CysfsTer40)
-
pathogenic (recessive)
g.39973386_39973419del
g.41817134_41817167del
320_353del (107_118del)
-
FKBP10_000113
-
PubMed: Li 2020
-
-
Germline
-
-
-
-
-
Xiuli Zhao
+/.
-
c.337G>A
r.(?)
p.(Glu113Lys)
-
pathogenic (recessive)
g.39973401G>A
g.41817149G>A
NM_021939.3:c.337G>A:p.(Glu113Lys)
-
FKBP10_000022
-
PubMed: Maddirevula 2018
-
-
Germline
-
-
-
-
-
LOVD
+/+
2
c.337G>A
r.(?)
p.(Glu113Lys)
-
pathogenic
g.39973401G>A
-
-
-
FKBP10_000022
-
PubMed: Schwarze 2013
-
-
Germline
-
-
-
-
-
Peter Byers
+/+
2
c.337G>A
r.(?)
p.(Glu113Lys)
-
pathogenic
g.39973401G>A
-
-
-
FKBP10_000022
-
PubMed: Schwarze 2013
-
-
Germline
-
-
-
-
-
Peter Byers
+/.
-
c.343C>T
r.(?)
p.(Arg115Ter)
-
pathogenic
g.39973407C>T
g.41817155C>T
FKBP10(NM_021939.4):c.343C>T (p.R115*)
-
FKBP10_000033
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
+/.
2
c.343C>T
r.(?)
p.(Arg115*)
-
pathogenic (recessive)
g.39973407C>T
g.41817155C>T
-
-
FKBP10_000033
-
PubMed: Liu 2017
-
-
Germline
-
1/101 cases OI
-
-
-
Johan den Dunnen
+/+
2
c.343C>T
r.(?)
p.(Arg115*)
-
pathogenic
g.39973407C>T
-
-
-
FKBP10_000033
The protein-level variant description for the paternal allele is incorrectly described by the authors as p.A362fsX1.; The family in this study was presented again as Family 30 by {PMID28725987:Liu et al., 2017} with a phenotype of OI III.
PubMed: Xu 2017
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
+/?
2
c.343C>T
r.(?)
p.(Arg115*)
-
pathogenic
g.39973407C>T
-
-
-
FKBP10_000033
-
PubMed: Vorster 2017
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
+/.
-
c.343C>T
r.(?)
p.(Arg115*)
-
pathogenic (recessive)
g.39973407C>T
-
-
-
FKBP10_000033
-
PubMed: Tan 2023
-
-
Germline/De novo (untested)
-
-
-
-
-
Kim Worring
+/+?
2
c.344G>A
r.(?)
p.(Arg115Gln)
-
pathogenic
g.39973408G>A
-
-
-
FKBP10_000009
No supporting evidence is presented that the misense p.(Arg115Gln) variant is disease-causing.; Kelley et al., classify this patient as having Bruck syndrome type 1, but that type maps to 17p12 ({PMID9927692:Bank et al., 1999}), whereas FKBP10 maps to 17q21.2.
PubMed: Kelley 2011
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
2
c.344G>A
r.(?)
p.(Arg115Gln)
-
pathogenic
g.39973408G>A
-
-
-
FKBP10_000009
-
PubMed: Schwarze 2013
-
-
Germline
-
-
-
-
-
Peter Byers
+/+
2
c.344G>A
r.(?)
p.(Arg115Gln)
-
pathogenic
g.39973408G>A
-
-
-
FKBP10_000009
-
PubMed: Umair 2016
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
2
c.344G>A
r.(?)
p.(Arg115Gln)
-
pathogenic (recessive)
g.39973408G>A
g.41817156G>A
-
-
FKBP10_000009
-
PubMed: Li 2019 , Journal: Li 2019 , PubMed: Li 2020
-
-
Germline
-
-
-
-
-
Xiuli Zhao
+/.
2
c.344G>A
r.(?)
p.(Arg115Gln)
-
pathogenic (recessive)
g.39973408G>A
g.41817156G>A
-
-
FKBP10_000009
-
PubMed: Li 2020
-
-
Germline
-
-
-
-
-
Xiuli Zhao
+/.
2
c.344G>A
r.(?)
p.(Arg115Gln)
-
pathogenic (recessive)
g.39973408G>A
g.41817156G>A
-
-
FKBP10_000009
-
PubMed: Li 2020
-
-
Germline
-
-
-
-
-
Xiuli Zhao
+?/.
-
c.354delT
r.(?)
p.(Ile118Metfs*41)
-
likely pathogenic
g.39973418del
g.41817166del
FKBP10 c.354delT, (p.Ile118Metfs*41)
-
FKBP10_000093
homozygous
PubMed: Alabdullatif 2017
-
-
Germline
yes
-
-
-
-
LOVD
?/.
-
c.391+3G>A
r.spl?
p.?
-
VUS
g.39973458G>A
-
FKBP10(NM_021939.4):c.391+3G>A
-
FKBP10_000109
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
2i
c.391+4A>T
r.spl
p.?
-
pathogenic
g.39973459A>T
-
-
-
FKBP10_000031
This variant was identified in 8 affected members of the same family. The variant causes a skip of exon 2.; This patient might be related to patient AN_005834 who is homozygous for the same variant and is also from the same city.
PubMed: Essawi 2018
-
-
Germline
-
-
-
-
-
Sofie Symoens
+/+
2i
c.391+4A>T
r.spl
p.?
-
pathogenic
g.39973459A>T
-
-
-
FKBP10_000031
-
PubMed: Essawi 2018
-
-
Germline
-
-
-
-
-
Sofie Symoens
-?/.
-
c.392-4A>G
r.spl?
p.?
-
likely benign
g.39974337A>G
-
FKBP10(NM_021939.4):c.392-4A>G
-
FKBP10_000115
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
3
c.407C>T
r.(?)
p.(Pro136Leu)
-
pathogenic
g.39974356C>T
-
-
-
FKBP10_000023
-
PubMed: Schwarze 2013
-
-
Germline
-
-
-
-
-
Peter Byers
-?/.
-
c.420C>G
r.(?)
p.(Leu140=)
-
likely benign
g.39974369C>G
g.41818117C>G
FKBP10(NM_021939.4):c.420C>G (p.L140=)
-
FKBP10_000064
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
?/.
-
c.429T>G
r.(?)
p.(Asp143Glu)
-
VUS
g.39974378T>G
-
FKBP10(NM_021939.3):c.429T>G (p.(Asp143Glu))
-
FKBP10_000096
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
-
c.582-4G>T
r.spl?
p.?
-
likely benign
g.39974630G>T
-
FKBP10(NM_021939.4):c.582-4G>T
-
FKBP10_000086
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
-/.
-
c.590A>G
r.(?)
p.(Lys197Arg)
-
benign
g.39974642A>G
g.41818390A>G
FKBP10(NM_021939.3):c.590A>G (p.(Lys197Arg)), FKBP10(NM_021939.4):c.590A>G (p.K197R)
-
FKBP10_000003
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
-?/.
-
c.590A>G
r.(?)
p.(Lys197Arg)
-
likely benign
g.39974642A>G
g.41818390A>G
FKBP10(NM_021939.3):c.590A>G (p.(Lys197Arg)), FKBP10(NM_021939.4):c.590A>G (p.K197R)
-
FKBP10_000003
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
-
c.590A>G
r.(?)
p.(Lys197Arg)
-
VUS
g.39974642A>G
g.41818390A>G
-
-
FKBP10_000003
conflicting interpretations of pathogenicity; 176 heterozygous; Clinindb (India)
PubMed: Narang 2020 , Journal: Narang 2020
-
rs34764749
Germline
-
176/2794 individuals
-
-
-
Mohammed Faruq
?/.
-
c.590A>G
r.(?)
p.(Lys197Arg)
-
VUS
g.39974642A>G
g.41818390A>G
-
-
FKBP10_000003
conflicting interpretations of pathogenicity; 4 homozygous; Clinindb (India)
PubMed: Narang 2020 , Journal: Narang 2020
-
rs34764749
Germline
-
4/2794 individuals
-
-
-
Mohammed Faruq
+/?
4
c.590A>G
r.(?)
p.(Lys197Arg)
-
pathogenic
g.39974642A>G
-
-
-
FKBP10_000003
-
PubMed: Barbirato 2016
-
rs34764749
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
4
c.600_604del
r.(?)
p.(Tyr201Hisfs*58)
-
pathogenic
g.39974652_39974656del
-
-
-
FKBP10_000012
-
PubMed: Setijowati 2011
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
4
c.612C>G
r.(?)
p.(Tyr204*)
-
pathogenic
g.39974664C>G
-
-
-
FKBP10_000038
-
PubMed: Velasco and Morales, 2017
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
4
c.689T>C
r.(?)
p.(Ile230Thr)
-
pathogenic
g.39974741T>C
-
-
-
FKBP10_000016
-
PubMed: Caparrós-Martin 2013
-
-
Germline
-
-
-
-
-
Victor L Ruiz-Perez
+/.
-
c.703del
r.(?)
p.(Leu235Trpfs*11)
-
pathogenic
g.39974755del
-
FKBP10(NM_021939.3):c.703delC (p.L235Wfs*11)
-
FKBP10_000091
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/+
4i_10_
c.727+105_*1661del
r.?
p.?
-
pathogenic (recessive)
g.39974884_39980321del
g.41818632_41824069del
hg19 39974881_39980318del
-
FKBP10_000045
-
PubMed: Li 2019 , Journal: Li 2019 , PubMed: Li 2020
-
-
Germline
-
-
-
-
-
Xiuli Zhao
-?/.
-
c.727+236C>A
r.(=)
p.(=)
-
likely benign
g.39975015C>A
g.41818763C>A
FKBP10(NM_021939.4):c.727+236C>A
-
FKBP10_000085
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
-/.
-
c.732A>G
r.(?)
p.(Thr244=)
-
benign
g.39975466A>G
g.41819214A>G
FKBP10(NM_021939.4):c.732A>G (p.T244=)
-
FKBP10_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_VUmc
?/-
5
c.732A>G
r.(?)
p.(=)
-
VUS
g.39975466A>G
-
-
-
FKBP10_000004
-
-
-
rs8078775
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
5
c.743dup
r.(?)
p.(Gln249Thrfs*12)
-
pathogenic
g.39975477dup
-
743dupC
-
FKBP10_000010
-
PubMed: Shaheen 2011
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
5
c.743dup
r.(?)
p.(Gln249Thrfs*12)
-
pathogenic
g.39975477dup
-
743dupC
-
FKBP10_000010
-
PubMed: Schwarze 2013
-
-
Germline
-
-
-
-
-
Peter Byers
+/.
-
c.745C>T
r.(?)
p.(Gln249*)
-
pathogenic
g.39975479C>T
-
-
-
FKBP10_000097
The patient is compound heterozygote for the gene.
PubMed: Tan 2023
-
-
Germline/De novo (untested)
-
-
-
-
-
Kim Worring
+/.
-
c.764_772dup
r.(?)
p.(His255_Leu257dup)
-
pathogenic (recessive)
g.39975498_39975506dup
g.41819246_41819254dup
764_772dupACGTCCTCC
-
FKBP10_000026
-
PubMed: Zhou 2014
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/+
5
c.764_772dup
r.(?)
p.(His255_Leu257dup)
-
pathogenic
g.39975498_39975506dup
-
-
-
FKBP10_000026
-
PubMed: Zhou 2014
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
5
c.809_843del
r.(?)
p.(Leu270Glnfs*91)
-
pathogenic (recessive)
g.39975543_39975577del
g.41819291_41819325del
-
-
FKBP10_000041
-
PubMed: Li 2019 , Journal: Li 2019 , PubMed: Li 2020
-
-
Germline
-
-
-
-
-
Xiuli Zhao
+/+
5
c.813_814del
r.(?)
p.(Glu271Aspfs*101)
-
pathogenic (recessive)
g.39975547_39975548del
g.41819295_41819296del
-
-
FKBP10_000040
-
PubMed: Li 2019 , Journal: Li 2019 , PubMed: Li 2020
-
-
Germline
-
-
-
-
-
Xiuli Zhao
+/+
5
c.813_814del
r.(?)
p.(Glu271Aspfs*101)
-
pathogenic
g.39975547_39975548del
-
-
-
FKBP10_000040
-
PubMed: Zhang 2018
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
?/.
-
c.820G>A
r.(?)
p.(Glu274Lys)
-
VUS
g.39975554G>A
-
FKBP10(NM_021939.3):c.820G>A (p.(Glu274Lys))
-
FKBP10_000094
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/+
5
c.831del
r.(?)
p.(Gly278Alafs*20)
-
pathogenic (recessive)
g.39975565del
g.41819313del
831delC
-
FKBP10_000039
-
PubMed: Li 2019 , Journal: Li 2019 , PubMed: Li 2020
-
-
Germline
-
-
-
-
-
Xiuli Zhao
+/+
5
c.831del
r.(?)
p.(Gly278Alafs*20)
-
pathogenic (recessive)
g.39975565del
g.41819313del
831delC
-
FKBP10_000039
-
PubMed: Li 2019 , Journal: Li 2019 , PubMed: Li 2020
-
-
Germline
-
-
-
-
-
Xiuli Zhao
+/+
5
c.831del
r.(?)
p.(Gly278Alafs*20)
-
pathogenic
g.39975565del
-
831delC
-
FKBP10_000039
-
PubMed: Zhang 2018
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
+/?
5
c.831del
r.(?)
p.(Gly278Alafs*20)
-
pathogenic
g.39975565del
-
831delC
-
FKBP10_000039
-
PubMed: Vorster 2017
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
+/?
5
c.831del
r.(?)
p.(Gly278Alafs*20)
-
pathogenic
g.39975565del
-
831delC
-
FKBP10_000039
-
PubMed: Vorster 2017
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
+/?
5
c.831del
r.(?)
p.(Gly278Alafs*20)
-
pathogenic
g.39975565del
-
831delC
-
FKBP10_000039
-
PubMed: Vorster 2017
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
+/?
5
c.831del
r.(?)
p.(Gly278Alafs*20)
-
pathogenic
g.39975565del
-
831delC
-
FKBP10_000039
-
PubMed: Vorster 2017
-
-
Germline
-
-
-
-
-
Raymond Dalgleish
+/.
-
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic (recessive)
g.39975565dup
g.41819313dup
831dupC
-
FKBP10_000002
-
PubMed: Santana 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
5
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic (recessive)
g.39975565dup
g.41819313dup
831dupC
-
FKBP10_000002
-
PubMed: Liu 2017
-
-
Germline
-
1/101 cases OI
-
-
-
Johan den Dunnen
+/.
-
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic (recessive)
g.39975565dup
g.41819313dup
NM_021939.3:c.831dupC:p.(Gly278Argfs*95)
-
FKBP10_000002
-
PubMed: Maddirevula 2018
-
-
Germline
-
-
-
-
-
LOVD
+/.
-
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic (recessive)
g.39975565dup
g.41819313dup
NM_021939.3:c.831dupC:p.(Gly278Argfs*95)
-
FKBP10_000002
-
PubMed: Maddirevula 2018
-
-
Germline
-
-
-
-
-
LOVD
+/+
5
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic
g.39975565dup
-
831dupC
-
FKBP10_000002
The variant is mistakenly described as c.831_832insC and the frameshift as p.Gly278ArgfsX295 in the paper.
PubMed: Alanay 2010
-
rs137853883
Germline
-
-
-
-
-
Peter Byers
+/+
5
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic
g.39975565dup
-
831dupC
-
FKBP10_000002
-
PubMed: Kelley 2011
-
rs137853883
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
5
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic
g.39975565dup
-
831dupC
-
FKBP10_000002
-
PubMed: Kelley 2011
-
rs137853883
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
5
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic
g.39975565dup
-
831dupC
-
FKBP10_000002
-
PubMed: Kelley 2011
-
rs137853883
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
5
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic
g.39975565dup
-
831dupC
-
FKBP10_000002
No supporting evidence is presented that the misense p.(Arg115Gln) variant is disease-causing.; Kelley et al., classify this patient as having Bruck syndrome type 1, but that type maps to 17p12 ({PMID9927692:Bank et al., 1999}), whereas FKBP10 maps to 17q21.2.
PubMed: Kelley 2011
-
rs137853883
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
5
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic
g.39975565dup
-
831dupC
-
FKBP10_000002
-
PubMed: Shaheen 2011
-
rs137853883
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
5
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic
g.39975565dup
-
831dupC
-
FKBP10_000002
-
PubMed: Caparrós-Martin 2013
-
rs137853883
Germline
-
-
-
-
-
Victor L Ruiz-Perez
+/+
5
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic
g.39975565dup
-
831dupC
-
FKBP10_000002
-
PubMed: Schwarze 2013
-
rs137853883
Germline
-
-
-
-
-
Peter Byers
+/+
5
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic
g.39975565dup
-
831dupC
-
FKBP10_000002
-
PubMed: Schwarze 2013
-
rs137853883
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
5
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic
g.39975565dup
-
831dupC
-
FKBP10_000002
-
PubMed: Schwarze 2013
-
rs137853883
Germline
-
-
-
-
-
Peter Byers
+/+
5
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic
g.39975565dup
-
831dupC
-
FKBP10_000002
-
PubMed: Schwarze 2013
-
rs137853883
Germline
-
-
-
-
-
Peter Byers
+/+
5
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic
g.39975565dup
-
831dupC
-
FKBP10_000002
-
PubMed: Umair 2016
-
rs137853883
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
5
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic
g.39975565dup
-
831dupC
-
FKBP10_000002
-
PubMed: Kaneto 2016
-
rs137853883
Germline
-
-
-
-
-
Raymond Dalgleish
+/+
5
c.831dup
r.(?)
p.(Gly278Argfs*95)
-
pathogenic
g.39975565dup
-
831dupC
-
FKBP10_000002
-
PubMed: Xu 2017
-
rs137853883
Germline
-
-
-
-
-
Raymond Dalgleish