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
|
+/. |
7i_9_ |
c.(1006+1_1007-1)_(*3982_?)del |
r.? |
p.? |
- |
pathogenic (recessive) |
g.(?_148560295)_(148568630_148571844)del |
g.(?_149478764)_(149487099_149490313)del |
del ex8-9 |
- |
IDS_000540 |
del ex8-9 |
PubMed: Zhang 2011 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
7i_9_ |
c.(1006+1_1007-1)_(*3982_?)del |
r.? |
p.? |
- |
pathogenic (recessive) |
g.(?_148560295)_(148568630_148571844)del |
g.(?_149478764)_(149487099_149490313)del |
del ex8-9 |
- |
IDS_000540 |
del ex8-9 |
PubMed: Zhang 2011 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
7i_9_ |
c.(1006+1_1007-1)_(*3982_?)del |
r.? |
p.? |
- |
pathogenic (recessive) |
g.(?_148560295)_(148568630_148571844)del |
g.(?_149478764)_(149487099_149490313)del |
del ex8-9 |
- |
IDS_000540 |
no detectable enzyme activity |
PubMed: Agrawal 2022 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
7i_9_ |
c.(1006+1_1007-1)_(*3982_?)del |
r.? |
p.? |
- |
pathogenic (recessive) |
g.(?_148560295)_(148568630_148571844)del |
g.(?_149478764)_(149487099_149490313)del |
del ex8-9 |
- |
IDS_000540 |
- |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
8i_9_ |
c.(1180+1_1181-1)_(*3982_?)del |
r.? |
p.? |
- |
pathogenic (recessive) |
g.(?_148560295)_(148564750_148568455)del |
g.(?_149478764)_(149483219_149486924)del |
del ex9 |
- |
IDS_000539 |
- |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
8i_9_ |
c.(1180+1_1181-1)_(*3982_?)del |
r.? |
p.? |
- |
pathogenic (recessive) |
g.(?_148560295)_(148564750_148568455)del |
g.(?_149478764)_(149483219_149486924)del |
del ex9 |
- |
IDS_000539 |
- |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
8i_9_ |
c.(1180+1_1181-1)_(*3982_?)del |
r.? |
p.? |
- |
pathogenic (recessive) |
g.(?_148560295)_(148564750_148568455)del |
g.(?_149478764)_(149483219_149486924)del |
del ex9 |
- |
IDS_000539 |
- |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
8i_9_ |
c.(1180+1_1181-1)_(*3982_?)del |
- |
p.? |
- |
pathogenic (recessive) |
g.(?_148560295)_(148564750_148568455)del |
g.(?_149478764)_(149483219_149486924)del |
del ex1-4, ex9 |
- |
IDS_000539 |
non-contiguous deletion ex1-4, ex9 |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
3i_9_ |
c.(418+1_419-1)_(*3982_?)del |
r.? |
p.? |
- |
pathogenic (recessive) |
g.(?_148560295)_(148582569_148584841)del |
g.(?_149478764)_(149501038_149503311)del |
del ex4-9 |
- |
IDS_000541 |
- |
PubMed: Brusius-Facchin 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
4i_9_ |
c.(507+1_508-1)_(*3982_?)dup |
r.? |
p.? |
- |
pathogenic (recessive) |
g.(?_148560295)_(148579839_148582479)dup |
g.(?_149478764)_(149498308_149500948)dup |
dup ex5-9 |
- |
IDS_000678 |
- |
PubMed: Agrawal 2022 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
6i_9_ |
c.(879+1_880-1)_(*3982_?)del |
r.? |
p.? |
- |
pathogenic (recessive) |
g.(?_148560295)_(148571972_148577876)del |
g.(?_149478764)_(149490441_149496345)del |
del ex7-9 |
- |
IDS_000677 |
- |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
6i_9_ |
c.(879+1_880-1)_(*3982_?)del |
r.? |
p.? |
- |
pathogenic (recessive) |
g.(?_148560295)_(148571972_148577876)del |
g.(?_149478764)_(149490441_149496345)del |
del ex7-9 |
- |
IDS_000677 |
- |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-20915)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_149478764)_(149526043_?)del |
g.(?_148560295)_(148607582_?)del |
- |
- |
IDS_000661 |
del IDS/IDSP1 |
PubMed: Ramirez-Hernandez 2022 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-20915)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_149478764)_(149526043_?)del |
g.(?_148560295)_(148607582_?)del |
- |
- |
IDS_000661 |
del IDS/IDSP1 |
PubMed: Ramirez-Hernandez 2022 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.(?_-212423)_(*1198445_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_147365832)_(148799090_?)del |
g.(?_148284301)_(149717560_?)del |
del IDS gene |
arr[hg19] Xq28(147365832_148799090)x0 |
IDS_000675 |
- |
PubMed: Vollebregt 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
IDS gene deletion extending from DXS1185 to DXS466 |
- |
IDS_000307 |
- |
PubMed: Karsten 1998 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
gene deleted |
- |
IDS_000307 |
not in 100 control chromosomes |
PubMed: Gort 1998, PubMed: Gort 1998 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
gene deleted |
- |
IDS_000307 |
not in 100 control chromosomes |
PubMed: Gort 1998, PubMed: Gort 1998 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del gene |
- |
IDS_000307 |
- |
PubMed: Hartog 1999 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del gene |
- |
IDS_000307 |
- |
PubMed: Hartog 1999 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del IDS |
- |
IDS_000307 |
del IDS |
PubMed: Zhang 2011 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del IDS gene |
- |
IDS_000307 |
- |
PubMed: Amartino 2014 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.? |
p.? |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del ex1-9 |
- |
IDS_000307 |
- |
PubMed: Fang 2022 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
whole gene deletion |
- |
IDS_000307 |
deficient iduronate 2-sulfatase levels |
PubMed: Agrawal 2022 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
whole gene deletion |
- |
IDS_000307 |
no detectable enzyme activity |
PubMed: Agrawal 2022 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del IDS gene |
- |
IDS_000307 |
deletion undetectable by FISH probe RP11-378O08 |
PubMed: Kosuga 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del IDS gene |
- |
IDS_000307 |
deletion undetectable by FISH probe RP11-378O08 |
PubMed: Kosuga 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del IDS gene |
- |
IDS_000307 |
1 Mb deletion Xq28incl. IDS, FMR2 |
PubMed: Kosuga 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del ex1-9 |
- |
IDS_000307 |
- |
PubMed: Zhang 2019 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del ex1-9 |
- |
IDS_000307 |
- |
PubMed: Zhang 2019 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del ex1-9 |
- |
IDS_000307 |
- |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del ex1-9 |
- |
IDS_000307 |
- |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del ex1-9 |
- |
IDS_000307 |
- |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del ex1-9 |
- |
IDS_000307 |
- |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del ex1-9 |
- |
IDS_000307 |
- |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del ex1-9 |
- |
IDS_000307 |
- |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del ex1-9 |
- |
IDS_000307 |
- |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del ex1-9 |
- |
IDS_000307 |
- |
PubMed: Zhao 2021 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del IDS gene |
- |
IDS_000307 |
- |
PubMed: Dvorakova 2017 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del IDS gene |
- |
IDS_000307 |
- |
PubMed: Dvorakova 2017 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
complete IDS del |
- |
IDS_000307 |
IDS activity plasma 0.01 (reference 297-705 nmol/4h/ml) |
PubMed: Semyachkina 2021 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
complete IDS del |
- |
IDS_000307 |
IDS activity plasma 0.01 (reference 297-705 nmol/4h/ml) |
PubMed: Semyachkina 2021 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
complete IDS del |
- |
IDS_000307 |
IDS activity plasma 1.3 (reference 297-705 nmol/4h/ml) |
PubMed: Semyachkina 2021 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
complete IDS del |
- |
IDS_000307 |
IDS activity plasma 0.01 (reference 297-705 nmol/4h/ml) |
PubMed: Semyachkina 2021 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
complete IDS del |
- |
IDS_000307 |
IDS activity plasma 0.01 (reference 297-705 nmol/4h/ml) |
PubMed: Semyachkina 2021 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
complete IDS del |
- |
IDS_000307 |
IDS activity plasma 0.01 (reference 297-705 nmol/4h/ml) |
PubMed: Semyachkina 2021 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
complete IDS del |
- |
IDS_000307 |
IDS activity plasma 0.01 (reference 297-705 nmol/4h/ml) |
PubMed: Semyachkina 2021 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
complete IDS del |
- |
IDS_000307 |
IDS activity plasma 0.01 (reference 297-705 nmol/4h/ml) |
PubMed: Semyachkina 2021 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_4i |
c.(?_-217)_(507+1_508-1) |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(148579839_148582479)_(148586884_?)del |
g.(149498308_149500948)_(149505354_?)del |
del ex1-4, ex9 |
- |
IDS_000782 |
non-contiguous deletion ex1-4, ex9 |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_4i |
c.(?_-217)_(507+1_508-1) |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(148579839_148582479)_(148586884_?)del |
g.(149498308_149500948)_(149505354_?)del |
del ex1-4 |
- |
IDS_000782 |
IDS activity plasma 1.9 (reference 297-705 nmol/4h/ml) |
PubMed: Semyachkina 2021 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_6i |
c.(?_-217)_(879+1_880-1)del |
r.? |
p.? |
- |
pathogenic (recessive) |
g.(148571972_148577876)_(148586884_?)del |
g.(149490441_149496345)_(149505354_?)del |
del ex1-6 |
- |
IDS_000309 |
- |
PubMed: Hartog 1999 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.(?_-948348)_(*171637_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148392640)_(149535015_?)del |
g.(?_149311109)_(150453485_?)del |
del IDS gene |
arr[hg19] Xq28(148392640_149535015)x0 |
IDS_000676 |
- |
PubMed: Vollebregt 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
8i_9 |
c.1181-1203_*262del |
r.spl |
p.? |
- |
pathogenic (recessive) |
g.148564018_148565955del |
g.149482487_149484424del |
- |
- |
IDS_000679 |
- |
PubMed: Dvorakova 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1 |
c.?_(-130890_-133487)delinsN[?] |
r.0? |
p.0? |
- |
pathogenic |
g.(148717557_148720154)_qterdelins[NC_000009.11:g.(50700001_65900000)_qter] |
g.(149635874_149638471)_qterdelins[NC_000009.12:g.(45500001_61500000)_qter] |
- |
46,X,t(X;9)(q28;q12) |
IDS_000835 |
- |
PubMed: Lonardo 2014 |
- |
- |
Germline |
- |
- |
- |
- |
non-random X-inactivation |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-1669707)_(*4464972_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_144099305)_(150256374_?)del |
g.(?_145017774)_(151174844_?)del |
whole gene deletion |
arr[GRCh38] Xq27.3 q28(145017774_151174844)x0 |
IDS_000652 |
4.7 Mb deletion containing 18 OMIM genes incl. IDS |
PubMed: Agrawal 2022 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-1096317)_(*8149751_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_140414526)_(149682984_?)del |
g.(?_141326740)_(150595198_?)del |
- |
hg19 Xq27.2q28(140414526_149682984)×0 |
IDS_000668 |
9.27 Mb deletion incl. AFF2, FMR1, IDS, MAMLD1 |
PubMed: Jezela-Stanek 2021 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-888828)_(*2284895_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_146279382)_(149475495_?)del |
g.(?_147197851)_(150393965_?)del |
hg18 146087287-149283399del |
- |
IDS_000836 |
3.2Mb deletion including IDS, FMR1, AFF2 |
PubMed: Burruss 2012 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.-817467_*8573872del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_139990405)_(149404134_?)del |
g.(?_140908874)_(150322604_?)del |
- |
hg19 arr Xq27.1Xq28(139,990,405–149,404,134)x0 |
IDS_000348 |
9.4 Mb deletion incl. FMR1, AFF2, IDS |
PubMed: Brusius-Facchin 2012, PubMed: Brusius-Facchin 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-638727)_(*1171993_?)del |
r.0 |
p.0 |
- |
pathogenic |
g.(147300000_147392284)_(149225394_149300000)del |
- |
- |
arrXq28(147,392,284-149,225,394)x0 |
IDS_000170 |
1.83 Mb complete AFF2/IDS/IDSP1 contiguous deletion. FMR1 preserved |
PubMed: Alcántara-Ortigoza 2016, Journal: Alcántara-Ortigoza 2016 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Miguel Angel Alcántara-Ortigoza |
+/. |
_1_9_ |
c.(?_-617769)_(*1474306_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_-147089971)_(149204436_?)del |
g.(?_-147961171)_(150075662_?)del |
- |
hg19 Xq27.3q28(147089971_149204436)×0 |
IDS_000669 |
2.11 Mb deletion incl. AFF2, IDS |
PubMed: Jezela-Stanek 2021 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-152178)_(*387863_?)del |
r.0 |
p.0 |
- |
pathogenic |
g.(148000000_148176414)_(148738845_149000000)del |
- |
- |
arr Xq28(148,176,414-148,738,845)x0 mat |
IDS_000188 |
562 kb complete IDS/IDSP1 deletion, AFF2/FMR1 loci preserved |
PubMed: Alcántara-Ortigoza 2016, Journal: Alcántara-Ortigoza 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Miguel Angel Alcántara-Ortigoza |
+/. |
_1_9_ |
c.(?_-145227)_(*1223986_?)del |
r.0 |
p.0 |
- |
pathogenic |
g.(147000000_147340291)_(148731894_149000000)del |
- |
- |
arr Xq28(147,340,291-148,731,894)x0 |
IDS_000189 |
1.39 Mb complete AFF2/IDS/IDSP1 contiguous deletion, FMR1 preserved |
PubMed: Alcántara-Ortigoza 2016, Journal: Alcántara-Ortigoza 2016 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Miguel Angel Alcántara-Ortigoza |
+/. |
_1_9_ |
c.(?_-37581)_(*97194_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(148400000_148467083)_(148624248_148700000)del |
- |
- |
arr Xq28(148,467,083-148,624,248)x0 mat |
IDS_000535 |
157 kb complete IDS/IDSP1 deletion, AFF2/FMR1 loci preserved |
PubMed: Alcántara-Ortigoza 2016, Journal: Alcántara-Ortigoza 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Miguel Angel Alcántara-Ortigoza |
+/. |
_1_9_ |
c.-37202_*3837516del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_144726761)_(148623869_?)del |
g.(?_145645230)_(149542339_?)del |
- |
hg19 arr Xq27.3Xq28(144,726,761–148,623,869)x0 |
IDS_000349 |
3.9Mb deletion incl. FMR1, AFF2, IDS |
PubMed: Brusius-Facchin 2012, PubMed: Brusius-Facchin 2014 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_7i |
c.(?_-36773)_(1007-157_1007-1)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(148568630_148568786)_(148623440_148630000)del |
- |
- |
- |
IDS_000505 |
54.7 kb deletion incl. 5'end CXorf40A, LOC1002876 and 5' end IDS |
PubMed: Zanetti 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_7i |
c.-30086_1007-1357delins[NC_000023.10:g.148606281_148608529inv] |
r.0? |
p.0? |
- |
pathogenic (recessive) |
g.148569986_148616753delins148606281_148608529inv |
g.149488455_149535223delins149524738_149526986inv |
hg18 148377891_148424657delins148414166_148416414inv |
- |
IDS_000959 |
36,275bp deletion encompassing IDS locus (ex1-7), 8234bp deletion (partial IDSP1) distal to IDS locus interrupted by 2248bp inverted region (portions of LOC727913 and IDSP1) |
PubMed: Oshima 2011 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_7i |
c.-27388_1006+1433delinsG |
r.1007_*3982delinsN[?] |
p.? |
- |
pathogenic (recessive) |
g.148570412_148614055delinsC |
g.149488881_149532525delinsC |
- |
- |
IDS_000343 |
fusion transcript detected of exon 2 gene W and exons 8 and 9 IDS gene |
PubMed: Lagerstedt 2000 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_7i |
c.-22130_1007-1089inv |
r.? |
p.? |
- |
pathogenic (recessive) |
g.148569718_148608797inv |
g.149488187_149527266inv |
IDS/IDS2 inversion |
- |
IDS_000664 |
break point sequences GenBank U77685/U77686 |
PubMed: Lagerstedt 1997 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_7i |
c.-22130_1007-1089inv |
r.? |
p.? |
- |
pathogenic (recessive) |
g.148569718_148608797inv |
g.149488187_149527266inv |
- |
- |
IDS_000664 |
break point sequences GenBank U77689/U77690 |
PubMed: Lagerstedt 1997 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_7i |
c.-22130_1007-1089inv |
r.? |
p.? |
- |
pathogenic (recessive) |
g.148569718_148608797inv |
g.149488187_149527266inv |
- |
- |
IDS_000664 |
break point sequences GenBank U77691/U77692 |
PubMed: Lagerstedt 1997 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_7i |
c.-22130_1007-1089inv |
r.? |
p.? |
- |
pathogenic (recessive) |
g.148569718_148608797inv |
g.149488187_149527266inv |
- |
- |
IDS_000664 |
break point sequences GenBank U77693/U77694 |
PubMed: Lagerstedt 1997 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_7i |
c.-22130_1007-1089inv |
r.? |
p.? |
- |
pathogenic (recessive) |
g.148569718_148608797inv |
g.149488187_149527266inv |
- |
- |
IDS_000664 |
break point sequences GenBank U77695/U77696 |
PubMed: Lagerstedt 1997 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_7i |
c.-22130_1007-1089inv |
r.? |
p.? |
- |
pathogenic (recessive) |
g.148569718_148608797inv |
g.149488187_149527266inv |
- |
- |
IDS_000664 |
break point sequences GenBank U77687/U77688 |
PubMed: Lagerstedt 1997 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_7i |
c.-21912_1007-1315inv |
r.1007_*3982delinsN[?] |
p.? |
- |
pathogenic (recessive) |
g.148569944_148608579inv |
g.149488413_149527049inv |
- |
- |
IDS_000095 |
- |
PubMed: Rathmann 1996 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.-21912_1007-1315inv |
r.1007_*3982delinsN[?] |
p.? |
- |
pathogenic (recessive) |
g.148569944_148608579inv |
g.149488413_149527049inv |
- |
- |
IDS_000095 |
inversion IDS intron 7 / IDSP1 intron 3 (1.3kb downstream) |
PubMed: Bondeson 1995 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-21644)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic |
g.(?_148560295)_(148608311_?)del |
g.(?_149478764)_(149526781_?)del |
- |
- |
IDS_000662 |
complete gene deletion incl. pseudogene |
PubMed: Vafiadaki 1998 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_7i |
c.-21059_1007-260del |
r.0? |
p.0? |
- |
pathogenic (recessive) |
g.148568889_148607726del |
g.149487358_149526196del |
NT_019686 1307880-1346697del |
- |
IDS_000344 |
- |
PubMed: Chou 2005 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_9_ |
c.(?_-20915)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148607582_?)del |
g.(?_149478764)_(149526043_?)del |
del IDS/IDSP1 |
- |
IDS_000661 |
del IDS/IDSP1 |
PubMed: Zhang 2011 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
_1 |
c.-816G>A |
r.(?) |
- |
- |
likely benign |
g.148587483C>T |
g.149505953= |
AF011889.1:g.272678A>G |
- |
IDS_000533 |
variant detected in 10/100 control chromosomes |
PubMed: Brusius-Facchin 2013 |
- |
rs611188 |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1 |
c.-311_-135del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.148586802_148586978del |
g.149505272_149505448del |
- |
- |
IDS_000353 |
no mRNA detectable |
PubMed: Timms 1998 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1 |
c.-310_-133del |
r.(=|red) |
p.(=) |
- |
pathogenic (recessive) |
g.148586802_148586979del |
g.149505272_149505449del |
g.271996_272174del |
- |
IDS_000000 |
- |
PubMed: Josahkian 2021 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1 |
c.-310_-133del |
r.(=|red) |
p.(=) |
- |
pathogenic (recessive) |
g.148586802_148586979del |
g.149505272_149505449del |
g.271996_272174del |
- |
IDS_000000 |
- |
PubMed: Josahkian 2021 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1 |
c.-307_-131del |
r.? |
p.? |
- |
pathogenic (recessive) |
g.148586799_148586975del |
g.149505269_149505445del |
- |
- |
IDS_000532 |
- |
PubMed: Brusius-Facchin 2013 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1 |
c.-307_-131del |
r.? |
p.? |
- |
pathogenic (recessive) |
g.148586799_148586975del |
g.149505269_149505445del |
- |
- |
IDS_000532 |
- |
PubMed: Brusius-Facchin 2013 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_4_ |
c.(?_-265)_(507+11_?)del |
r.0? |
p.0? |
- |
pathogenic (recessive) |
g.(?_148582469)_(148586932_?)del |
g.(?_149500938)_(149505402_?)del |
del ex1-4 (1–103_184del) |
- |
IDS_000834 |
- |
PubMed: Jurecka 2012 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_1i |
c.(?_-217)_(103+1_104-1)del |
r.0? |
p.0? |
- |
pathogenic |
g.(148585824_148586564)_(148586884_?)del |
g.(149504294_149505034)_(149505408_?)del |
exon 1 deletion |
- |
IDS_000169 |
exon 1 deletion, breakpoint not sequenced |
PubMed: Alcántara-Ortigoza 2016, Journal: Alcántara-Ortigoza 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Miguel Angel Alcántara-Ortigoza |
+/. |
_1_1i |
c.(?_-217)_(103+1_104-1)del |
r.0? |
p.0? |
- |
pathogenic (recessive) |
g.(148585824_148586564)_(148586884_?)del |
g.(149504294_149505034)_(149505408_?)del |
del ex1 dup ex4 |
- |
IDS_000169 |
deficient iduronate 2-sulfatase levels |
PubMed: Agrawal 2022 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_3i |
c.(?_-217)_(418+1_419-1)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(148582569_148584841)_(148586884_?)del |
g.(149501038_149503311)_(149505354_?)del |
del ex1-3 |
- |
IDS_000801 |
- |
PubMed: Zhang 2019 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_3i |
c.(?_-217)_(418+1_419-1)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(148582569_148584841)_(148586884_?)del |
g.(149501038_149503311)_(149505354_?)del |
del ex1-3 |
- |
IDS_000801 |
IDS activity dried blood spot 0.01 (reference 2.5-50 umM/l/h) |
PubMed: Semyachkina 2021 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_3i |
c.(?_-217)_(418+1_419-1)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(148582569_148584841)_(148586884_?)del |
g.(149501038_149503311)_(149505354_?)del |
del ex1-3 |
- |
IDS_000801 |
IDS activity dried blood spot 0.01 (reference 2.5-50 umM/l/h) |
PubMed: Semyachkina 2021 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_6i |
c.(?_-217)_(879+1_880-1)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(148571972_148577876)_(148586884_?)del |
g.(149490441_149496345)_(149505354_?)del |
del ex1-6 |
- |
IDS_000309 |
- |
PubMed: Sohn 2012 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_6i |
c.(?_-217)_(879+1_880-1)del |
r.? |
p.? |
- |
pathogenic (recessive) |
g.(148571972_148577876)_(148586884_?)del |
g.(149490441_149496345)_(149505354_?)del |
del ex1-6 |
- |
IDS_000309 |
- |
PubMed: Agrawal 2022 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_6i |
c.(?_-217)_(879+1_880-1)del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(148571972_148577876)_(148586884_?)del |
g.(149490441_149496345)_(149505354_?)del |
del ex1-6 |
- |
IDS_000309 |
- |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1_7i |
c.(?_-217)_(1006+1_1007-1)inv |
r.? |
p.? |
- |
pathogenic |
g.(148568630_148571844)_(148586884_?)inv |
g.(149486999_149490313)_(149505354_?)inv |
- |
- |
IDS_000082 |
inversion IDS-intron7 / IDS2 exon3; present in 13% patients |
PubMed: Bondeson 1995 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1_7i |
c.(?_-217)_(1006+1_1007-1)inv |
r.? |
p.? |
- |
pathogenic |
g.(148568630_148571844)_(148586884_?)inv |
g.(149486999_149490313)_(149505354_?)inv |
- |
- |
IDS_000082 |
- |
PubMed: Isogai 1998 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1_7i |
c.(?_-217)_(1006+1_1007-1)inv |
r.? |
p.? |
- |
pathogenic |
g.(148568630_148571844)_(148586884_?)inv |
g.(149486999_149490313)_(149505354_?)inv |
- |
- |
IDS_000082 |
- |
PubMed: Isogai 1998 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1_7i |
c.(?_-217)_(1006+1_1007-1)inv |
r.? |
p.? |
- |
pathogenic |
g.(148568630_148571844)_(148586884_?)inv |
g.(149486999_149490313)_(149505354_?)inv |
- |
- |
IDS_000082 |
- |
PubMed: Isogai 1998 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1_7i |
c.(?_-217)_(1006+1_1007-1)inv |
r.? |
p.? |
- |
pathogenic |
g.(148568630_148571844)_(148586884_?)inv |
g.(149486999_149490313)_(149505354_?)inv |
- |
- |
IDS_000082 |
- |
PubMed: Isogai 1998 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1_7i |
c.(?_-217)_(1006+1_1007-1)inv |
r.? |
p.? |
- |
pathogenic |
g.(148568630_148571844)_(148586884_?)inv |
g.(149486999_149490313)_(149505354_?)inv |
- |
- |
IDS_000082 |
- |
PubMed: Isogai 1998 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1_7i |
c.(?_-217)_(1006+1_1007-1)inv |
r.? |
p.? |
- |
pathogenic |
g.(148568630_148571844)_(148586884_?)inv |
g.(149486999_149490313)_(149505354_?)inv |
- |
- |
IDS_000082 |
- |
PubMed: Isogai 1998 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |