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%)

 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
|
+/. |
4 |
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, no detectable enzyme activity |
PubMed: Agrawal 2022, PubMed: Zhang 2011, PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
4 |
8i_9_ |
c.(1180+1_1181-1)_(*3982_?)del |
-, r.? |
p.? |
- |
pathogenic (recessive) |
g.(?_148560295)_(148564750_148568455)del |
g.(?_149478764)_(149483219_149486924)del |
del ex1-4, ex9, del ex9 |
- |
IDS_000539 |
non-contiguous deletion ex1-4, ex9 |
PubMed: Zhong 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
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 |
+/. |
1 |
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 |
+/. |
2 |
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 |
+/. |
2 |
_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 |
- |
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 |
+/. |
33 |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0, r.? |
p.0, p.? |
- |
pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
complete IDS del, del ex1-9, del gene, del IDS, del IDS gene, gene deleted, whole gene deletion, 1 more item |
- |
IDS_000307 |
1 Mb deletion Xq28incl. IDS, FMR2, deficient iduronate 2-sulfatase levels, del IDS, 5 more items |
PubMed: Agrawal 2022, PubMed: Amartino 2014, PubMed: Dvorakova 2017, PubMed: Fang 2022, 9 more items |
- |
- |
De novo, Germline, Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
_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, del ex1-4, ex9 |
- |
IDS_000782 |
IDS activity plasma 1.9 (reference 297-705 nmol/4h/ml), non-contiguous deletion ex1-4, ex9 |
PubMed: Semyachkina 2021, PubMed: Zhong 2023 |
- |
- |
Germline, Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
_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 |
+/. |
1 |
- |
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 |
+/. |
1 |
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 |
_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 |
_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 |
_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 |
_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 |
_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 |
_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 |
_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 |
_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 |
_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 |
_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 |
_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 |
_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 |
_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 |
1 more item |
PubMed: Oshima 2011 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
_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 |
+/. |
5 |
_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, break point sequences GenBank U77689/U77690, 3 more items |
PubMed: Lagerstedt 1997 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
_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 |
+/. |
2 |
_1_7i |
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, PubMed: Rathmann 1996 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
_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 |
_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 |
_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 |
_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 |
_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 |
+/. |
2 |
_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 |
+/. |
2 |
_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 |
_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 |
+/. |
2 |
_1_1i |
c.(?_-217)_(103+1_104-1)del |
r.0? |
p.0? |
- |
pathogenic, pathogenic (recessive) |
g.(148585824_148586564)_(148586884_?)del |
g.(149504294_149505034)_(149505408_?)del |
del ex1 dup ex4, exon 1 deletion |
- |
IDS_000169 |
deficient iduronate 2-sulfatase levels, exon 1 deletion, breakpoint not sequenced |
PubMed: Agrawal 2022, PubMed: Alcántara-Ortigoza 2016, Journal: Alcántara-Ortigoza 2016 |
- |
- |
Germline, Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen, Miguel Angel Alcántara-Ortigoza |
+/. |
3 |
_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, PubMed: Zhang 2019 |
- |
- |
Germline, Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
3 |
_1_6i |
c.(?_-217)_(879+1_880-1)del |
r.0, r.? |
p.0, p.? |
- |
pathogenic (recessive) |
g.(148571972_148577876)_(148586884_?)del |
g.(149490441_149496345)_(149505354_?)del |
del ex1-6 |
- |
IDS_000309 |
- |
PubMed: Agrawal 2022, PubMed: Sohn 2012, PubMed: Zhong 2023 |
- |
- |
Germline, Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
16 |
1_7i, _1_7i |
c.(?_-217)_(1006+1_1007-1)inv |
r.?, 1 more item |
p.0, p.? |
- |
pathogenic, pathogenic (recessive) |
g.(148568630_148571844)_(148586884_?)inv, g.(148569718_148608804)inv, g.148569718_148608804inv |
g.(149486999_149490313)_(149505354_?)inv, g.(149488187_14952727)4inv, g.149488187_149527274inv |
IDS/IDS2 inversion |
- |
IDS_000082 |
inversion IDS-intron7 / IDS2 exon3; present in 13% patients, recombination IDS/IDS2 gene, 1 more item |
PubMed: Alves 2006, PubMed: Bondeson 1995, PubMed: Isogai 1998, PubMed: Jezela-Stanek 2021, 2 more items |
- |
- |
De novo, Germline, Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen, Yu Sun |
+/. |
4 |
_1_7i |
c.(?_-217)_(1006+1_1107-1)del |
r.0, r.0? |
p.0, p.0? |
- |
pathogenic (recessive) |
g.(148568630_148571844)_(148586884_?)del |
g.(149486999_149490313)_(149505354_?)del |
del ex1-7 |
- |
IDS_000734 |
IDS activity plasma 0.01 (reference 297-705 nmol/4h/ml) |
PubMed: Chkioua 2020, PubMed: Semyachkina 2021, PubMed: Zhao 2021 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
3 |
_1_7i |
c.(?_-217)_(1006+1_1107-1)inv |
r.? |
p.? |
- |
pathogenic |
g.(148568630_148571844)_(148586884_?)inv |
g.(149486999_149490313)_(149505354_?)inv |
IDS inversion IDSP1-mediated |
- |
IDS_000000 |
IDS inversion IDSP1-mediated, IDS inversion IDSP1-mediated. |
PubMed: Alcántara-Ortigoza 2016, Journal: Alcántara-Ortigoza 2016 |
- |
- |
Germline |
- |
- |
HinfI |
- |
- |
Miguel Angel Alcántara-Ortigoza |
+/. |
2 |
_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 |
1_6i |
c.(?_-199)_(879+1_880-1)del |
r.0 |
p.0 |
- |
pathogenic |
g.? |
- |
del ex1-6 |
- |
IDS_000010 |
deletion extends 200 kb upstream |
PubMed: Birot 1996 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
10 |
_1_9_ |
c.(?_-199)_(*3979_?)del |
r.0 |
p.0 |
- |
pathogenic |
g.? |
- |
- |
- |
IDS_000156 |
complete gene deletion, complete gene deletion incl. DXS 295, DXS466; DXS296 and DXS304 present, 7 more items |
PubMed: Alves 2006, PubMed: Beck, OMIM:var0007, PubMed: Bunge, PubMed: Wraith, OMIM:var0007, 1 more item |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen, Yu Sun |
?/. |
1 |
- |
c.-5C>T |
r.(?) |
p.(=) |
- |
VUS |
g.148586672G>A |
- |
IDS(NM_000202.5):c.-5C>T (p.(=)) |
- |
IDS_000278 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
+/. |
1 |
3 |
c.(285_288)[del3] |
r.(?) |
p.(Arg96del) |
- |
pathogenic |
g.(148584972_148584975)[del3] |
- |
- |
- |
IDS_000030 |
- |
PubMed: Froisart 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
5 |
_1_9_ |
c.(?_-217)_(*3982_?)del |
r.0 |
p.0 |
- |
pathogenic, pathogenic (recessive) |
g.(?_148560295)_(148586884_?)del |
g.(?_149478764)_(149505354_?)del |
del gene |
- |
IDS_000307 |
complete gene deletion incl. DXS296; DXS304 present, complete gene deletion, IDS2 present, 1 more item |
PubMed: Flomen 1992, OMIM:var0002, PubMed: Froissart 1993, PubMed: Karsten 1998, 1 more item |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
_1_6i |
c.(?_-217)_(879+1_880-1)del |
r.0 |
p.0 |
- |
pathogenic |
g.(148571972_148577876)_(148586884_?)del |
g.(149490441_149496345)_(149505354_?)del |
del ex1-6 |
- |
IDS_000309 |
- |
PubMed: Vafiadaki 1998 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
_1_9_ |
c.-21375_*3982{0} |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.(?_148560295)_(148608042_?)del |
g.(?_149478764)_(149526512_?)del |
hg19 arr Xq28 (148,566,217–148,608,042)x0 |
- |
IDS_000347 |
- |
PubMed: Brusius-Facchin 2012, PubMed: Brusius-Facchin 2014 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
14 |
- |
c.-22130_1007-1089inv |
r.? |
p.? |
- |
pathogenic (recessive) |
g.148569718_148608797inv |
g.149488187_149527266inv |
recomb 7i to distal ex3 IDS2, no del ex (Lagerstedt) |
- |
IDS_000664 |
IDS activity plasma 0.01 (reference 297-705 nmol/4h/ml), 7 more items |
PubMed: Semyachkina 2021 |
- |
- |
Germline, Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
30 |
1_2, 2i_3i, 7i, 7i_9 |
c.? |
r.1_115del, r.? |
p.? |
- |
pathogenic (recessive) |
g.(138000001_147100000)_qterdelins[NC_000005.10:g.(130600001_149800000)_qter], g.? |
g.(138900001_148000000)_qterdelins[NC_000005.10:g.(131200001_150400000)_qter] |
conversion ex3 (Lualdi 2005), del125-239, IDS gene-pseudogene recombination, IDS inversion, IDS-IDS2, 12 more items |
46,XX,t(X;5)(q27;q31-q32) |
ARMCX4_000000, IDS_000000 |
deficient iduronate 2-sulfatase levels, genomic rearrangement; no detectable enzyme activity, 7 more items |
PubMed: Agrawal 2022, PubMed: Brusius-Facchin 2014, PubMed: Dvorakova 2017, PubMed: Galvis 2015, 9 more items |
- |
- |
De novo, Germline, Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
1 |
c.1A>T |
r.(?) |
p.(Met1?) |
ACMG |
pathogenic (recessive) |
g.148586667T>A |
g.149505137T>A |
(Met1Leu), M1L |
- |
IDS_000485 |
not in 100 control chromosomes |
PubMed: Gort 1998, PubMed: Zanetti 2024 |
SCV005088913 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
1 |
c.9_10insT |
r.(?), r.[9_10insu,9_10=] |
p.(Pro4SerfsTer43), p.[Pro4SerfsTer43,Pro4=] |
ACMG |
pathogenic (recessive) |
g.148586658_148586659insA |
g.149505128_149505129insA |
10insT |
- |
IDS_000522 |
1 more item |
PubMed: Lualdi 2010, PubMed: Lualdi 2017, PubMed: Zanetti 2024 |
SCV005089023 |
- |
De novo, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
3 |
1 |
c.12_13del |
r.(?) |
p.(Arg5AspfsTer41) |
ACMG |
pathogenic (recessive) |
g.148586657_148586658del |
g.149505127_149505128del |
12_13delCC |
- |
IDS_000833 |
- |
PubMed: Zanetti 2024, PubMed: Zhao 2021 |
SCV005089133 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
2 |
- |
c.19G>C |
r.(?) |
p.(Gly7Arg) |
- |
likely benign |
g.148586649C>G |
g.149505119C>G |
IDS(NM_000202.5):c.19G>C (p.(Gly7Arg)), IDS(NM_000202.7):c.19G>C (p.G7R) |
- |
IDS_000249 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden, VKGL-NL_Rotterdam |
+/. |
2 |
1 |
c.21_22insG |
r.(?) |
p.(Arg8AlafsTer39), p.Arg8fs |
ACMG |
pathogenic, pathogenic (recessive) |
g.148586646_148586647insC |
g.149505116_149505117insC |
- |
- |
IDS_000141 |
- |
PubMed: Kim 2003, PubMed: Sohn 2012, PubMed: Zanetti 2024 |
SCV005089242 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
9 |
1 |
c.22C>T |
r.(?), r.[22c>u,22=] |
p.(Arg8*), p.(Arg8Ter), p.[Arg8Ter,Arg8=] |
ACMG |
pathogenic, pathogenic (recessive) |
g.148586646G>A |
g.149505116G>A |
R8X |
- |
IDS_000019 |
2 more items |
PubMed: Brusius-Facchin 2014, PubMed: Filocamo 2001, PubMed: Lualdi 2010, PubMed: Josahkian 2021, 4 more items |
SCV005089352 |
rs1602750610 |
Germline, SUMMARY record |
yes |
- |
-EagI |
- |
- |
Johan den Dunnen |
+/. |
2 |
1 |
c.22_37del |
r.(?) |
p.(Arg8Trpfs*5), p.(Arg8TrpfsTer5) |
ACMG |
pathogenic (recessive) |
g.148586634_148586649del |
g.149505104_149505119del |
22_37del16pb |
- |
IDS_000214 |
- |
PubMed: Amartino 2014, PubMed: Zanetti 2024 |
SCV005089463 |
rs864622775 |
De novo, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen, Paula Rozenfeld |
-?/. |
1 |
- |
c.23G>T |
r.(?) |
p.(Arg8Leu) |
- |
likely benign |
g.148586645C>A |
g.149505115C>A |
IDS(NM_000202.7):c.23G>T (p.R8L) |
- |
IDS_000245 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+/. |
2 |
1 |
c.28_37del |
r.(?) |
p.(Leu10TrpfsTer5) |
ACMG |
pathogenic (recessive) |
g.148586634_148586643del |
g.149505104_149505113del |
28_37delCTTCTCTGGC |
- |
IDS_000832 |
- |
PubMed: Zanetti 2024, PubMed: Zhong 2023 |
SCV005089570 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
1 |
c.35G>A |
r.(?), r.35g>a |
p.(Trp12Ter), p.Trp12* |
ACMG |
pathogenic, pathogenic (recessive) |
g.148586633C>T |
g.149505103C>T |
- |
- |
IDS_000057 |
- |
PubMed: Filocamo 2001, PubMed: Zanetti 2024 |
SCV005089648 |
- |
Germline, SUMMARY record |
- |
- |
MaeI+ |
- |
- |
Johan den Dunnen |
+/. |
2 |
1 |
c.36G>A |
r.(?) |
p.(Trp12Ter) |
ACMG |
pathogenic (recessive) |
g.148586632C>T |
g.149505102C>T |
- |
- |
IDS_000647 |
no detectable enzyme activity |
PubMed: Kato 2005, PubMed: Zanetti 2024 |
SCV005089659 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
1 |
c.41del |
r.(?) |
p.(Gly14ValfsTer4) |
ACMG |
pathogenic (recessive) |
g.148586629del |
g.149505099del |
- |
- |
IDS_000956 |
- |
PubMed: Zanetti 2024 |
SCV005088914 |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
1 |
c.43del |
r.(?) |
p.(Leu15Trpfs*3), p.(Leu15TrpfsTer3) |
ACMG |
pathogenic (recessive) |
g.148586625del |
g.149505095del |
43delC |
- |
IDS_000313 |
- |
PubMed: Hopwood 1993 (Wilson, unpublished), PubMed: Zanetti 2024 |
SCV005088925 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
1 |
c.57del |
r.(?) |
p.(Val20SerfsTer18) |
ACMG |
pathogenic (recessive) |
g.148586612del |
g.149505082del |
- |
- |
IDS_000653 |
- |
PubMed: Zanetti 2024 |
SCV005088936 |
rs2124069318 |
SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
1 |
c.57delC |
r.(?) |
p.(Val20SerfsTer18) |
ACMG |
pathogenic (recessive) |
g.148586612del |
g.149505082del |
57delC |
- |
IDS_000653 |
no detectable enzyme activity; ACMG PVS1, PP4 |
PubMed: Agrawal 2022 |
SCV001480484 |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
1 |
c.71dup |
r.(?) |
p.(Gly25Argfs*22), p.(Gly25ArgfsTer22) |
ACMG |
pathogenic, pathogenic (recessive) |
g.148586597dup |
g.149505067dup |
70_71insT |
- |
IDS_000124 |
- |
PubMed: Vafiadaki 1998, PubMed: Zanetti 2024 |
SCV005088947 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
1 |
c.71_90del |
r.(?) |
p.(Leu24GlnfsTer16), p.Ala23fs |
ACMG |
pathogenic, pathogenic (recessive) |
g.148586580_148586599del |
g.149505050_149505069del |
69_88del |
- |
IDS_000136 |
- |
PubMed: Kim 2003, PubMed: Sohn 2012, PubMed: Zanetti 2024 |
SCV005088958 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
1 |
c.73G>T |
r.(?) |
p.(Gly25Ter) |
ACMG |
pathogenic (recessive) |
g.148586595C>A |
g.149505065C>A |
- |
- |
IDS_000831 |
- |
PubMed: Zanetti 2024, PubMed: Zhong 2023 |
SCV005088969 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
1 |
c.76_80dup |
r.(?) |
p.(Glu27AspfsTer13) |
ACMG |
pathogenic (recessive) |
g.148586590_148586594dup |
g.149505060_149505064dup |
- |
- |
IDS_000955 |
- |
PubMed: Zanetti 2024 |
SCV005088980 |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
1 |
c.79G>T |
r.(?) |
p.(Glu27Ter) |
ACMG |
pathogenic (recessive) |
g.148586589C>A |
g.149505059C>A |
- |
- |
IDS_000484 |
- |
PubMed: Pollard 2016, PubMed: Zanetti 2024 |
SCV005088991 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
1 |
c.82dup |
r.(?) |
p.(Thr28Asnfs*19), p.(Thr28AsnfsTer19) |
ACMG |
pathogenic (recessive) |
g.148586588dup |
g.149505058dup |
82-83insA |
- |
IDS_000329 |
- |
PubMed: Goldenfum 1996, PubMed: Zanetti 2024 |
SCV005089002 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
1 |
c.85C>T |
r.(?) |
p.(Gln29*), p.(Gln29Ter) |
ACMG |
pathogenic (recessive) |
g.148586583G>A |
g.149505053G>A |
- |
- |
IDS_000536 |
- |
PubMed: Varga 2016, PubMed: Zanetti 2024 |
SCV005089012 |
- |
Germline, SUMMARY record |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
1_1i |
c.93_103+65del |
r.[93_103delins[103+66_104-1],40_103del] |
p.? |
- |
pathogenic (recessive) |
g.148586500_148586575del |
g.149504970_149505045del |
93_103+65del76 |
- |
IDS_000837 |
- |
PubMed: Fukuhara 2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
4 |
1 |
c.95C>A |
r.(?) |
p.(Ser32Ter) |
ACMG |
pathogenic (recessive) |
g.148586573G>T |
g.149505043G>T |
S32X |
- |
IDS_000483 |
not in 100 control chromosomes |
PubMed: Gort 1998, PubMed: Zanetti 2024, PubMed: Zhang 2011, PubMed: Zhong 2023 |
SCV005089024 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
1 |
c.99del |
r.(?), r.99del |
p.(Thr34GlnfsTer4), p.Thr34GlnfsTer4 |
ACMG |
pathogenic (recessive) |
g.148586570del |
g.149505040del |
223delC |
- |
IDS_000351 |
- |
PubMed: Froissart 1998, PubMed: Zanetti 2024 |
SCV005089035 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
1 |
c.102_103delAGinsT |
r.spl |
p.? |
- |
pathogenic (recessive) |
g.148586565_148586566delinsA |
g.149505035_149505036delinsA |
102_103delAG>T (D35Cfs*11) |
- |
IDS_000954 |
IDS enzyme activity plasma 4.5 mMol/l/4 h |
PubMed: Alkhzouz 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
1 |
c.102_103delinsT |
r.(?) |
p.(Asp35MetfsTer3) |
ACMG |
pathogenic (recessive) |
g.148586565_148586566delinsA |
g.149505035_149505036delinsA |
- |
- |
IDS_000954 |
- |
PubMed: Zanetti 2024 |
SCV005089046 |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
3 |
1 |
c.103G>C |
r.(?) |
p.(Asp35His) |
ACMG |
likely pathogenic (recessive) |
g.148586565C>G |
g.149505035C>G |
- |
- |
IDS_000953 |
IDS activity plasma 10.4 (reference 297-705 nmol/4h/ml), 1 more item |
PubMed: Semyachkina 2021, PubMed: Zanetti 2024 |
SCV001450592, SCV005089057 |
rs2089514224 |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
1i |
c.103+1G>A |
r.spl |
p.? |
ACMG |
pathogenic (recessive) |
g.148586564C>T |
g.149505034C>T |
- |
- |
IDS_000830 |
- |
PubMed: Zanetti 2024, PubMed: Zhong 2023 |
SCV005089079 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
- |
c.103+1G>T |
r.spl |
p.? |
- |
pathogenic (recessive) |
g.148586564C>A |
g.149505034C>A |
- |
- |
IDS_000328 |
- |
PubMed: Goldenfum 1996 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
1i |
c.103+2T>A |
r.spl, r.spl? |
p.? |
ACMG |
pathogenic, pathogenic (recessive) |
g.148586563A>T |
g.149505033A>T |
IVS1+2T>A |
- |
IDS_000072 |
- |
PubMed: Froisart 2007, PubMed: Zanetti 2024 |
SCV005089068 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
1 |
- |
c.103+4C>T |
r.spl? |
p.? |
- |
likely benign |
g.148586561G>A |
- |
IDS(NM_000202.8):c.103+4C>T |
- |
IDS_000267 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |
-?/. |
1 |
- |
c.103+7C>T |
r.(=) |
p.(=) |
- |
likely benign |
g.148586558G>A |
- |
IDS(NM_000202.7):c.103+7C>T |
- |
IDS_000266 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
?/. |
1 |
1i |
c.103+34_103+56dup |
r.103_104= |
p.Asp35= |
- |
VUS |
g.148586509_148586531dup |
g.149504979_149505001dup |
103+34_56dup |
- |
IDS_000646 |
- |
PubMed: Lin 2019 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-/. |
3 |
1i |
c.103+41_103+63dup |
r.(?) |
p.(=) |
- |
benign |
g.148586509_148586531dup |
g.149504979_149505001dup |
103+34_56dup |
- |
IDS_000646 |
reduced leukocyte IDS enzyme activity, negative urinary first-line biochemistry examinations, 1 more item |
PubMed: Chuang 2018 |
- |
- |
Germline |
- |
1/153,032 newborn infants, 49/153,032 newborn infants |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
1i_4i |
c.103+274_508-1105del |
r.104_507del |
p.Asp35fs |
- |
pathogenic (recessive) |
g.148580943_148586291del |
g.149499412_149504761del |
g.376_5725del, del ex2-4 |
- |
IDS_000035 |
- |
PubMed: Bonuccelli 1998, PubMed: Filocamo 2001 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/., +?/. |
2 |
1i |
c.104-3T>G |
c.[103_104ins[104-216_104-4;gag],104_212del], r.spl |
p.? |
ACMG |
likely pathogenic (recessive), pathogenic (recessive) |
g.148585826A>C |
g.149504296A>C |
IVS1-3T>G |
- |
IDS_000670 |
- |
PubMed: Xie 2020, PubMed: Zanetti 2024 |
SCV005089090 |
- |
Germline/De novo (untested), SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
1i |
c.104-2A>C |
r.spl |
p.? |
- |
pathogenic (recessive) |
g.148585825T>G |
g.149504295T>G |
- |
- |
IDS_000829 |
deficient iduronate 2-sulfatase levels |
PubMed: Agrawal 2022 |
SCV001480475 |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
6 |
1i |
c.104-2A>G |
r.spl, r.spl?, r.[103_104ins[104-216_104-3;gg],104_212del], 1 more item |
p.?, p.fs* |
ACMG |
pathogenic, pathogenic (recessive) |
g.148585825T>C |
g.149504295T>C |
IVS1-2A>G, IVS1asA-G-2 |
- |
IDS_000023 |
IDS activity plasma 0.01 (reference 297-705 nmol/4h/ml), RNA level 0.7 of normal |
PubMed: Alves 2006, PubMed: Karsten 1998, PubMed: Moreira da Silva 2001, PubMed: Semyachkina 2021, 2 more items |
SCV005089101 |
rs2089505773 |
Germline, Germline/De novo (untested), SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen, Yu Sun |
+/. |
4 |
1i |
c.104-1G>A |
r.(104_240del), r.spl |
p.(Asp35Alafs*18), p.? |
ACMG |
pathogenic, pathogenic (recessive) |
g.148585824C>T |
g.149504294C>T |
- |
- |
IDS_000184 |
- |
PubMed: Pollard 2016, PubMed: Zanetti 2024, PubMed: Zhong 2023 |
SCV005089112 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen, Paula Rozenfeld |
+/. |
1 |
1i |
c.104-1_104delGAinsT |
r.spl |
p.? |
- |
pathogenic (recessive) |
g.148585823_148585824delinsA |
g.149504293_149504294delinsA |
- |
- |
IDS_000952 |
IDS activity plasma 2.64 (reference 297-705 nmol/4h/ml) |
PubMed: Semyachkina 2021 |
SCV001450632 |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
1 |
2 |
c.104-1_104delinsT |
r.spl |
p.? |
ACMG |
likely pathogenic (recessive) |
g.148585823_148585824delinsA |
g.149504293_149504294delinsA |
- |
- |
IDS_000952 |
- |
PubMed: Zanetti 2024 |
SCV005089123 |
rs2089505743 |
SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
1i_2i |
c.(103+1_104-1)_(240+1_241-1)del |
r.(104_240del), r.? |
p.(Asp35fs), p.? |
- |
pathogenic (recessive) |
g.(148585020_148585686)_(148585824_148586564)del |
g.(149503490_149504156)_(149504294_149505034)del |
del ex2 |
- |
IDS_000665 |
- |
PubMed: Fang 2022, PubMed: Zhao 2022 |
- |
- |
De novo, Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
2 |
- |
c.104A>G |
r.(?) |
p.(Asp35Gly) |
- |
likely benign |
g.148585823T>C |
g.149504293T>C |
IDS(NM_000202.7):c.104A>G (p.D35G), IDS(NM_000202.8):c.104A>G (p.D35G) |
- |
IDS_000232 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam, VKGL-NL_Groningen |
+/. |
2 |
2 |
c.113dup |
r.(?) |
p.(Asn38LysfsTer9) |
ACMG |
pathogenic (recessive) |
g.148585815dup |
g.149504285dup |
236_237insA |
- |
IDS_000482 |
not in 100 control chromosomes |
PubMed: Gort 1998, PubMed: Zanetti 2024 |
SCV005089134 |
- |
Germline, SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
- |
c.114_117dup |
r.(?) |
p.(Leu40Argfs*8) |
- |
pathogenic (recessive) |
g.148585810_148585813dup |
g.149504280_149504283dup |
114_117dupCGTT |
- |
IDS_000648 |
- |
PubMed: Zubaida 2019 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
2 |
c.115_118dup |
r.(?) |
p.(Leu40ArgfsTer8) |
ACMG |
pathogenic (recessive) |
g.148585810_148585813dup |
g.149504280_149504283dup |
- |
- |
IDS_000648 |
- |
PubMed: Zanetti 2024 |
SCV005089144 |
- |
SUMMARY record |
- |
- |
- |
- |
- |
Johan den Dunnen |