Unique variants in the IDS gene

SUMMARY records: IDS variants classified following ACMG recommendations
Information The variants shown are described using the NM_000202.5 transcript reference sequence.

968 entries on 10 pages. Showing entries 1 - 100.
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Effect     

Reported     

Exon     

AscendingDNA 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
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