Unique variants in the SGSH gene

An NCL gene variant database
Information The variants shown are described using the NM_000199.3 transcript reference sequence.

240 entries on 3 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     
-?/. 1 - c.-2434G>T r.(?) p.(=) - likely benign g.78196546C>A - SLC26A11(NM_173626.3):c.327C>A (p.S109=) - SGSH_000121 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.-1315C>T r.(?) p.(=) - VUS g.78195427G>A g.80221628G>A SLC26A11(NM_001166347.2):c.68G>A (p.C23Y) - SLC26A11_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 2 _1_5i c.-687_664-23del r.0? p.0? - pathogenic (recessive) g.78187707_78194799del g.80213908_80221000del g.75802301_75809393del - SGSH_000042 - PubMed: Ouesleti 2011 - - Germline - - - - - Johan den Dunnen
+?/. 1 - c.1A>G r.(?) p.(Met1?) - likely pathogenic (recessive) g.78194112T>C g.80220313T>C - - SGSH_000198 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
+/. 3 1 c.2T>C r.(?), r.? p.0?, p.? - pathogenic, pathogenic (recessive) g.78194111A>G g.80220312A>G c.2T>C - SGSH_000038 - PubMed: Ouesleti 2011, PubMed: Ousleti-2011 - - Germline - - - - - Johan den Dunnen
+/. 3 1 c.29dup r.(?) p.(Leu11Alafs*22) - pathogenic, pathogenic (recessive) g.78194084dup g.80220285dup c.29dup - SGSH_000040 - PubMed: Ouesleti 2011, PubMed: Ousleti-2011 - - Germline - - - - - Johan den Dunnen
?/. 1 1 c.83T>C r.(?) p.(Leu28Pro) - VUS g.78194030A>G g.80220231A>G - - SGSH_000123 - PubMed: Ganapathy 2019 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.88G>A r.(?) p.(Ala30Thr) - VUS g.78194025C>T - SGSH(NM_001352921.1):c.88G>A (p.A30T) - SGSH_000109 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.88G>C r.(?) p.(Ala30Pro) - likely pathogenic (recessive) g.78194025C>G g.80220226C>G - - SGSH_000197 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.90G>A r.(?) p.(=) - VUS g.78190990C>T - SGSH(NM_000199.5):c.90G>A (p.(Ala30=)) - SGSH_000210 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 2 c.96C>A r.(?) p.(Asp32Glu) - likely pathogenic g.78190984G>T g.80217185G>T - - SGSH_000010 1 more item - - - Germline - - - - - Dr. Alexandra Wey-Fabrizius
+?/. 1 - c.97G>A r.(?) p.(Gly33Arg) - likely pathogenic (recessive) g.78190983C>T g.80217184C>T - - SGSH_000196 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.111T>A r.(?) p.(Ser37Arg) ACMG likely pathogenic g.78190969A>T - - - SGSH_000122 - - - - Germline - - - - - Anju Shukla
+?/. 1 - c.149_169del r.(?) p.(Leu50_Ser57delinsArg) - likely pathogenic (recessive) g.78190911_78190931del g.80217112_80217132del 149_169del21 - SGSH_000195 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.163C>T r.(?) p.(Arg55Cys) - VUS g.78190917G>A - SGSH(NM_001352921.1):c.163C>T (p.R55C) - SGSH_000108 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.177C>T r.(?) p.(Leu59=) - likely benign g.78190903G>A - SGSH(NM_001352921.1):c.177C>T (p.L59=) - SGSH_000120 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/., ?/. 9 2 c.197C>G r.(?) p.(Ser66Trp) - pathogenic, pathogenic (recessive), VUS g.78190883G>C g.80217084G>C c.197C>G, SGSH(NM_000199.4):c.197C>G (p.S66W), SGSH(NM_000199.5):c.197C>G (p.S66W) - SGSH_000008 VKGL data sharing initiative Nederland PubMed: Ouesleti 2011, PubMed: Ousleti-2011, PubMed: Pollard 2016 - - CLASSIFICATION record, Germline - - - - - Gerard C.P. Schaafsma, Johan den Dunnen, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen
+/., ?/. 6 2 c.220C>T r.(?) p.(Arg74Cys) - pathogenic, VUS g.78190860G>A g.80217061G>A SGSH(NM_000199.4):c.220C>T (p.R74C), SGSH(NM_000199.5):c.220C>T (p.R74C) - SGSH_000011 VKGL data sharing initiative Nederland PubMed: Kaiwar 2017, PubMed: Pollard 2016 - rs104894636 CLASSIFICATION record, Germline ? - - - - Johan den Dunnen, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen, Dr. Alexandra Wey-Fabrizius, Benjamin Billiet
+?/. 1 - c.221G>A r.(?) p.(Arg74His) - likely pathogenic g.78190859C>T - - - SGSH_000133 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.235A>C r.(?) p.(Thr79Pro) - VUS g.78190845T>G g.80217046T>G - - SGSH_000194 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
-/. 1 - c.250-26C>T r.(=) p.(=) - benign g.78188963G>A g.80215164G>A SGSH(NM_000199.4):c.250-26C>T - SGSH_000036 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.265_269del r.(?) p.(Tyr89AlafsTer45) - likely pathogenic (recessive) g.78188919_78188923del g.80215120_80215124del 265_269delTACGG - SGSH_000193 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.276C>G r.(?) p.(His92Gln) - VUS g.78188911G>C - SGSH(NM_000199.5):c.276C>G (p.(His92Gln)) - SGSH_000209 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 - c.301_303del r.(?) p.(Phe101del) - likely pathogenic (recessive) g.78188885_78188887del g.80215086_80215088del 301_303delTTC - SGSH_000192 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.308A>G r.(?) p.(Lys103Arg) - VUS g.78188879T>C g.80215080T>C - - SGSH_000199 - PubMed: Fernandez-Marmiesse 2014 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.344G>T r.(?) p.(Gly115Val) - VUS g.78188843C>A g.80215044C>A SGSH(NM_000199.4):c.344G>T (p.G115V) - SGSH_000095 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.349C>T r.(?) p.(Arg117Cys) - VUS g.78188838G>A - SGSH(NM_000199.5):c.349C>T (p.(Arg117Cys)) - SGSH_000208 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.350G>T r.(?) p.(Arg117Leu) - VUS g.78188837C>A g.80215038C>A SGSH(NM_001352921.3):c.350G>T (p.R117L) - SGSH_000094 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. 1 - c.356-6G>A r.(=) p.(=) - likely benign g.78188570C>T - SGSH(NM_000199.5):c.356-6G>A - SGSH_000207 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 3 - c.364G>A r.(?) p.(Gly122Arg) - pathogenic, pathogenic (recessive) g.78188556C>T g.80214757C>T SGSH(NM_000199.4):c.364G>A (p.G122R), SGSH(NM_000199.5):c.364G>A (p.G122R) - SGSH_000035 VKGL data sharing initiative Nederland PubMed: Pollard 2016 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Rotterdam, VKGL-NL_Groningen
+/. 2 - c.376dup r.(?) p.(Val126GlyfsTer10) - pathogenic g.78188544dup g.80214745dup SGSH(NM_000199.4):c.376dupG (p.V126Gfs*10) - SGSH_000080 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
?/. 1 - c.391G>A r.(?) p.(Val131Met) - VUS g.78188529C>T g.80214730C>T - - SGSH_000005 - - - - Germline - - - - - Gerard C.P. Schaafsma
+?/. 1 - c.408_444dup r.(?) p.(Gly149CysfsTer18) - likely pathogenic (recessive) g.78188476_78188512dup g.80214677_80214713dup 408_444dup37 - SGSH_000191 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
-/., -?/. 2 - c.411G>A r.(?) p.(Ala137=) - benign, likely benign g.78188509C>T g.80214710C>T SGSH(NM_000199.4):c.411G>A (p.A137=), SGSH(NM_000199.5):c.411G>A (p.A137=) - SGSH_000079 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
+/. 1 - c.449G>A r.(?) p.(Arg150Gln) - pathogenic g.78188471C>T g.80214672C>T SGSH(NM_000199.4):c.449G>A (p.R150Q) - SGSH_000033 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.494C>G r.(?) p.(Thr165Ser) - likely benign g.78188426G>C - SGSH(NM_001352921.1):c.494C>G (p.T165S) - SGSH_000145 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.506+3A>G r.spl? p.? - VUS g.78188411T>C - SGSH(NM_000199.5):c.506+3A>G - SGSH_000175 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 2 - c.507-7C>T r.(=) p.(=) - likely benign g.78188134G>A - SGSH(NM_000199.3):c.507-7C>T (p.(=)), SGSH(NM_001352921.1):c.507-7C>T - SGSH_000103 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
?/. 1 - c.508C>G r.(?) p.(Pro170Ala) - VUS g.78188126G>C g.80214327G>C SGSH(NM_000199.3):c.508C>G (p.(Pro170Ala)) - SGSH_000078 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/., -?/. 2 - c.522C>T r.(?) p.(Tyr174=) - benign, likely benign g.78188112G>A - SGSH(NM_000199.5):c.522C>T (p.(Tyr174=)), SGSH(NM_001352921.3):c.522C>T (p.Y174=) - SGSH_000119 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_AMC
+/. 1 5 c.544C>T r.(?) p.(Arg182Cys) - pathogenic (recessive) g.78188090G>A g.80214291G>A - - SGSH_000202 - PubMed: Fang 2022 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.545G>A r.(?) p.(Arg182His) - VUS g.78188089C>T g.80214290C>T - - SGSH_000076 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.563C>T r.(?) p.(Pro188Leu) - VUS g.78188071G>A - SGSH(NM_000199.5):c.563C>T (p.(Pro188Leu)) - SGSH_000206 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/., +?/. 3 5 c.571G>A r.(?) p.(Gly191Arg) - likely pathogenic, pathogenic, pathogenic (recessive) g.78188063C>T g.80214264C>T SGSH(NM_000199.4):c.571G>A (p.G191R) - SGSH_000032 VKGL data sharing initiative Nederland 0, PubMed: Fang 2022 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Rotterdam
+?/. 1 - c.582T>A r.(?) p.(Cys194Ter) - likely pathogenic (recessive) g.78188052A>T g.80214253A>T - - SGSH_000190 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.598G>A r.(?) p.(Gly200Arg) - VUS g.78188036C>T g.80214237C>T SGSH(NM_001352921.1):c.598G>A (p.G200R) - SGSH_000093 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.614G>T r.(?) p.(Gly205Val) - likely pathogenic g.78188020C>A - - - SGSH_000163 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. 1 - c.617G>A r.(?) p.(Arg206His) - likely benign g.78188017C>T - SGSH(NM_000199.3):c.617G>A (p.(Arg206His)) - SGSH_000118 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/., ?/. 2 - c.641C>A r.(?) p.(Ala214Asp) - likely benign, VUS g.78187993G>T g.80214194G>T SGSH(NM_000199.4):c.641C>A (p.A214D), SGSH(NM_000199.5):c.641C>A (p.A214D) - SGSH_000075 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_AMC
-?/., ?/. 2 - c.658G>T r.(?) p.(Val220Leu) - likely benign, VUS g.78187976C>A g.80214177C>A SGSH(NM_000199.3):c.658G>T (p.(Val220Leu)) - SGSH_000092 1 homozygous; Clinindb (India), VKGL data sharing initiative Nederland PubMed: Narang 2020, Journal: Narang 2020 - rs150508741 CLASSIFICATION record, Germline - 1/2795 individuals - - - VKGL-NL_Leiden, Mohammed Faruq
-/. 5 - c.663+17T>C r.(=) p.(=) - benign g.78187954A>G g.80214155A>G 1 more item - SGSH_000030 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen, VKGL-NL_VUmc, VKGL-NL_AMC
-/. 1 - c.664-39_664-36delinsGC r.(=) p.(=) - benign g.78187720_78187723delinsGC g.80213921_80213924delinsGC SGSH(NM_000199.4):c.664-39_664-36delCTGTinsGC - SGSH_000029 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/., ?/. 3 - c.701C>G r.(?) p.(Ala234Gly) - benign, VUS g.78187647G>C g.80213848G>C SGSH(NM_000199.4):c.701C>G (p.A234G) - SGSH_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record, Germline - - - - - Gerard C.P. Schaafsma, VKGL-NL_Nijmegen, VKGL-NL_VUmc
+/., ?/. 13 7 c.734G>A r.(?) p.(Arg245His) - pathogenic, pathogenic (recessive), VUS g.78187614C>T g.80213815C>T 746G>A, 1 more item - SGSH_000003 protein analysis (LC-MS/MS) brain SGSH absent, VKGL data sharing initiative Nederland PubMed: Pollard 2016, PubMed: Sleat 2009 - - CLASSIFICATION record, Germline - - - - - Gerard C.P. Schaafsma, Johan den Dunnen, Sara Mole, VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen
-/., -?/. 2 - c.746-4A>G r.spl? p.? - benign, likely benign g.78186077T>C g.80212278T>C SGSH(NM_000199.3):c.746-4A>G (p.?), SGSH(NM_001352921.3):c.746-4A>G - SGSH_000074 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_VUmc
?/. 1 - c.751G>A r.(?) p.(Gly251Arg) - VUS g.78186068C>T - SGSH(NM_001352921.1):c.751G>A (p.G251R) - SGSH_000144 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.752G>C r.(?) p.(Gly251Ala) - VUS g.78186067C>G - SGSH(NM_001352921.1):c.752G>C (p.G251A) - SGSH_000117 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 2 - c.808T>C r.(?) p.(Phe270Leu) - VUS g.78186011A>G g.80212212A>G SGSH(NM_000199.3):c.808T>C (p.(Phe270Leu)), SGSH(NM_001352921.3):c.808T>C (p.F270L) - SGSH_000091 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Groningen
+/. 1 - c.817G>A r.(?) p.(Asp273Asn) - pathogenic g.78186002C>T g.80212203C>T SGSH(NM_000199.4):c.817G>A (p.D273N) - SGSH_000028 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.863C>T r.(?) p.(Pro288Leu) - likely pathogenic (recessive) g.78185956G>A g.80212157G>A - - SGSH_000189 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
+/. 2 - c.877C>T r.(?) p.(Pro293Ser) - pathogenic, pathogenic (recessive) g.78185942G>A g.80212143G>A SGSH(NM_000199.5):c.877C>T (p.P293S) - SGSH_000027 VKGL data sharing initiative Nederland PubMed: Pollard 2016 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Groningen
+/. 7 7 c.892T>C r.(?) p.(Ser298Pro) - pathogenic, pathogenic (recessive) g.78185927A>G g.80212128A>G 904T>C, 1 more item - SGSH_000001 protein analysis (LC-MS/MS) brain SGSH absent, VKGL data sharing initiative Nederland PubMed: Pollard 2016, PubMed: Sleat 2009 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, Sara Mole, VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen
+?/. 1 - c.911G>T r.(?) p.(Arg304Leu) - likely pathogenic (recessive) g.78185908C>A g.80212109C>A - - SGSH_000188 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.932C>A r.(?) p.(Ala311Asp) - VUS g.78185887G>T g.80212088G>T - - SGSH_000187 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.937G>A r.(?) p.(Val313Met) - VUS g.78185882C>T - SGSH(NM_000199.3):c.937G>A (p.(Val313Met)) - SGSH_000178 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 - c.949G>C r.(?) p.(Asp317His) - likely pathogenic (recessive) g.78185870C>G g.80212071C>G - - SGSH_000186 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.950-149C>T r.(=) p.(=) - likely benign g.78184959G>A - SGSH(NM_001352921.3):c.977C>T (p.(Ala326Val)) - SGSH_000205 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.950-131C>T r.(=) p.(=) - VUS g.78184941G>A - SGSH(NM_001352921.3):c.995C>T (p.(Ser332Leu)) - SGSH_000174 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.950-93G>A r.(=) p.(=) - VUS g.78184903C>T - SGSH(NM_001352921.1):c.1033G>A (p.G345R) - SGSH_000132 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 2 - c.950-75G>A r.(=) p.(=) - VUS g.78184885C>T - SGSH(NM_001352921.1):c.1051G>A (p.V351I), SGSH(NM_001352921.3):c.1051G>A (p.(Val351Ile)) - SGSH_000116 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
-?/. 1 - c.950-65C>T r.(=) p.(=) - likely benign g.78184875G>A - SGSH(NM_001352921.3):c.1061C>T (p.(Ala354Val)) - SGSH_000173 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/. 1 - c.1023C>T r.(?) p.(Ile341=) - benign g.78184737G>A g.80210938G>A SGSH(NM_000199.5):c.1023C>T (p.I341=) - SGSH_000026 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
?/. 1 - c.1049C>T r.(?) p.(Pro350Leu) - VUS g.78184711G>A - SGSH(NM_000199.4):c.1049C>T (p.P350L) - SGSH_000143 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 8 c.1063G>A r.(?) p.(Glu355Lys) - pathogenic g.78184697C>T g.80210898C>T 1075G>A - SGSH_000002 protein analysis (LC-MS/MS) brain SGSH absent PubMed: Sleat 2009 - - Germline - - - - - Sara Mole
?/. 1 - c.1079_1080del r.(?) p.(Thr360Serfs*141) - VUS g.78184680_78184681del g.80210881_80210882del - - SGSH_000007 1 more item - - - Germline - - - - - Gerard C.P. Schaafsma
+/. 8 8 c.1080del r.(?) p.(Val361Serfs*52), p.(Val361SerfsTer52) - pathogenic, pathogenic (recessive) g.78184680del, g.78184681del g.80210882del 1080delC, c.1080del, 1 more item - SGSH_000025 VKGL data sharing initiative Nederland PubMed: Ouesleti 2011, PubMed: Ousleti-2011, PubMed: Pollard 2016 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Rotterdam, VKGL-NL_Groningen
-/., -?/. 6 - c.1081G>A r.(?) p.(Val361Ile) - benign, likely benign g.78184679C>T g.80210880C>T 1 more item - SGSH_000024 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Utrecht, VKGL-NL_VUmc, VKGL-NL_AMC
-?/. 1 - c.1089C>T r.(?) p.(Gly363=) - likely benign g.78184671G>A - SGSH(NM_000199.4):c.1089C>T (p.G363=) - SGSH_000107 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 3 8 c.1093C>T r.(?) p.(Gln365*) - pathogenic, pathogenic (recessive) g.78184667G>A g.80210868G>A c.1093C>T - SGSH_000039 - PubMed: Ouesleti 2011, PubMed: Ousleti-2011 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.1103A>G r.(?) p.(His368Arg) - likely pathogenic (recessive) g.78184657T>C g.80210858T>C NM_000199.3:c.1103A>G:p.(His368Arg) - SGSH_000115 - PubMed: Maddirevula 2018 - - Germline - - - - - LOVD
+/. 3 - c.1105G>A r.(?) p.(Glu369Lys) - pathogenic, pathogenic (recessive) g.78184655C>T g.80210856C>T SGSH(NM_000199.4):c.1105G>A (p.E369K) - SGSH_000022 VKGL data sharing initiative Nederland PubMed: Pollard 2016 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
+/. 1 - c.1105G>T r.(?) p.(Glu369Ter) - pathogenic g.78184655C>A g.80210856C>A SGSH(NM_000199.4):c.1105G>T (p.E369*) - SGSH_000023 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 1 - c.1116G>A r.(?) p.(Met372Ile) - benign g.78184644C>T g.80210845C>T SGSH(NM_000199.5):c.1116G>A (p.M372I) - SGSH_000072 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 3 8 c.1129C>T r.(?) p.(Arg377Cys) - pathogenic, pathogenic (recessive) g.78184631G>A g.80210832G>A c.1129C>T - SGSH_000041 - PubMed: Fang 2022, PubMed: Ouesleti 2011, PubMed: Ousleti-2011 - - Germline - - - - - Johan den Dunnen
+/., +?/. 2 - c.1130G>A r.(?) p.(Arg377His) - likely pathogenic, pathogenic g.78184630C>T g.80210831C>T SGSH(NM_000199.4):c.1130G>A (p.R377H) - SGSH_000021 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
+?/. 1 - c.1130G>T r.(?) p.(Arg377Leu) - likely pathogenic (recessive) g.78184630C>A g.80210831C>A - - SGSH_000185 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.1135del r.(?) p.(Val379CysfsTer34) - pathogenic g.78184625del g.80210826del SGSH(NM_000199.4):c.1135delG (p.V379Cfs*34) - SGSH_000090 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 2 - c.1139A>G r.(?) p.(Gln380Arg) - pathogenic g.78184621T>C g.80210822T>C SGSH(NM_000199.3):c.1139A>G (p.(Gln380Arg)), SGSH(NM_000199.4):c.1139A>G (p.Q380R) - SGSH_000020 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
-?/. 1 - c.1144C>T r.(?) p.(Arg382Trp) - likely benign g.78184616G>A g.80210817G>A SGSH(NM_000199.3):c.1144C>T (p.(Arg382Trp)) - SGSH_000071 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/., -?/. 4 - c.1159G>A r.(?) p.(Val387Met) - benign, likely benign g.78184601C>T g.80210802C>T SGSH(NM_000199.3):c.1159G>A (p.(Val387Met)), SGSH(NM_000199.5):c.1159G>A (p.V387M) - SGSH_000019 178 heterozygous; Clinindb (India), 2 homozygous; Clinindb (India), 1 more item PubMed: Narang 2020, Journal: Narang 2020 - rs62620232 CLASSIFICATION record, Germline - 178/2794 individuals, 2/2794 individuals - - - VKGL-NL_Leiden, VKGL-NL_AMC, Mohammed Faruq
+?/. 2 - c.1166A>G r.(?) p.(Asn389Ser) - likely pathogenic (recessive) g.78184594T>C g.80210795T>C - - SGSH_000184 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.1167C>A r.(?) p.(Asn389Lys) - pathogenic g.78184593G>T - SGSH(NM_000199.5):c.1167C>A (p.N389K) - SGSH_000131 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. 1 - c.1182G>T r.(?) p.(Met394Ile) - likely benign g.78184578C>A g.80210779C>A SGSH(NM_000199.3):c.1182G>T (p.(Met394Ile)) - SGSH_000070 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 2 8 c.1207_1209del r.(?) p.(Tyr403del) - pathogenic g.78184552_78184554del g.80210753_80210755del SGSH(NM_000199.4):c.1207_1209delTAC (p.Y403del) - SGSH_000009 VKGL data sharing initiative Nederland, 1 more item - - - CLASSIFICATION record, Germline - - - - - VKGL-NL_Rotterdam, Dr. Alexandra Wey-Fabrizius
+?/. 1 - c.1209C>A r.(?) p.(Tyr403Ter) - likely pathogenic (recessive) g.78184551G>T g.80210752G>T - - SGSH_000183 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
-/. 1 - c.1209C>T r.(?) p.(Tyr403=) - benign g.78184551G>A g.80210752G>A SGSH(NM_000199.5):c.1209C>T (p.Y403=) - SGSH_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. 1 - c.1215A>C r.(?) p.(Ser405=) - likely benign g.78184545T>G - SGSH(NM_000199.4):c.1215A>C (p.S405=) - SGSH_000102 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.1231C>T r.(?) p.(Leu411Phe) - VUS g.78184529G>A - - - SGSH_000176 - - - rs1200985827 CLASSIFICATION record - - - - - MobiDetails
+?/. 1 - c.1243A>C r.(?) p.(Thr415Pro) - likely pathogenic (recessive) g.78184517T>G g.80210718T>G - - SGSH_000182 - PubMed: Pollard 2016 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.1283G>A r.(?) p.(Arg428His) - likely benign g.78184477C>T g.80210678C>T SGSH(NM_000199.4):c.1283G>A (p.R428H) - SGSH_000089 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
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