Unique variants in the ASS1 gene

Information The variants shown are described using the NM_000050.4 transcript reference sequence.

211 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.-6+8C>T r.(=) p.(=) - VUS g.133325728C>T g.130450341C>T ASS1(NM_000050.4):c.-6+8C>T (p.(=)) - ASS1_000101 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/. 1 - c.-5-13C>T r.(=) p.(=) - benign g.133327598C>T g.130452211C>T ASS1(NM_000050.4):c.-5-13C>T - ASS1_000106 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 1 2i c.-5-10C>G r.(-5_35del) p.)? - pathogenic (recessive) g.133327601C>G g.130452214C>G - - ASS1_000154 - PubMed: Diez-Fernandez 2017 - - Germline - - - - - Johan den Dunnen
+/. 2 2, 3 c.-4C>T r.(?) p.? - pathogenic, pathogenic (recessive) g.133327612C>T g.130452225C>T int0 -4C>T - ASS1_000055 described as nonsense variant; combination of variants not reported PubMed: Diez-Fernandez 2017, PubMed: Engel 2009 - - Germline - - - - - Johan den Dunnen
+/. 1 10i_12i c.(c.688+1_689-1)_(838+1_839-1)del r.689_838del p.? - pathogenic g.(133352349_133355102)_(133355837_133364719)del - - - ASS1_000008 combination of variants not reported PubMed: Engel 2009 - - Germline - - - - - Johan den Dunnen
+/. 1 _1_2i c.-356_(-6+1_-5-1){0} r.0? p.0? - pathogenic (recessive) g.(?_133320094)_(133325721_133327610)del g.(?_130444707)_(130450334_130452223)del del ex1-2 - ASS1_000206 - PubMed: Lee 2014 - - Germline - - - - - Johan den Dunnen
+/. 3 _1_1i c.-356_-68+75{0} -, r.(=|red) p.(=|red), p.=|0.03 - NA, pathogenic (recessive) g.133319980_133320457del g.130444593_130445070del hg19 133319979-133320456del - ASS1_000205 expression cloning (A2058 and HepG2 cells) shows reduced reporter expression (0.033-0.036), 1 more item PubMed: Imagawa 2020 - - Germline, In vitro (cloned) - - - - - Johan den Dunnen
?/. 1 - c.18C>T r.(?) p.(Ser6=) - VUS g.133327633C>T - ASS1(NM_054012.4):c.18C>T (p.(Ser6=)) - ASS1_000121 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.19G>A - p.Val7Met - VUS g.133327634G>A g.130452247G>A - - ASS1_000155 <0.80 no residual enzymatic ASS1 activity combined variants PubMed: Zielonka 2019 - - Germline - - - - - Johan den Dunnen
+?/. 1 2 c.34A>G r.(?) p.(Ser12Gly) ACMG likely pathogenic (recessive) g.133327649A>G g.130452262A>G - - ASS1_000136 1 more item - - rs1283372037 Germline yes - - - - Miriam Erandi Reyna-Fabián
+/. 7 2, 3 c.40G>A r.(?), r.40g>a p.(Gly14Ser), p.Gly14Ser ACMG pathogenic (recessive) g.133327655G>A g.130452268G>A G14S - ASS1_000056 plasma citrulline 1100 umol/L, plasma citrulline 3809 μmol/L, plasma citrulline 747 umol/L, 1 more item Kobayashi ASH1989 A201, PubMed: Kobayashi 1990, OMIM:var0004, PubMed: Diez-Fernandez 2016, 3 more items ClinVar-6324 rs121908636 Germline yes - - - - Johan den Dunnen, Miriam Erandi Reyna-Fabián
+/. 1 3 c.53C>T r.53c>u p.Ser18Leu - pathogenic (recessive) g.133327668C>T g.130452281C>T - - ASS1_000057 plasma citrulline elevated PubMed: Kobayashi 1991, PubMed: Kobayashi 1994, OMIM:var0011 - - Germline - - - - - Johan den Dunnen
+/. 1 3 c.55T>C r.(?) p.(Cys19Arg) - pathogenic (recessive) g.133327670T>C g.130452283T>C - - ASS1_000058 plasma citrulline 3510 umol/L PubMed: Gao 2003 - - Germline - - - - - Johan den Dunnen
+/. 5 3 c.79C>T r.(?) p.(Gln27Ter) - pathogenic (recessive) g.133327694C>T g.130452307C>T 79T>C - ASS1_000156 - PubMed: Diez-Fernandez 2017, PubMed: Silvera-Ruiz 2019 - - Germline - 1/24 case chromosomes - - - Johan den Dunnen
-/. 1 - c.99C>T r.(?) p.(Ala33=) - benign g.133327714C>T g.130452327C>T ASS1(NM_000050.4):c.99C>T (p.A33=) - ASS1_000107 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 1 3 c.100dup r.(?) p.(Tyr34LeufsTer29) - pathogenic (recessive) g.133327715dup g.130452328dup 100dupT - ASS1_000157 - PubMed: Diez-Fernandez 2017 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.102T>G - p.Tyr34Ter - pathogenic (recessive) g.133327717T>G g.130452330T>G - - ASS1_000158 <0.20 no residual enzymatic ASS1 activity combined variants PubMed: Zielonka 2019 - - Germline - - - - - Johan den Dunnen
-/. 1 - c.106-10T>G r.(=) p.(=) - benign g.133329682T>G g.130454295T>G ASS1(NM_000050.4):c.106-10T>G - ASS1_000108 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 2 4 c.119A>T r.(?) p.(Gln40Leu) - pathogenic (recessive) g.133329705A>T g.130454318A>T - - ASS1_000059 citrulline level 100–200 (109) uM, plasma citrulline 2517 umol/L PubMed: Dimmock 2008, PubMed: Haberle 2003 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.132C>T r.(?) p.(Phe44=) - likely benign g.133329718C>T g.130454331C>T ASS1(NM_000050.4):c.132C>T (p.F44=) - ASS1_000113 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.133G>A r.(?) p.(Glu45Lys) - VUS g.133329719G>A g.130454332G>A - - ASS1_000151 - PubMed: Xiong 2022 - - Germline - - - - - Johan den Dunnen
+/. 1 4i c.174+1G>A r.(106_174del) p.(Ala36_Lys58del) - pathogenic (recessive) g.133329761G>A g.130454374G>A - - ASS1_000143 plasma citrulline 961 umol/L; effect on splicing predicted from pSPL3 splicing assay PubMed: Kimani 2015 - - Germline - - - - - Johan den Dunnen
+/. 1 4i c.174+1G>T r.spl p.? - pathogenic (recessive) g.133329761G>T g.130454374G>T IVS4+1G>T - ASS1_000061 - PubMed: Kleijer 2006 - - Germline - - - - - Johan den Dunnen
+/. 2 4i c.175-1119G>A c.174_175ins175-1180_175-1112, r.(?) p.(Lys58fs), p.Val59ProfsTer14 - pathogenic (recessive) g.133332669G>A g.130457282G>A (Lys58_Val59ins12*), g.4967G>A (Lys58ins69bpX13) - ASS1_000147 plasma citrulline 1720 umol/L PubMed: Diez-Fernandez 2017, PubMed: Faghfoury 2011 - - Germline - - - - - Johan den Dunnen
+/. 4 4i_5i c.(174+1_175-1)_(363+1_364-1)del r.175_363del, r.del p.del, p.Val59_Lys121 - pathogenic, pathogenic (recessive) g.(133329761_133333787)_(133333977_133339497)del g.(130454374_130458400)_(130458590_130464110)del ex5 del - ASS1_000062 3-4 Kb deletion, no variant 2nd chromosome Kobayashi ASH1989 A201, {Kobayashi 1990}, PubMed: Kobayashi 1990, OMIM:var0006, PubMed: Kobayashi 1990, 2 more items - - Germline - - - - - Johan den Dunnen
+/. 1 5 c.190G>A r.(?) p.(Val64Ile) - pathogenic (recessive) g.133333803G>A g.130458416G>A - - ASS1_000159 slightly elevated PubMed: Diez-Fernandez 2017 - - Germline - - - - - Johan den Dunnen
+/. 4 5 c.206T>C -, r.(?) p.(Val69Ala), p.Val69Ala - pathogenic (recessive) g.133333819T>C g.130458432T>C - - ASS1_000063 <0.30 no residual enzymatic ASS1 activity combined variants, plasma citrulline 2551 umol/l PubMed: Diez-Fernandez 2017, PubMed: Martin-Hernandez 2014, PubMed: Vilaseca 2001, 1 more item - - Germline - - - - - Johan den Dunnen
+/., ?/. 2 - c.216C>G -, r.(?) p.(Phe72Leu), p.Phe72Leu - pathogenic (recessive), VUS g.133333829C>G g.130458442C>G - - ASS1_000138 citrulline level 200–400 uM, near normal enzymatic ASS1 activity PubMed: Dimmock 2008, PubMed: Zielonka 2019 - - Germline - - - - - Johan den Dunnen
+/. 2 5 c.235T>C r.(?) p.(Ser79Pro) - pathogenic, pathogenic (recessive) g.133333848T>C g.130458461T>C - - ASS1_000064 combination of variants not reported, plasma citrulline 1000 umol/L PubMed: Diez-Fernandez 2017, PubMed: Engel 2009 - - Germline - - - - - Johan den Dunnen
+/. 14 4, 5 c.256C>T -, r.(?), r.256c>u p.(Arg86Cys), p.Arg86Cys ACMG pathogenic, pathogenic (recessive) g.133333869C>T g.130458482C>T - - ASS1_000065 <0.20 no residual enzymatic ASS1 activity, plasma citrulline 1437 umol/L PubMed: Diez-Fernandez 2017, PubMed: Kobayashi 1991, OMIM:var0012, PubMed: Martin-Hernandez 2014, 2 more items 6332, ClinVar-6332 rs121908644 Germline yes - - - - Johan den Dunnen, Miriam Erandi Reyna-Fabián
+/., +?/. 3 5 c.257G>A r.(?), r.257g>a p.(Arg86His), p.Arg86His - likely pathogenic (recessive), pathogenic (recessive) g.133333870G>A g.130458483G>A - - ASS1_000066 plasma citrulline 1383 μmol/L, plasma citrulline 903 μmol/L, 1 more item PubMed: Diez-Fernandez 2017, PubMed: Gao 2003 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.262C>A r.(?) p.(Leu88Ile) - pathogenic (recessive) g.133333875C>A g.130458488C>A 262C>G (L88I) - ASS1_000139 citrulline level <1000 uM; citruline enzyme activity 0 nM/min/mg protein PubMed: Dimmock 2008 - - Germline - - - - - Johan den Dunnen
+/., ?/. 4 - c.271A>C -, r.(?) p.(Thr91Pro), p.Thr91Pro - NA, pathogenic (recessive) g.133333884A>C g.130458497A>C T91P - ASS1_000127 <0.05 no residual enzymatic ASS1 activity, plasma citrulline 2417 μmol/L, 1 more item PubMed: Diez-Fernandez 2016, PubMed: Miller 2014, PubMed: Zielonka 2019 - - Germline, In vitro (cloned) - - - - - Johan den Dunnen
+/. 2 - c.271A>G -, r.(?) p.(Thr91Ala), p.Thr91Ala - pathogenic (recessive) g.133333884A>G g.130458497A>G - - ASS1_000125 <0.50 no residual enzymatic ASS1 activity combined variants PubMed: Martin-Hernandez 2014, PubMed: Zielonka 2019 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.282C>T r.(?) p.(Ala94=) - likely benign g.133333895C>T g.130458508C>T ASS1(NM_000050.4):c.282C>T (p.A94=) - ASS1_000114 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 - c.285G>C - p.Arg95Ser - NA g.133333898G>C g.130458511G>C R95S - ASS1_000140 cDNA expression cloning shows no argininosuccinate synthetase enzyme activity PubMed: Diez-Fernandez 2016 - - In vitro (cloned) - - - - - Johan den Dunnen
+/. 1 5 c.285_286delinsTT r.(?) p.(Arg95_Pro96delinsSerSer) - pathogenic (recessive) g.133333898_133333899delinsTT g.130458511_130458512delinsTT [285G>T;286C>T] [R95S;P96S] - ASS1_000128 plasma citrulline 1718 μmol/L (ref<50) PubMed: Gao 2003 - - Germline - - - - - Johan den Dunnen
+?/. 1 - c.286C>T - p.Pro96Ser - NA g.133333899C>T g.130458512C>T P96S - ASS1_000141 cDNA expression cloning shows 0.35 argininosuccinate synthetase enzyme activity PubMed: Diez-Fernandez 2016 - - In vitro (cloned) - - - - - Johan den Dunnen
+/., +?/. 2 5 c.287C>A -, r.(?) p.(Pro96His), p.Pro96His - NA, pathogenic (recessive) g.133333900C>A g.130458513C>A P96H - ASS1_000069 cDNA expression cloning shows 0.25 argininosuccinate synthetase enzyme activity PubMed: Diez-Fernandez 2016, PubMed: Engel 2009, PubMed: Diez-Fernandez 2016 - - Germline, In vitro (cloned) - - - - - Johan den Dunnen
+/. 3 - c.287C>T -, r.(?) p.(Pro96Leu), p.Pro96Leu - NA, pathogenic (recessive) g.133333900C>T g.130458513C>T P96L - ASS1_000130 cDNA expression cloning shows no argininosuccinate synthetase enzyme activity, 1 more item PubMed: Diez-Fernandez 2016, PubMed: Faghfoury 2011 - - Germline, In vitro (cloned) - - - - - Johan den Dunnen
+/. 2 5 c.291C>A -, r.(?) p.(Cys97Ter), p.Cys97Ter - pathogenic (recessive) g.133333904C>A g.130458517C>A - - ASS1_000160 no residual enzymatic ASS1 activity, plasma citrulline >4000 umol/L PubMed: Diez-Fernandez 2017, PubMed: Zielonka 2019 - - Germline - - - - - Johan den Dunnen
+/. 1 5 c.298C>T r.(?) p.(Arg100Cys) - pathogenic (recessive) g.133333911C>T g.130458524C>T - - ASS1_000161 plasma citrulline 200 umol/L PubMed: Diez-Fernandez 2017 - - Germline - - - - - Johan den Dunnen
+/. 1 5 c.299del r.(?) p.(Arg100Profs*40) - pathogenic (recessive) g.133333912del g.130458525del - - ASS1_000070 plasma citrulline 3248 μmol/L PubMed: Gao 2003 - - Germline - - - - - Johan den Dunnen
+/. 2 5 c.299G>A -, r.(?) p.(Arg100His), p.Arg100His - pathogenic (recessive) g.133333912G>A g.130458525G>A - - ASS1_000162 <0.80 no residual enzymatic ASS1 activity combined variants PubMed: Diez-Fernandez 2017, PubMed: Zielonka 2019 - - Germline - - - - - Johan den Dunnen
+/. 1 5 c.304del r.(?) p.(Gln102LysfsTer38) - pathogenic (recessive) g.133333917del g.130458530del 304delC - ASS1_000163 plasma citrulline 1800 umol/L PubMed: Diez-Fernandez 2017 - - Germline - - - - - Johan den Dunnen
+/., -/., -?/., ?/. 8 5 c.323G>T r.(?) p.(Arg108Leu) - benign, likely benign, pathogenic (recessive), VUS g.133333936G>T g.130458549G>T ASS1(NM_000050.4):c.323G>T (p.R108L) - ASS1_000071 no variant 2nd chromosome;plasma citrulline 914 umol/l, plasma citrulline 736 umol/L, 2 more items PubMed: Diez-Fernandez 2017, PubMed: Haberle 2002, OMIM:var0014, 1 more item - - CLASSIFICATION record, Germline - - - - - Gerard C.P. Schaafsma, Johan den Dunnen, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_AMC
+/. 1 5 c.332C>A r.(?) p.(Ala111Asp) - pathogenic (recessive) g.133333945C>A g.130458558C>A - - ASS1_000164 - PubMed: Diez-Fernandez 2017 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.333C>T r.(?) p.(Ala111=) - likely benign g.133333946C>T g.130458559C>T ASS1(NM_000050.4):c.333C>T (p.A111=) - ASS1_000115 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/., +?/. 6 5 c.349G>A -, r.(?) p.(Gly117Ser), p.Gly117Ser - likely pathogenic (recessive), NA, pathogenic (recessive) g.133333962G>A g.130458575G>A G117S - ASS1_000073 <0.20 no residual enzymatic ASS1 activity, plasma citrulline 1218 μmol/L, 4 more items PubMed: Diez-Fernandez 2016, PubMed: Gao 2003, PubMed: Zielonka 2019 - - Germline, In vitro (cloned) - - - - - Johan den Dunnen
+/. 1 5 c.349G>T r.(?) p.(Gly117Cys) - pathogenic (recessive) g.133333962G>T g.130458575G>T - - ASS1_000165 - PubMed: Diez-Fernandez 2017 - - Germline - - - - - Johan den Dunnen
+/., +?/. 4 5 c.350G>A r.(?) p.(Gly117Asp) - likely pathogenic (recessive), pathogenic (recessive) g.133333963G>A g.130458576G>A - - ASS1_000074 plasma citrulline 1504 μmol/L, plasma citrulline 1785 umol/l PubMed: Gao 2003, PubMed: Moarefian 2022, PubMed: Vilaseca 2001, PubMed: Martin-Hernandez 2014 - - Germline yes 1/10 families - - - Johan den Dunnen
-/. 1 4 c.351C>T r.(?) p.(=) - benign g.133333964C>T g.130458577C>T - - ASS1_000060 - PubMed: Engel 2009 - rs35772983 Germline - - - - - Johan den Dunnen
+/., +?/., ?/. 12 5 c.352G>A -, r.(?), r.352g>a p.(Ala118Thr), p.Ala118Thr - likely pathogenic (recessive), NA, pathogenic (recessive), VUS g.133333965G>A g.130458578G>A A118T, ASS1(NM_000050.4):c.352G>A (p.A118T) - ASS1_000075 <0.20 no residual enzymatic ASS1 activity combined variants, plasma citrulline 1000 umol/L, 8 more items PubMed: Diez-Fernandez 2016, PubMed: Diez-Fernandez 2017, PubMed: Engel 2009, PubMed: Gao 2003, 4 more items - - CLASSIFICATION record, Germline, In vitro (cloned) - - - - - Johan den Dunnen, VKGL-NL_Rotterdam
+/., ?/. 4 5 c.356C>T -, r.(?) p.(Thr119Ile), p.Thr119Ile - NA, pathogenic (recessive), VUS g.133333969C>T g.130458582C>T T119I - ASS1_000076 cDNA expression cloning shows no argininosuccinate synthetase enzyme activity, 3 more items PubMed: Diez-Fernandez 2016, PubMed: Diez-Fernandez 2017, 1 more item - - Germline, In vitro (cloned) - - - - - Johan den Dunnen
-?/., ?/. 2 - c.364-6T>C r.(=) p.(=) - likely benign, VUS g.133339492T>C g.130464105T>C ASS1(NM_000050.4):c.364-6T>C (p.(=)) - ASS1_000102 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_AMC
+/. 1 5i c.364-2A>G r.spl p.? - pathogenic (recessive) g.133339496A>G g.130464109A>G - - ASS1_000166 - PubMed: Diez-Fernandez 2017 - - Germline - - - - - Johan den Dunnen
+/. 1 5i_6i c.(363+1_364-1)_(420+1_421-1)del r.del p.del - pathogenic g.(133333977_133339497)_(133339555_133342111)del - ex6 del - ASS1_000077 2-3 Kb deletion Kobayashi ASH1989 A201, PubMed: Kobayashi 1995, OMIM:var0001 - - Germline - - - - - Johan den Dunnen
+/., +?/. 4 6 c.370G>A -, r.(?) p.(Asp124Asn), p.Asp124Asn - likely pathogenic (recessive), NA, pathogenic (recessive) g.133339504G>A g.130464117G>A D124N - ASS1_000078 <0.30 no residual enzymatic ASS1 activity combined variants, 1 more item PubMed: Diez-Fernandez 2016, PubMed: Kleijer 2006, PubMed: Zielonka 2019 - - Germline, In vitro (cloned) - - - - - Johan den Dunnen
+/. 1 - c.372T>A - p.Asp124Glu - pathogenic (recessive) g.133339506T>A g.130464119T>A - - ASS1_000167 <0.60 no residual enzymatic ASS1 activity combined variants PubMed: Zielonka 2019 - - Germline - - - - - Johan den Dunnen
+/. 3 - c.379C>G r.(?) p.(Arg127Gly) - pathogenic (recessive) g.133339513C>G g.130464126C>G - - ASS1_000168 plasma citrulline 1447 umol/L, plasma citrulline 969 umol/L PubMed: Lee 2013, PubMed: Woo 2013 - - Germline - - - - - Johan den Dunnen
+/. 3 6 c.379C>T -, r.(?) p.(Arg127Trp), p.Arg127Trp - NA, pathogenic g.133339513C>T g.130464126C>T R127W - ASS1_000079 cDNA expression cloning shows no argininosuccinate synthetase enzyme activity, 1 more item PubMed: Diez-Fernandez 2016, PubMed: Engel 2009 - - Germline, In vitro (cloned) - - - - - Johan den Dunnen
+/. 8 6 c.380G>A -, r.(?) p.(Arg127Gln), p.Arg127Gln - NA, pathogenic, pathogenic (recessive) g.133339514G>A g.130464127G>A R127Q - ASS1_000080 <0.20 no residual enzymatic ASS1 activity, combination of variants not reported, 3 more items PubMed: Diez-Fernandez 2016, PubMed: Diez-Fernandez 2017, PubMed: Engel 2009, PubMed: Haberle 2003, 4 more items - - Germline, In vitro (cloned) - - - - - Johan den Dunnen
+/., ?/. 2 - c.380G>T -, r.(?) p.(Arg127Leu), p.Arg127Leu - NA, VUS g.133339514G>T g.130464127G>T R127L - ASS1_000134 cDNA expression cloning shows no argininosuccinate synthetase enzyme activity PubMed: Diez-Fernandez 2016, PubMed: Miller 2014 - - Germline, In vitro (cloned) - - - - - Johan den Dunnen
+/. 2 6 c.412C>T r.(?) p.(Gln138*), p.(Gln138Ter) - pathogenic, pathogenic (recessive) g.133339546C>T g.130464159C>T ASS1(NM_054012.3):c.412C>T (p.(Gln138*)) - ASS1_000169 VKGL data sharing initiative Nederland PubMed: Diez-Fernandez 2017 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Leiden
-/. 1 - c.421-19C>T r.(=) p.(=) - benign g.133342093C>T g.130466706C>T ASS1(NM_000050.4):c.421-19C>T - ASS1_000109 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 52 6i c.421-2A>G r.(421_495del), r.421_495del, r.spl, r.spl? p.(Val141_Lys165del), p.?, p.del, p.Val141_Lys165del - pathogenic, pathogenic (recessive) g.133342110A>G g.130466723A>G ASS1(NM_000050.4):c.421-2A>G, del ex7, IVS6-2A>G - ASS1_000081 patient chromosomes, plasma citrulline 1376 umol/L, plasma citrulline 1836 umol/L, 17 more items Kobayashi ASH1989 A201, PubMed: Kobayashi 1995, OMIM:var0003, PubMed: Ban 2001, PubMed: Gao 2003, 11 more items - - CLASSIFICATION record, Germline - 19/33 MspI- - - Johan den Dunnen, VKGL-NL_Groningen
+/. 1 7 c.422T>G r.(422u>g,421_495del) p.(Val141Gly,Val141_Lys165del) - pathogenic (recessive) g.133342113T>G g.130466726T>G - - ASS1_000144 plasma citrulline 961 umol/L; effect on splicing predicted from pSPL3 splicing assay PubMed: Kimani 2015 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.428C>A r.(?) p.(Ala143Asp) - VUS g.133342119C>A - - - ASS1_000120 - - - - Germline/De novo (untested) - - - - - Gemeinschaftspraxis für Humangenetik Dresden
+?/. 2 7 c.437G>A r.(?) p.(Arg146Lys) - likely pathogenic g.133342128G>A g.130466741G>A - - ASS1_000104 - PubMed: Navarrete 2019 - - Germline yes - - - - Johan den Dunnen, Belen Perez
+/. 1 - c.440T>C r.(?) p.(Met147Thr) - pathogenic (recessive) g.133342131T>C g.130466744T>C - - ASS1_000209 - PubMed: Moarefian 2022 - - Germline yes 1/10 families - - - Johan den Dunnen
+/. 2 7 c.450_451del r.(?) p.(Phe150Leufs*9), p.(Phe150LeufsTer9) - pathogenic, pathogenic (recessive) g.133342141_133342142del g.130466754_130466755del 450_451delCT, c.450_451delCT - ASS1_000082 combination of variants not reported, unknown variant 2nd chromosome; plasma citrulline 1778 umol/L PubMed: Diez-Fernandez 2017, PubMed: Engel 2009 - - Germline - - - - - Johan den Dunnen
+/. 2 7 c.460_467del r.(?) p.(Phe154Profs*3), p.(Phe154ProfsTer3) - pathogenic, pathogenic (recessive) g.133342151_133342158del g.130466764_130466771del 459_466del - ASS1_000083 combination of variants not reported PubMed: Diez-Fernandez 2017, PubMed: Engel 2009 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.467G>A - p.Gly156Asp - pathogenic (recessive) g.133342158G>A g.130466771G>A - - ASS1_000170 <0.20 no residual enzymatic ASS1 activity PubMed: Zielonka 2019 - - Germline - - - - - Johan den Dunnen
+/. 1 7 c.469C>A r.(?) p.(Arg157Ser) - pathogenic (recessive) g.133342160C>A g.130466773C>A - - ASS1_000171 plasma citrulline 2877 umol/L PubMed: Diez-Fernandez 2017 - - Germline - - - - - Johan den Dunnen
+/. 8 7 c.469C>T -, r.(?) p.(Arg157Cys), p.Arg157Cys - NA, pathogenic (recessive) g.133342160C>T g.130466773C>T R157C - ASS1_000084 <0.10 no residual enzymatic ASS1 activity combined variants, plasma citrulline 200 umol/L, 3 more items PubMed: Diez-Fernandez 2016, PubMed: Diez-Fernandez 2017, PubMed: Gao 2003, PubMed: Miller 2014, 1 more item - - Germline, In vitro (cloned) - - - - - Johan den Dunnen
+/., ?/. 20 7 c.470G>A -, r.(?), r.470g>a p.(Arg157His), p.Arg157His - NA, pathogenic (recessive), VUS g.133342161G>A g.130466774G>A R157H - ASS1_000085 <0.05 no residual enzymatic ASS1 activity, plasma citrulline 200 umol/L, plasma citrulline high, 6 more items Kobayashi ASH1989 A201, PubMed: Kobayashi 1990, OMIM:var0005, PubMed: Diez-Fernandez 2016, 5 more items - rs121908637 Germline, In vitro (cloned) yes 1/10 families, 1/2795 individuals - - - Johan den Dunnen, Mohammed Faruq
+/. 4 7 c.479T>C r.(?) p.(Leu160Pro) - pathogenic, pathogenic (recessive) g.133342170T>C g.130466783T>C - - ASS1_000086 combination of variants not reported PubMed: Diez-Fernandez 2017, PubMed: Engel 2009 - - Germline - - - - - Johan den Dunnen
+/. 1 7 c.489C>A r.(?) p.(Tyr163Ter) - pathogenic (recessive) g.133342180C>A g.130466793C>A - - ASS1_000172 - PubMed: Diez-Fernandez 2017 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.489C>T r.(?) p.(Tyr163=) - likely benign g.133342180C>T - ASS1(NM_000050.4):c.489C>T (p.Y163=) - ASS1_000118 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 4 7 c.490G>C r.(?) p.(Ala164Pro) - pathogenic (recessive) g.133342181G>C g.130466794G>C - - ASS1_000173 plasma citrulline 1789 umol/L, plasma citrulline 1975 umol/L, plasma citrulline 2247 umol/L, 1 more item PubMed: Diez-Fernandez 2017 - - Germline - - - - - Johan den Dunnen
+/. 2 7i c.495+1G>T r.421_495del, r.spl p.(Val141_Lys165del), p.Val141_Lys165del - pathogenic, pathogenic (recessive) g.133342187G>T g.130466800G>T - - ASS1_000087 combination of variants not reported PubMed: Diez-Fernandez 2017, PubMed: Engel 2009 - - Germline - - - - - Johan den Dunnen
+/., ?/. 2 - c.496-1766_597+732delinsGC r.(496_597del), r.496_597delinsGC p.? - pathogenic, VUS g.133344455_133345489delinsGC, g.133344455_133347634delinsGC g.130469068_130472247delinsGC - - ASS1_000105 1 more item PubMed: Navarrete 2019 - - Germline - - - - - Johan den Dunnen, Belen Perez
+/. 3 7i_8 c.496-21_515del r.(?), r.spl p.(Gln166GlyfsTer11), p.del - pathogenic, pathogenic (recessive) g.133346200_133346240del g.130470813_130470853del - - ASS1_000088 combination of variants not reported PubMed: Diez-Fernandez 2017, PubMed: Engel 2009 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.496-4G>C r.spl? p.? - likely benign g.133346217G>C - ASS1(NM_054012.4):c.496-4G>C - ASS1_000122 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 7i c.496-2A>G r.spl p.? - pathogenic (recessive) g.133346219A>G g.130470832A>G - - ASS1_000174 - PubMed: Diez-Fernandez 2017 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.499_500del - p.His167ArgfsTer16 - pathogenic (recessive) g.133346225_133346226del g.130470838_130470839del - - ASS1_000175 <0.10 no residual enzymatic ASS1 activity combined variants PubMed: Zielonka 2019 - - Germline - - - - - Johan den Dunnen
-/. 4 8 c.501C>T r.(?) p.(=), p.(His167=) - benign g.133346226C>T g.130470839C>T ASS1(NM_000050.4):c.501C>T (p.H167=), His167His - ASS1_000001 VKGL data sharing initiative Nederland PubMed: Diez-Fernandez 2017, PubMed: Engel 2009, PubMed: Mitchell 2009 - rs10901072 CLASSIFICATION record, Germline - 15/94 - - - Johan den Dunnen, VKGL-NL_AMC
+/., +?/. 33 8 c.535T>C -, r.(?) p.(Trp179Arg), p.Trp179Arg - likely pathogenic (recessive), pathogenic (recessive) g.133346260T>C g.130470873T>C - - ASS1_000089 <0.10 no residual enzymatic ASS1 activity combined variants, citrulline level 300–1000 uM, 19 more items PubMed: Diez-Fernandez 2017, PubMed: Dimmock 2008, PubMed: Gao 2003, PubMed: Haberle 2003, 2 more items - - Germline - - - - - Johan den Dunnen
+/. 5 - c.539G>A -, r.(?), r.539g>a p.(Ser180Asn), p.Ser180Asn - NA, pathogenic (recessive) g.133346264G>A g.130470877G>A S180N - ASS1_000091 <0.30 no residual enzymatic ASS1 activity combined variants, citrulline level 100–200 (109) uM, 3 more items Kobayashi ASH1989 A201, PubMed: Kobayashi 1990, PubMed: Diez-Fernandez 2016, PubMed: Dimmock 2008, 1 more item - - Germline, In vitro (cloned) - - - - - Johan den Dunnen
+/., ?/. 2 - c.539G>T -, r.(?) p.(Ser180Ile), p.Ser180Ile - NA, VUS g.133346264G>T g.130470877G>T S180I - ASS1_000133 cDNA expression cloning shows no argininosuccinate synthetase enzyme activity PubMed: Diez-Fernandez 2016, PubMed: Miller 2014 - - Germline, In vitro (cloned) - - - - - Johan den Dunnen
+/. 1 8 c.547_561delinsAGAGAACCTCATGCACAT r.(?) p.(Glu183_Met186delinsArgGluProHisAla) - pathogenic (recessive) g.133346272_133346286delinsAGAGAACCTCATGCACAT g.130470885_130470899delinsAGAGAACCTCATGCACAT [546_547insA;562_563dupAT] - ASS1_000176 - PubMed: Diez-Fernandez 2017 - - Germline - - - - - Johan den Dunnen
+/. 2 8 c.552C>A r.(?) p.(Asn184Lys) - pathogenic (recessive) g.133346277C>A g.130470890C>A - - ASS1_000153 mild citrullinemia PubMed: Diez-Fernandez 2017, PubMed: Xiong 2022 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.556A>G - p.Met186Val - pathogenic (recessive) g.133346281A>G g.130470894A>G - - ASS1_000177 <0.30 no residual enzymatic ASS1 activity combined variants PubMed: Zielonka 2019 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.557T>G r.(?) p.(Met186Arg) - pathogenic (recessive) g.133346282T>G g.130470895T>G - - ASS1_000126 - PubMed: Martin-Hernandez 2014 - - Germline - - - - - Johan den Dunnen
-/. 1 8 c.561C>T r.(?) p.(=) - benign g.133346286C>T g.130470899C>T - - ASS1_000100 - PubMed: Engel 2009 - rs2297599 Germline - - - - - Johan den Dunnen
-/. 2 8 c.564C>T r.(?) p.(=) - benign g.133346289C>T g.130470902C>T Ile188Ile - ASS1_000002 - PubMed: Mitchell 2009 - rs2297599 Germline - 1/94 - - - Johan den Dunnen
-?/. 1 8i c.566+80G>A r.566_567= p.= - benign g.133346371G>A g.130470984G>A - - ASS1_000208 - PubMed: Sun 2018 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.567-4G>A r.spl? p.? - likely benign g.133346868G>A - ASS1(NM_000050.4):c.567-4G>A - ASS1_000116 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 - c.568T>G r.(?) p.(Tyr190Asp) - pathogenic (recessive) g.133346873T>G g.130471486T>G - - ASS1_000092 - PubMed: Haberle 2003 - - Germline - - - - - Johan den Dunnen
+/. 9 9 c.571G>A -, r.(?), r.579g>a p.(Glu191Lys), p.Glu191Lys - pathogenic (recessive) g.133346876G>A g.130471489G>A E191K - ASS1_000093 <0.10 no residual enzymatic ASS1 activity, plasma citrulline 1172 umol/L, 4 more items PubMed: Diez-Fernandez 2017, PubMed: Gao 2003, PubMed: Haberle 2004, PubMed: Zielonka 2019 - - Germline - - - - - Johan den Dunnen
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