All variants in the CPS1 gene

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

461 entries on 5 pages. Showing entries 1 - 100.
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Effect     

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     
-/. - c.-16G>A r.(?) p.(=) - benign g.211342472G>A g.210477748G>A CPS1(NM_001122633.3):c.-34G>A - CPS1_000244 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. - c.-16G>A r.(=) p.(=) - benign g.211342472G>A g.210477748G>A - - CPS1_000244 3 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs111491997 Germline - 3/2792 individuals - - - Mohammed Faruq
+/. _1_39_ c.(?_-1)_(*1_?)del r.0 p.0 - pathogenic g.(?_g.211342487)_(211542710_?)del - - - CPS1_000000 no 2nd allele found in cDNA PubMed: Summar 1998 - - Germline - - - - - Johan den Dunnen
+/. _1_39_ c.(?_-1)_(*1_?)del r.0 p.0 - pathogenic g.(?_g.211342487)_(211542710_?)del - - - CPS1_000000 - PubMed: Summar 1998 - - Germline - - - - - Johan den Dunnen
-/. 34i c.4021-108_4021-81GT[?] r.(?) p.(=) - benign g.211532802_211532829GT[?] - 4003-82TG(n) - CPS1_000018 47 individuals tested PubMed: Mitchell 2009 - - Germline - - - - - Johan den Dunnen
-/. 6i c.546+40GT(n) r.(?) p.(=) - benign g.? - 528+40GT(n) - CPS1_000002 47 individuals tested PubMed: Mitchell 2009 - - Germline - - - - - Johan den Dunnen
-/. - c.3+12G>A r.(=) p.(=) - benign g.211342502G>A g.210477778G>A CPS1(NM_001122633.2):c.3+12G>A, CPS1(NM_001122633.3):c.-16+12G>A - CPS1_000245 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. - c.3+12G>A r.(=) p.(=) - benign g.211342502G>A g.210477778G>A CPS1(NM_001122633.2):c.3+12G>A, CPS1(NM_001122633.3):c.-16+12G>A - CPS1_000245 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. - c.3+12G>A r.(=) p.(=) - benign g.211342502G>A g.210477778G>A CPS1(NM_001122633.2):c.3+12G>A, CPS1(NM_001122633.3):c.-16+12G>A - CPS1_000245 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. - c.4-14T>G r.(=) p.(=) - benign g.211421429T>G g.210556705T>G CPS1(NM_001875.5):c.-29T>G - CPS1_000246 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. - c.15_16insTTC r.(?) p.(Ile5_Lys6insPhe) - benign g.211421454_211421455insTTC g.210556730_210556731insTTC CPS1(NM_001122633.2):c.15_16insTTC (p.I5_K6insF), CPS1(NM_001122633.3):c.-4_-3insTTC - CPS1_000269 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. - c.15_16insTTC r.(?) p.(Ile5_Lys6insPhe) - benign g.211421454_211421455insTTC g.210556730_210556731insTTC CPS1(NM_001122633.2):c.15_16insTTC (p.I5_K6insF), CPS1(NM_001122633.3):c.-4_-3insTTC - CPS1_000269 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. - c.15_16insTTC r.(?) p.(Ile5_Lys6insPhe) - benign g.211421454_211421455insTTC g.210556730_210556731insTTC CPS1(NM_001122633.2):c.15_16insTTC (p.I5_K6insF), CPS1(NM_001122633.3):c.-4_-3insTTC - CPS1_000269 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-/. - c.15_16insTTC r.(?) p.(Ile5_Lys6insPhe) - benign g.211421454_211421455insTTC g.210556730_210556731insTTC CPS1(NM_001122633.2):c.15_16insTTC (p.I5_K6insF), CPS1(NM_001122633.3):c.-4_-3insTTC - CPS1_000269 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 2 c.143del r.(?) p.(Lys48Argfs*15) - pathogenic g.211421582del g.210556858del 125delA - CPS1_000027 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 3 c.146C>T r.(?) p.(Ala49Val) - pathogenic g.211438023C>T g.210573299C>T 128C>T - CPS1_000025 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 3 c.148C>T r.(?) p.(Gln50*) - pathogenic g.211438025C>T g.210573301C>T 130C>T - CPS1_000024 - PubMed: Kurokawa 2007, OMIM:var0003 - - Germline - - - - - Johan den Dunnen
+/. 3 c.148C>T r.(?) p.(Gln50*) - pathogenic g.211438025C>T g.210573301C>T 130C>T - CPS1_000024 - PubMed: Kurokawa 2007, OMIM:var0003 - - Germline - - - - - Johan den Dunnen
+/. 3 c.148C>T r.(?) p.(Gln50*) - pathogenic g.211438025C>T g.210573301C>T 130C>T - CPS1_000024 unknown pathogenic variant in 2nd chromosome Ihara 1999 (HumGenet 105:375), OMIM:var0003 - - Germline - - - - - Johan den Dunnen
?/. - c.172G>A r.(?) p.(Asp58Asn) - VUS g.211438049G>A g.210573325G>A CPS1(NM_001875.5):c.154G>A (p.D52N) - CPS1_000249 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 3 c.172_178del r.(?) p.(Asp58Leufs*3) - pathogenic g.211438049_211438055del g.210573325_210573331del 154_160delGATGGAA (D52LfsX3) - CPS1_000081 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 3 c.191G>A r.(?) p.(Gly64Asp) - pathogenic g.211438068G>A g.210573344G>A 173G>A (G58D) - CPS1_000082 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 3 c.212C>T r.(?) p.(Ser71Phe) - pathogenic g.211438089C>T g.210573365C>T 194C>T (S65F) - CPS1_000083 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 3 c.230T>G r.(?) p.(Val77Gly) - pathogenic g.211438107T>G g.210573383T>G 212T>G (V71G) - CPS1_000084 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 3 c.254G>A r.(spl?) p.(Gly85Glu?) - pathogenic g.211438131G>A g.210573407G>A 236G>A (Gly79Glu) - CPS1_000038 last nucleotide exon, donor splice site possibly affected PubMed: Kurokawa 2007 - - Germline - - - - - Johan den Dunnen
+/. 3i c.254+6T>C r.(spl?) p.(?) - pathogenic g.211438137T>C g.210573413T>C IVS2+6T>C - CPS1_000046 - PubMed: Kurokawa 2007 - - Germline - - - - - Johan den Dunnen
+?/. - c.274G>A r.(?) p.(Asp92Asn) - likely pathogenic g.211441089G>A g.210576365G>A CPS1(NM_001875.5):c.256G>A (p.D86N) - CPS1_000270 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. 4 c.277C>T r.(?) p.(Pro93Ser) - pathogenic g.211441092C>T g.210576368C>T 259C>T (P87S) - CPS1_000062 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 4 c.277C>T r.(?) p.(Arg809Cys) - pathogenic g.211441092C>T g.210576368C>T 259C>T (R803C) - CPS1_000062 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 4 c.277C>T r.(?) p.(Pro93Ser) - pathogenic g.211441092C>T g.210576368C>T 259C>T (P87S) - CPS1_000062 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 4 c.283T>G r.(?) p.(Tyr95Asp) - pathogenic g.211441098T>G g.210576374T>G 265T>G (Y89D) - CPS1_000085 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 4 c.285C>G r.(?) p.(Tyr95*) - pathogenic g.211441100C>G g.210576376C>G 267C>G (Y89X) - CPS1_000086 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
?/. - c.313C>A r.(?) p.(Pro105Thr) - VUS g.211441128C>A g.210576404C>A CPS1(NM_001875.5):c.295C>A (p.P99T) - CPS1_000250 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. 4 c.324_329dup r.(?) p.(Asn109_Gly110dup) - pathogenic g.211441139_211441144dup g.210576415_210576420dup 306_311dupGAATGG (N103_G104insGN) - CPS1_000087 - PubMed: Summar 1998, PubMed: Eeds 2006 - - Germline - - - - - Johan den Dunnen
?/. - c.335C>T r.(?) p.(Ala112Val) - VUS g.211441150C>T - CPS1(NM_001875.5):c.317C>T (p.(Ala106Val)) - CPS1_000323 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. - c.352C>T r.(?) p.(Leu118=) - likely benign g.211441167C>T g.210576443C>T CPS1(NM_001875.4):c.334C>T (p.L112=) - CPS1_000294 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 4 c.386C>T r.(?) p.(Ser129Phe) - pathogenic g.211441201C>T g.210576477C>T 368C>T (S123F) - CPS1_000061 - PubMed: Summar 1998 - - Germline - - - - - Johan den Dunnen
+/. 4 c.386C>T r.(?) p.(Ser129Phe) - NA g.211441201C>T g.210576477C>T 368C>T (S123F) - CPS1_000061 CPSI expression studies show 60% decreased activity PubMed: Pekkala 2010 - - In vitro (cloned) - - - - - Johan den Dunnen
+/. 5 c.424_427dup r.(?) p.(Ser143Ilefs*2) - pathogenic g.211442169_211442172dup g.210577445_210577448dup 409_410insTATA (S137IfsX2) - CPS1_000088 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 5 c.438C>A r.(?) p.(Tyr146*) - pathogenic g.211442183C>A g.210577459C>A 420C>A (Y140X) - CPS1_000089 - PubMed: Eeds 2006 - - Germline - - - - - Johan den Dunnen
-/. 5 c.467G>A r.(?) p.(Gly156Glu) - benign g.211442212G>A g.210577488G>A 449G>A (Gly150Glu) - CPS1_000001 - PubMed: Mitchell 2009 - - Germline - 4/94 - - - Johan den Dunnen
-/. - c.467G>A r.(?) p.(Gly156Glu) - benign g.211442212G>A g.210577488G>A CPS1(NM_001875.4):c.449G>A (p.G150E) - CPS1_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 5i c.489+1G>A r.400_489del p.Val134_Lys163del - pathogenic g.211442235G>A g.210577511G>A 471+1G>A (128V_157Kdel30) - CPS1_000238 affects donor splice site PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
-/. - c.504T>C r.(?) p.(Tyr168=) - benign g.211444452T>C g.210579728T>C CPS1(NM_001875.4):c.486T>C (p.Y162=), CPS1(NM_001875.5):c.486T>C (p.Y162=) - CPS1_000271 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-?/. - c.504T>C r.(?) p.(Tyr168=) - likely benign g.211444452T>C g.210579728T>C CPS1(NM_001875.4):c.486T>C (p.Y162=), CPS1(NM_001875.5):c.486T>C (p.Y162=) - CPS1_000271 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. - c.505G>A r.(?) p.(Gly169Arg) - pathogenic g.211444453G>A g.210579729G>A - - CPS1_000293 1 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs200214298 Germline - 1/2795 individuals - - - Mohammed Faruq
+/. 6 c.512A>G r.(?) p.(Asp171Gly) - pathogenic g.211444460A>G g.210579736A>G 494A>G (D165G) - CPS1_000090 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 6 c.545_546dup r.(?) p.(Gly183Argfs*25) - pathogenic g.211444493_211444494dup g.210579769_210579770dup 527_528insGA (G177RfsX25) - CPS1_000091 last nucleotide exon, donor splice site possibly affected PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
-?/. - c.547-4A>T r.spl? p.? - likely benign g.211447337A>T g.210582613A>T CPS1(NM_001875.4):c.529-4A>T - CPS1_000272 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 6i c.547-3T>G r.(spl?) p.? - pathogenic g.211447338T>G g.210582614T>G - - CPS1_000227 creates acceptor splice site PubMed: Eeds 2006 - - Germline - - - - - Johan den Dunnen
+/. 7i_8i c.(639+1_730-1)? r.640_729del p.Asp214_Lys242del - pathogenic g.(211447434_211454829)? - 622_711del (D208_K236del30) - CPS1_000000 unknown variant genomic DNA PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 7i c.640-7A>G r.621_622ins622-6_622-1 p.Lys213_Asp214insTrpGln - pathogenic g.211452775A>G g.210588051A>G 622-7A>G (K207_D208insWQ) - CPS1_000242 affects acceptor splice site PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 8 c.652T>A r.(?) p.(Tyr218Asn) - pathogenic g.211452794T>A g.210588070T>A 634T>A (Tyr212Asn) - CPS1_000059 - PubMed: Kurokawa 2007 - - Germline - - - - - Johan den Dunnen
?/. - c.655G>A r.(?) p.(Gly219Ser) - VUS g.211452797G>A - CPS1(NM_001875.5):c.637G>A (p.(Gly213Ser)) - CPS1_000317 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 8 c.689A>T r.(?) p.(Asp230Val) - pathogenic g.211452831A>T g.210588107A>T 671A>T (D224V) - CPS1_000092 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 8 c.715C>T r.(?) p.(Arg239Cys) - pathogenic g.211452857C>T g.210588133C>T 697C>T (R233C) - CPS1_000093 CpG dinucleotide PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 8 c.718del r.(?) p.(Leu240Cysfs*2) - pathogenic g.211452860del g.210588136del 700delC (L234CfsX2) - CPS1_000094 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 8i c.729+1G>A r.640_729del p.Asp214_Lys242del - pathogenic g.211452872G>A g.210588148G>A 711+1G>A (D208_K236del30) - CPS1_000243 affects donor splice site PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 8i_12i c.729+686_1182+136del r.730_1104del p.Arg244_Glu368del - pathogenic g.211453557_211457816del g.210588833_210593092del g.117646_113441del (Arg238_Glu362del)) - CPS1_000000 4.2-kb deletion exons 9-11, discrepancy description genomic DNA (ex8-11del) and cDNA (ex8-10del); undetectable hepatic enzyme activity PubMed: Aoshima 2001, OMIM:var0005 - - Germline - - - - - Johan den Dunnen
-/. - c.730-16T>C r.(=) p.(=) - benign g.211454814T>C g.210590090T>C CPS1(NM_001875.5):c.712-16T>C - CPS1_000251 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 9 c.730C>T r.(spl?) p.(Arg244*) - pathogenic g.211454830C>T g.210590106C>T 712C>T (R238X) - CPS1_000225 CpG dinucleotide; first nucleotide exon 9, effect on splicing unlikely PubMed: Häberle 2003 - - Germline - - - - - Johan den Dunnen
?/. - c.731G>A r.(?) p.(Arg244Gln) - VUS g.211454831G>A - CPS1(NM_001875.4):c.713G>A (p.R238Q), CPS1(NM_001875.5):c.713G>A (p.(Arg238Gln)) - CPS1_000310 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. - c.731G>A r.(?) p.(Arg244Gln) - VUS g.211454831G>A - CPS1(NM_001875.4):c.713G>A (p.R238Q), CPS1(NM_001875.5):c.713G>A (p.(Arg238Gln)) - CPS1_000310 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 9 c.746A>C r.(?) p.(His249Pro) - pathogenic g.211454846A>C g.210590122A>C 728A>C (H243P) - CPS1_000095 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 9 c.749del r.(?) p.(Leu250*) - pathogenic g.211454849del g.210590125del 731delT (L244X) - CPS1_000096 - PubMed: Eeds 2006 - - Germline - - - - - Johan den Dunnen
+/. 9 c.749del r.(?) p.(Leu250*) - pathogenic g.211454849del g.210590125del 731delT (L244X) - CPS1_000096 - PubMed: Eeds 2006 - - Germline - - - - - Johan den Dunnen
+/. 9 c.791G>A r.(?) p.(Gly264Glu) - pathogenic g.211454891G>A g.210590167G>A 773G>A (G258E) - CPS1_000097 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
-/. 9 c.800T>G r.(?) p.(Ile267Ser ) - benign g.211454900T>G g.210590176T>G 782T>G - CPS1_000003 - PubMed: Mitchell 2009 - - Germline - 2/94 - - - Johan den Dunnen
-?/. - c.801C>T r.(?) p.(Ile267=) - likely benign g.211454901C>T - CPS1(NM_001875.4):c.783C>T (p.I261=) - CPS1_000307 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 9 c.806G>A r.(?) p.(Gly269Glu) - pathogenic g.211454906G>A g.210590182G>A 788G>A (G263E) - CPS1_000098 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 9 c.858G>C r.(spl?) p.(Lys286Asn?) - pathogenic g.211454958G>C g.210590234G>C 840G>C (Lys280Asn) - CPS1_000022 last nucleotide exon, donor splice site possibly affected; undetectable liver CPS activity; unknown pathogenic variant in 2nd chromosome PubMed: Kurokawa 2007 - - Germline - - - - - Johan den Dunnen
+/. 9 c.858G>C r.850_858del p.Val284_Lys286del - pathogenic g.211454958G>C g.210590234G>C 840G>C - CPS1_000022 marked decrease CPS1 mRNA, 11% hepatic enzyme activity; sporadic, consanguineous parents PubMed: Hoshide 1993, OMIM:var0001 - - Germline - - - - - Johan den Dunnen
+/. 9 c.858G>C r.850_858del p.Val284_Lys286del - pathogenic g.211454958G>C g.210590234G>C 840G>C - CPS1_000022 marked decrease CPS1 mRNA, 11% hepatic enzyme activity; sporadic, consanguineous parents PubMed: Hoshide 1993, OMIM:var0001 - - Germline - - - - - Johan den Dunnen
+/. 9 c.858G>C r.(spl?) p.(Lys286Asn?) - pathogenic g.211454958G>C g.210590234G>C 840G>C (Lys280Asn) - CPS1_000022 last nucleotide exon, donor splice site possibly affected; 11% CPS activity PubMed: Kurokawa 2007 - - Germline - - - - - Johan den Dunnen
+/. 9 c.858G>C r.(spl?) p.(Lys286Asn?) - pathogenic g.211454958G>C g.210590234G>C 840G>C (Lys280Asn) - CPS1_000022 last nucleotide exon, donor splice site possibly affected; 11% CPS activity PubMed: Kurokawa 2007 - - Germline - - - - - Johan den Dunnen
+/. 9 c.858G>C r.(spl?) p.(Lys286Asn?) - pathogenic g.211454958G>C g.210590234G>C 840G>C (Lys280Asn) - CPS1_000022 last nucleotide exon, donor splice site possibly affected; 6% CPS activity PubMed: Kurokawa 2007 - - Germline - - - - - Johan den Dunnen
+/. 9 c.858G>C r.850_858del p.Val284_Lys286del - pathogenic g.211454958G>C g.210590234G>C 840G>C (V278_K280delVRK) - CPS1_000022 - PubMed: Hoshide 1993 - - Germline - - - - - Johan den Dunnen
+/. 10 c.866del r.(?) p.(Glu289Glyfs*16) - pathogenic g.211455531del g.210590807del 848delA (E283GfsX16) - CPS1_000099 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 10 c.878del r.(?) p.(Lys293ArgfsTer12) - pathogenic g.211455543del g.210590819del 860delA (fsX) - CPS1_000060 undetectable liver CPS activity PubMed: Kurokawa 2007 - - Germline - - - - - Johan den Dunnen
+/. 10 c.884del r.(?) p.(Pro295Hisfs*10) - pathogenic g.211455549del g.210590825del 866delC (P289HfsX10) - CPS1_000100 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 10 c.918_919dup r.(?) p.(Gly307Glufs*24) - pathogenic g.211455583_211455584dup g.210590859_210590860dup 900_901dupAG (G301EfsX24) - CPS1_000101 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 10 c.920G>A r.(?) p.(Gly307Glu) - pathogenic g.211455585G>A g.210590861G>A 902G>A (G301E) - CPS1_000102 - PubMed: Eeds 2006 - - Germline - - - - - Johan den Dunnen
+?/. 10 c.929C>T r.(?) p.(Ala310Val) - likely pathogenic g.211455594C>T g.210590870C>T 911C>T (A304V) - CPS1_000063 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
-?/. - c.966-6C>T r.(=) p.(=) - likely benign g.211456549C>T - CPS1(NM_001875.4):c.948-6C>T - CPS1_000305 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 11 c.968G>A r.(?) p.(Gly323Glu) - pathogenic g.211456557G>A g.210591833G>A 950G>A (G317E) - CPS1_000105 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+?/. 11 c.1010T>C r.(?) p.(Ile992Thr) - likely pathogenic g.211456599T>C g.210591875T>C 992T>C (I986T) - CPS1_000064 - PubMed: Häberle 2011 - - Germline - - - - - Johan den Dunnen
+/. 11 c.1028A>G r.(?) p.(His343Arg) - pathogenic g.211456617A>G g.210591893A>G 1010A>G (His337Arg) - CPS1_000026 undetectable liver CPS activity PubMed: Aoshima 2001, OMIM:var0004 - - Germline - - - - - Johan den Dunnen
+/. 11 c.1028A>G r.(?) p.(His343Arg) - NA g.211456617A>G g.210591893A>G 1010A>G His337Arg) - CPS1_000026 CPSI expression studies show 70% decreased activity PubMed: Pekkala 2010 - - In vitro (cloned) - - - - - Johan den Dunnen
-/. 11 c.1048A>G r.(?) p.(=) - benign g.211456637A>G g.210591913A>G 1030A>G - CPS1_000005 - PubMed: Mitchell 2009 - - Germline - 0.309 - - - Johan den Dunnen
-/. - c.1048A>G r.(?) p.(Thr350Ala) - benign g.211456637A>G g.210591913A>G CPS1(NM_001122633.2):c.1048A>G (p.T350A), CPS1(NM_001875.5):c.1030A>G (p.T344A) - CPS1_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. - c.1048A>G r.(?) p.(Thr350Ala) - benign g.211456637A>G g.210591913A>G CPS1(NM_001122633.2):c.1048A>G (p.T350A), CPS1(NM_001875.5):c.1030A>G (p.T344A) - CPS1_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-/. - c.1048A>G r.(?) p.(Thr350Ala) - benign g.211456637A>G g.210591913A>G CPS1(NM_001122633.2):c.1048A>G (p.T350A), CPS1(NM_001875.5):c.1030A>G (p.T344A) - CPS1_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. - c.1048A>G r.(?) p.(Thr350Ala) - benign g.211456637A>G g.210591913A>G CPS1(NM_001122633.2):c.1048A>G (p.T350A), CPS1(NM_001875.5):c.1030A>G (p.T344A) - CPS1_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. - c.1048A>T r.(?) p.(Thr350Ser) - benign g.211456637A>T g.210591913A>T CPS1(NM_001875.5):c.1030A>T (p.T344S) - CPS1_000273 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. 11 c.1050C>T r.(?) p.(Thr350Ala) - benign g.211456639C>T g.210591915C>T 1032C>T (Thr344Ala ) - CPS1_000004 - PubMed: Mitchell 2009 - - Germline - - - - - Johan den Dunnen
-/. - c.1050C>T r.(?) p.(Thr350=) - benign g.211456639C>T g.210591915C>T CPS1(NM_001122633.2):c.1050C>T (p.T350=), CPS1(NM_001875.5):c.1032C>T (p.T344=) - CPS1_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. - c.1050C>T r.(?) p.(Thr350=) - benign g.211456639C>T g.210591915C>T CPS1(NM_001122633.2):c.1050C>T (p.T350=), CPS1(NM_001875.5):c.1032C>T (p.T344=) - CPS1_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-/. - c.1050C>T r.(?) p.(Thr350=) - benign g.211456639C>T g.210591915C>T CPS1(NM_001122633.2):c.1050C>T (p.T350=), CPS1(NM_001875.5):c.1032C>T (p.T344=) - CPS1_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. - c.1050C>T r.(?) p.(Thr350=) - benign g.211456639C>T g.210591915C>T CPS1(NM_001122633.2):c.1050C>T (p.T350=), CPS1(NM_001875.5):c.1032C>T (p.T344=) - CPS1_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. 11 c.1057G>T r.(?) p.(Ala353Ser) - benign g.211456646G>T g.210591922G>T 1039G>T (Ala347Ser ) - CPS1_000006 - PubMed: Mitchell 2009 - - Germline - 2/94 - - - Johan den Dunnen
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