Unique variants in the G6PC gene

NOTE: gene name changed from G6PC to G6PC1
Information The variants shown are described using the NM_000151.3 transcript reference sequence.

52 entries on 1 page. Showing entries 1 - 52.
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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 _1_1i c.(?_-80)_(230+19_231-1)del r.0? p.0? - pathogenic g.(41000000_41050890)_(41053142_41055947)del - g.41050890_41053142del - G6PC_000013 - PubMed: Giugliano 2018 - - Germline - - - - - Teresa Giugliano
-?/. 1 - c.-77G>A r.(?) p.(=) - likely benign g.41052817G>A - G6PC1(NM_001270397.2):c.-77G>A - G6PC_000023 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
+/. 1 2i_5_ c.(340+1_341-1)_*3007{0} r.? p.? - pathogenic (recessive) g.(41056058_41059539)_(41066450_?)del - E3-E5 del - G6PC_000038 - PubMed: Wang 2013 - - Germline - - - - - LOVD
+/. 3 5 c.79del r.(?) p.(Gln27Argfs*9), p.(Gln27ArgfsTer9) - pathogenic, pathogenic (recessive) g.41052972del g.42900955del 158delC (35X), 79delC, G6PC1(NM_000151.4):c.79delC (p.Q27Rfs*9) - G6PC_000037 ACMG PSV1, PS4mod, PM2, VKGL data sharing initiative Nederland PubMed: Kessel 2021, PubMed: Lei 1995 VCV000021062.8 - CLASSIFICATION record, Germline - 4/140 case chromosomes - - - Johan den Dunnen, VKGL-NL_Groningen
./. 1 - c.96_97del r.(?) p.(Leu32Phefs*28) - likely pathogenic g.41052989_41052990del g.42900972_42900973del NM_000151.3(G6PC):c.96_97del p.(Leu32Phefs*28) - G6PC_000021 variant could not be associated with disease phenotype PubMed: Vogelaar 2017, Journal: Vogelaar 2017 - - Germline - - - - - Marjolijn JL Ligtenberg
+/. 1 - c.113A>T r.(?) p.(Asp38Val) - pathogenic g.41053006A>T - G6PC1(NM_000151.4):c.113A>T (p.D38V) - G6PC_000042 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. 1 - c.132C>T r.(?) p.(Tyr44=) - likely benign g.41053025C>T - G6PC(NM_001270397.1):c.132C>T (p.Y44=) - G6PC_000046 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.136C>T r.(?) p.(Leu46Phe) - likely benign g.41053029C>T g.42901012C>T G6PC(NM_000151.2):c.136C>T (p.L46F) - G6PC_000024 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
./. 1 - c.181C>T r.(?) p.(Leu61Phe) - likely pathogenic g.41053074C>T g.42901057C>T NM_000151.3(G6PC):c.181C>T p.(Leu61Phe) - G6PC_000022 variant could not be associated with disease phenotype PubMed: Vogelaar 2017, Journal: Vogelaar 2017 - - Germline - - - - - Marjolijn JL Ligtenberg
+/. 1 - c.188G>A r.(?) p.(Trp63*) - pathogenic (recessive) g.41053081G>A - G268A (W63X) - G6PC_000055 - PubMed: Lei 1995 - - Germline - 2/140 case chromosomes - - - Johan den Dunnen
+/. 2 - c.189G>A r.(?) p.(Trp63*) - pathogenic g.41053082G>A - G6PC(NM_000151.2):c.189G>A (p.W63*), G6PC1(NM_000151.4):c.189G>A (p.W63*) - G6PC_000040 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen, VKGL-NL_Utrecht
+/. 1 - c.209G>A r.(?) p.(Trp70Ter) - pathogenic g.41053102G>A g.42901085G>A - - G6PC_000035 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. 1 - c.231-4A>G r.spl? p.? - likely benign g.41055944A>G g.42903927A>G G6PC(NM_001270397.1):c.231-4A>G - G6PC_000025 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/., +/?, +?/., ?/., ?/? 23 2 c.247C>T r.(?) p.(Arg83Cys) ACMG likely pathogenic, pathogenic, pathogenic (recessive), VUS g.41055964C>T g.42903947C>T 362C>T, C326T, G6PC(NM_000151.2):c.247C>T (p.R83C), G6PC(NM_001270397.1):c.247C>T (p.R83C), 2 more items - G6PC_000001 1 heterozygous, no homozygous; Clinindb (India), combination alleles not determined, homozygous, 2 more items PubMed: Alfares 2018, PubMed: Lei 1993, OMIM:var0002, PubMed: Lei 1994, PubMed: Lei 1995, 4 more items - rs1801175 CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown ? 1/2795 individuals, 1/73755 controls, 52/140 case chromosomes, frequency in 1500 in-house samples: 0 - - - Johan den Dunnen, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, Shu Yau, Mohammed Faruq
+/. 4 - c.248G>A r.(?) p.(Arg83His) - pathogenic (recessive) g.41055965G>A g.42903948G>A G327A (R83H), R83H - G6PC_000056 ACMG PS3, PS4mod, PM2, PP3 PubMed: Kessel 2021, PubMed: Lei 1995 VCV000038300.7 - Germline - 8/140 case chromosomes - - - Johan den Dunnen
-/., -?/. 2 - c.340+10C>A r.(=) p.(=) - benign, likely benign g.41056067C>A g.42904050C>A G6PC(NM_000151.2):c.340+10C>A, G6PC(NM_001270397.1):c.340+10C>A - G6PC_000027 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Utrecht
+/., +/?, ?/? 9 3 c.379_380dup r.(?) p.(Tyr128Thrfs*3) ACMG pathogenic, pathogenic (recessive), VUS g.41059578_41059579dup g.42907561_42907562dup 130X (459insTA), 379_380dupTA, 459insAT, 459insTA (130X) - G6PC_000004 Peeks 2017:28397058, Wang 2013:22899091, Koeberl 2021:19541498 PubMed: Lei 1993, OMIM:var0001, PubMed: Lei 1993, OMIM:var0002, PubMed: Lei 1995, PubMed: Lei 1995, 1 more item ClinVar-11997 rs80356488 Germline, Unknown yes 9/140 case chromosomes - - - Johan den Dunnen, Miriam Erandi Reyna-Fabián
-?/. 1 - c.408C>T r.(?) p.(Ser136=) - likely benign g.41059607C>T - G6PC(NM_000151.2):c.408C>T (p.S136=) - G6PC_000062 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-/. 1 - c.432G>A r.(?) p.(Pro144=) - benign g.41059631G>A g.42907614G>A G6PC(NM_001270397.2):c.355G>A (p.D119N) - G6PC_000028 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+?/. 1 4 c.500G>A r.(?) p.(Cys167Tyr) ACMG likely pathogenic (recessive) g.41061373G>A g.42909356G>A - - G6PC_000065 1 more item - ClinVar-1682511 rs777552825 Germline yes - - - - Miriam Erandi Reyna-Fabián
+/. 2 - c.508C>T r.(?) p.(Arg170Ter) - pathogenic g.41061381C>T g.42909364C>T G6PC(NM_000151.2):c.508C>T (p.R170*), G6PC1(NM_000151.4):c.508C>T (p.R170*) - G6PC_000029 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_AMC
+/. 1 - c.509G>A r.(?) p.(Arg170Gln) - pathogenic (recessive) g.41061382G>A g.42909365G>A - - G6PC_000050 combination alleles not determined PubMed: Reiner 2022 - - Germline - 1/73755 controls - - - Johan den Dunnen
+/. 1 - c.532C>T r.(?) p.(Pro178Ser) - pathogenic (recessive) g.41061405C>T - C611T (P178S) - G6PC_000057 - PubMed: Lei 1995 - - Germline - 1/140 case chromosomes - - - Johan den Dunnen
+/. 2 4 c.533C>T r.(?) p.(Pro178Leu) ACMG pathogenic (recessive) g.41061406C>T g.42909389C>T - - G6PC_000039 1 more item PubMed: Wang 2013 ClinVar-1682514 rs768803329 Germline yes - - - - Miriam Erandi Reyna-Fabián
+/. 1 - c.562G>A r.(?) p.(Gly188Ser) - pathogenic (recessive) g.41061435G>A - G641A (G188S) - G6PC_000058 - PubMed: Lei 1995 - - Germline - 2/140 case chromosomes - - - Johan den Dunnen
+/. 2 - c.562G>C r.(?) p.(Gly188Arg) - pathogenic g.41061435G>C g.42909418G>C G6PC(NM_001270397.1):c.485G>C (p.R162T) - G6PC_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
-?/. 1 - c.563-20T>A r.(=) p.(=) - likely benign g.41062912T>A - G6PC(NM_000151.2):c.563-20T>A - G6PC_000063 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/. 1 - c.595C>T r.(?) p.(His199Tyr) - VUS g.41062964C>T g.42910947C>T G6PC(NM_001270397.1):c.518C>T (p.P173L) - G6PC_000030 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 2 - c.664G>C r.(?) p.(Gly222Arg) - pathogenic (recessive) g.41063033G>C g.42911016G>C G222R (G743C), G743C - G6PC_000061 - PubMed: Lei 1995, PubMed: Lei 1995 - - Germline - 1/140 case chromosomes - - - Johan den Dunnen
+/. 1 - c.706T>A r.(?) p.(Trp236Arg) - pathogenic (recessive) g.41063075T>A - T785A (W236R) - G6PC_000059 - PubMed: Lei 1995 - - Germline - 1/140 case chromosomes - - - Johan den Dunnen
+/. 1 - c.724C>T r.(?) p.(Gln242*) - pathogenic (recessive) g.41063093C>T - C803T (Q242X) - G6PC_000060 - PubMed: Lei 1995 - - Germline - 3/140 case chromosomes - - - Johan den Dunnen
+/. 1 5 c.735_742delinsGCGAGCAG r.(?) p.(Cys245_Pro248delinsTrpArgAlaAla) - pathogenic (recessive) g.41063104_41063111delinsGCGAGCAG g.42911087_42911094delinsGCGAGCAG 813insG-822delC (CEQP245-248WRAA), (734dup;743del) - G6PC_000052 - PubMed: Lei 1995 - - Germline - 1/140 case chromosomes - - - Johan den Dunnen
+/., +?/. 2 - c.788del r.(?) p.(Lys263ArgfsTer38) - likely pathogenic, pathogenic g.41063157del g.42911140del G6PC1(NM_000151.4):c.788delA (p.K263Rfs*38) - G6PC_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen, VKGL-NL_VUmc
+/. 3 5 c.809G>T r.(?) p.(Gly270Val) ACMG pathogenic, pathogenic (recessive) g.41063178G>T g.42911161G>T G6PC1(NM_000151.4):c.809G>T (p.G270V), G888T (G270V) - G6PC_000053 Peeks 2017:28397058, Allegrini 2017:28659124, Beegle 2015:25308557, 1 more item PubMed: Lei 1995 ClinVar-21063 rs80356483 CLASSIFICATION record, Germline yes 1/140 case chromosomes - - - Johan den Dunnen, VKGL-NL_VUmc, Miriam Erandi Reyna-Fabián
+/., +/?, ?/? 3 5 c.883C>T r.(?) p.(Arg295Cys) - pathogenic, pathogenic (recessive), VUS g.41063252C>T g.42911235C>T 962C>T, R295C - G6PC_000003 - PubMed: Lei 1993, OMIM:var0003, PubMed: Lei 1995 - rs104894563 Germline, Unknown - 2/140 case chromosomes - - - Johan den Dunnen
+?/. 1 - c.884G>A r.(?) p.(Arg295His) - likely pathogenic g.41063253G>A g.42911236G>A - - G6PC_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. 1 - c.909C>T r.(?) p.(Leu303=) - likely benign g.41063278C>T - G6PC(NM_000151.2):c.909C>T (p.L303=) - G6PC_000047 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. 2 - c.910G>A r.(?) p.(Val304Ile) - likely benign g.41063279G>A g.42911262G>A G6PC(NM_000151.2):c.910G>A (p.V304I) - G6PC_000036 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Utrecht
?/. 1 - c.944C>T r.(?) p.(Pro315Leu) - VUS g.41063313C>T - G6PC(NM_000151.2):c.944C>T (p.P315L) - G6PC_000051 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. 1 - c.948C>A r.(?) p.(Ser316=) - likely benign g.41063317C>A - G6PC(NM_000151.2):c.948C>A (p.S316=) - G6PC_000043 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.954C>T r.(?) p.(Val318=) - likely benign g.41063323C>T g.42911306C>T G6PC(NM_000151.2):c.954C>T (p.V318=) - G6PC_000031 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 4 - c.980_982del r.(?) p.(Phe327del) - pathogenic, pathogenic (recessive) g.41063349_41063351del g.42911332_42911334del 1057delTTC, delF327, 1 more item - G6PC_000044 VKGL data sharing initiative Nederland PubMed: Lei 1995, PubMed: Lei 1995 - - CLASSIFICATION record, Germline - 4/140 case chromosomes - - - Johan den Dunnen, VKGL-NL_Groningen, VKGL-NL_Utrecht
-?/. 1 - c.992C>T r.(?) p.(Ala331Val) - likely benign g.41063361C>T - G6PC(NM_000151.2):c.992C>T (p.A331V) - G6PC_000064 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. 1 - c.1002C>T r.(?) p.(Pro334=) - likely benign g.41063371C>T - G6PC(NM_000151.2):c.1002C>T (p.P334=) - G6PC_000041 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.1012G>T r.(?) p.(Val338Phe) - likely pathogenic g.41063381G>T g.42911364G>T G6PC1(NM_000151.4):c.1012G>T (p.V338F) - G6PC_000032 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. 1 - c.1022T>A r.(?) p.(Ile341Asn) - pathogenic g.41063391T>A g.42911374T>A - - G6PC_000033 1 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs387906505 Germline - 1/2791 individuals - - - Mohammed Faruq
+/. 1 5 c.1034_1035inv r.(?) p.(Leu345Arg) - pathogenic (recessive) g.41063403_41063404inv - T1113G-C1114A (L345R) - G6PC_000054 - PubMed: Lei 1995 - - Germline - 1/140 case chromosomes - - - Johan den Dunnen
+/., +/?, ?/? 13 5 c.1039C>T r.(?) p.(Gln347*) ACMG pathogenic, pathogenic (recessive), VUS g.41063408C>T g.42911391C>T C1118T, G6PC(NM_000151.2):c.1039C>T (p.Q347*), Q347stop, Q347X - G6PC_000002 VKGL data sharing initiative Nederland PubMed: Lei 1994, PubMed: Lei 1995, PubMed: Lei 1995 ClinVar-12000 rs80356487 CLASSIFICATION record, Germline, Unknown yes 31/140 case chromosomes - - - Johan den Dunnen, VKGL-NL_Rotterdam, VKGL-NL_Nijmegen, Shu Yau, Miriam Erandi Reyna-Fabián
-?/. 1 - c.1047G>T r.(?) p.(Leu349=) - likely benign g.41063416G>T - G6PC(NM_000151.2):c.1047G>T (p.L349=) - G6PC_000045 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.1068G>A r.(?) p.(Ser356=) - likely benign g.41063437G>A - G6PC(NM_000151.2):c.1068G>A (p.S356=) - G6PC_000048 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.*23T>C r.(=) p.(=) - likely benign g.41063466T>C - G6PC1(NM_000151.4):c.*23T>C - G6PC_000049 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
?/. 1 - c.*745G>T r.(=) p.(=) - VUS g.41064188G>T g.42912171G>T - - G6PC_000034 15 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs116897758 Germline - 15/2795 individuals - - - Mohammed Faruq
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