All variants in the GTPBP3 gene

Information The variants shown are described using the NM_032620.3 transcript reference sequence.

54 entries on 1 page. Showing entries 1 - 54.
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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.-4590C>T r.(?) p.(=) - likely benign g.17443831C>T g.17333022C>T ANO8(NM_020959.2):c.494G>A (p.(Arg165His)) - ANO8_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. - c.-4581G>A r.(?) p.(=) - likely benign g.17443840G>A - ANO8(NM_020959.3):c.490-5C>T - ANO8_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.-2588del r.(?) p.(=) - VUS g.17445833del g.17335024del GTPBP3(NM_001195422.1):c.21delT (p.H9Tfs*29) - ANO8_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. - c.8G>T r.(?) p.(Arg3Leu) - VUS g.17448428G>T g.17337619G>T - - GTPBP3_000011 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.8G>T - p.Arg3Leu - NA g.17448428G>T g.17337619G>T - - GTPBP3_000011 functional analysis by expression cloning HEK293 cells; blocked hGTPBP3 import into mitochondria, reduced stability, no yeast complementation, reduced hMTO1 interaction PubMed: Peng 2021 - - In vitro (cloned) - - - - - Johan den Dunnen
+/. - c.32_33delinsGTG r.(?) p.(Gln11ArgfsTer98) - pathogenic (recessive) g.17448452_17448453delinsGTG g.17337643_17337644delinsGTG - - GTPBP3_000012 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.32_33delinsGTG r.(?) p.(Gln11ArgfsTer98) - pathogenic (recessive) g.17448452_17448453delinsGTG g.17337643_17337644delinsGTG - - GTPBP3_000012 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
?/. - c.54-3C>T r.spl? p.? - VUS g.17448814C>T g.17338005C>T GTPBP3(NM_133644.3):c.54-3C>T - ANO8_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. - c.198A>C r.(?) p.(Thr66=) - benign g.17448961A>C - GTPBP3(NM_133644.4):c.198A>C (p.T66=) - ANO8_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. - c.253C>T r.(?) p.(Pro85Ser) - pathogenic g.17449016C>T g.17338207C>T - - GTPBP3_000003 - - - - Germline - - - - - Sha Hong
-?/. - c.256C>T r.(?) p.(Arg86Cys) - likely benign g.17449019C>T g.17338210C>T GTPBP3(NM_001128855.2):c.256C>T (p.(Arg86Cys)) - ANO8_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.410C>G r.(?) p.(Pro137Arg) - VUS g.17449369C>G - GTPBP3(NM_032620.4):c.410C>G (p.(Pro137Arg)) - ANO8_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. - c.413C>T r.(?) p.(Ala138Val) - likely pathogenic g.17449372C>T g.17338563C>T NM_001195422.1:c.479C>T (Ala160Val) - GTPBP3_000004 - - - - Germline - - - - - Sha Hong
+?/. - c.413C>T r.(?) p.(Ala138Val) ACMG likely pathogenic (recessive) g.17449372C>T g.17338563C>T - - GTPBP3_000004 ACMG PM2, PM3, PP3, PP4 PubMed: Yan 2021 - - Germline - - - - - Johan den Dunnen
?/. - c.424G>A r.(?) p.(Glu142Lys) ACMG VUS g.17449383G>A g.17338574G>A - - GTPBP3_000008 ACMG PM2, PP3 PubMed: Yan 2021 - - Germline - - - - - Johan den Dunnen
+/. - c.424G>A r.(?) p.(Glu142Lys) - pathogenic (recessive) g.17449383G>A g.17338574G>A - - GTPBP3_000008 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.424G>A - p.Glu142Lys - NA g.17449383G>A g.17338574G>A - - GTPBP3_000008 functional analysis by expression cloning HEK293 cells; reduced stability, no yeast complementation, normal hMTO1 interaction PubMed: Peng 2021 - - In vitro (cloned) - - - - - Johan den Dunnen
+?/. - c.440C>T r.(?) p.(Ala147Val) - likely pathogenic g.17449399C>T - GTPBP3(NM_133644.4):c.440C>T (p.A147V) - ANO8_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/. - c.476A>T r.(?) p.(Glu159Val) - pathogenic (recessive) g.17449435A>T g.17338626A>T - - GTPBP3_000013 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.476A>T - p.Glu159Val - NA g.17449435A>T g.17338626A>T - - GTPBP3_000013 functional analysis by expression cloning HEK293 cells; 0.22 GTPase activityreduced stability, no yeast complementation, normal hMTO1 interaction PubMed: Peng 2021 - - In vitro (cloned) - - - - - Johan den Dunnen
-?/. - c.484G>A r.(?) p.(Ala162Thr) - likely benign g.17449443G>A g.17338634G>A GTPBP3(NM_001128855.2):c.484G>A (p.(Ala162Thr)) - ANO8_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. - c.484G>C r.(?) p.(Ala162Pro) - pathogenic (recessive) g.17449443G>C g.17338634G>C - - GTPBP3_000014 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.484G>C - p.Ala162Pro - NA g.17449443G>C g.17338634G>C - - GTPBP3_000014 functional analysis by expression cloning HEK293 cells; normal stability, 0.09 GTPase activityreduced stability, no yeast complementation, reduced hMTO1 interaction PubMed: Peng 2021 - - In vitro (cloned) - - - - - Johan den Dunnen
+/. - c.509_510del r.(?) p.(Glu170Glyfs*42) ACMG likely pathogenic (recessive) g.17449468_17449469del g.17338659_17338660del - - GTPBP3_000005 ACMG PVS1, PM2 PubMed: Yan 2021 - - Germline - - - - - Johan den Dunnen
+?/. - c.544G>T r.(?) p.(Gly182*) ACMG likely pathogenic (recessive) g.17449503G>T g.17338694G>T - - GTPBP3_000006 ACMG PVS1, PM2 PubMed: Yan 2021 - - Germline - - - - - Johan den Dunnen
+?/. - c.545G>A r.(?) p.(Gly182Glu) ACMG likely pathogenic (recessive) g.17449504G>A g.17338695G>A - - GTPBP3_000010 - PubMed: Wang 2022 - - Germline/De novo (untested) - - - - - Johan den Dunnen
?/. - c.551T>G r.(?) p.(Leu184Arg) - VUS g.17449510T>G g.17338701T>G GTPBP3(NM_133644.3):c.551T>G (p.L184R) - GTPBP3_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. - c.633_647del r.(?) p.(Asp211_Glu215del) - VUS g.17449804_17449818del - GTPBP3(NM_032620.4):c.633_647del (p.(Asp211_Glu215del)) - ANO8_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. - c.665-2del r.665_808del p.Ala222_Ser270delinsGly - pathogenic (recessive) g.17449930del g.17339121del 665-2delA [Ala222Gly; Asp223_Ser270del] - GTPBP3_000015 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
?/. - c.666C>A r.(?) p.(=) - VUS g.17449933C>A - GTPBP3(NM_032620.4):c.666C>A (p.(Ala222=)) - ANO8_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.673G>A r.(?) p.(Glu225Lys) - VUS g.17449940G>A g.17339131G>A [673G>A;964G>C] - GTPBP3_000016 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
?/. - c.673G>A r.(?) p.(Glu225Lys) - VUS g.17449940G>A g.17339131G>A [673G>A;964G>C] - GTPBP3_000016 unknown variant 2nd chromosome PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.[673G>A;964G>C] - p.[Glu225Lys;Ala322Pro] - NA g.[17449940G>A;17450398G>C] g.[17339131G>A;17339589G>C] - - GTPBP3_000017 functional analysis by expression cloning HEK293 cells; normal stability, 0.13 GTPase activityreduced stability, no yeast complementation, normal hMTO1 interaction PubMed: Peng 2021 - - In vitro (cloned) - - - - - Johan den Dunnen
?/. - c.689A>C r.(?) p.(Gln230Pro) ACMG VUS g.17449956A>C g.17339147A>C NM_133644.3:c.785A>C (p.Q262P) - GTPBP3_000007 ACMG PM2, BP4 PubMed: Yan 2021 - - Germline - - - - - Johan den Dunnen
?/. - c.689A>C r.(?) p.(Gln230Pro) ACMG VUS g.17449956A>C g.17339147A>C NM_133644.3:c.785A>C (Q262P) - GTPBP3_000007 ACMG PM2, BP4 PubMed: Yan 2021 - - Germline - - - - - Johan den Dunnen
-/. - c.749T>C r.(?) p.(Val250Ala) - benign g.17450016T>C - GTPBP3(NM_133644.4):c.845T>C (p.V282A) - ANO8_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. - c.770C>A r.(?) p.(Pro257His) - pathogenic (recessive) g.17450037C>A g.17339228C>A - - GTPBP3_000018 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.770C>A - p.Pro257His - NA g.17450037C>A g.17339228C>A - - GTPBP3_000018 functional analysis by expression cloning HEK293 cells; reduced stability, 0.03 GTPase activityreduced stability, no yeast complementation, normal hMTO1 interaction PubMed: Peng 2021 - - In vitro (cloned) - - - - - Johan den Dunnen
+/. - c.836C>T r.(?) p.(Pro279Leu) - pathogenic (recessive) g.17450270C>T g.17339461C>T NM_133644.3:c.932C>T (Pro311Leu) - GTPBP3_000024 - PubMed: Elmas 2019 - - Germline - - - - - Johan den Dunnen
+/. - c.934_957del r.(?) p.(Gly312_Val319del) - pathogenic (recessive) g.17450368_17450391del g.17339559_17339582del - - GTPBP3_000019 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.934_957del - p.Gly312_Val319del - NA g.17450368_17450391del g.17339559_17339582del - - GTPBP3_000019 functional analysis by expression cloning HEK293 cells; normal stability, 0.06 GTPase activityreduced stability, no yeast complementation, reduced hMTO1 interaction PubMed: Peng 2021 - - In vitro (cloned) - - - - - Johan den Dunnen
+/. - c.964G>C r.(?) p.(Ala322Pro) - pathogenic (recessive) g.17450398G>C g.17339589G>C 964G>A [673G>A;964G>C] - GTPBP3_000020 unknown variant 2nd chromosome PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.964G>C r.(?) p.(Ala322Pro) - pathogenic (recessive) g.17450398G>C g.17339589G>C - - GTPBP3_000020 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.964G>C r.(?) p.(Ala322Pro) - pathogenic (recessive) g.17450398G>C g.17339589G>C [673G>A;964G>C] - GTPBP3_000020 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.964G>C - p.Ala322Pro - NA g.17450398G>C g.17339589G>C - - GTPBP3_000020 functional analysis by expression cloning HEK293 cells; normal stability, 0.03 GTPase activityreduced stability, no yeast complementation, normal hMTO1 interaction PubMed: Peng 2021 - - In vitro (cloned) - - - - - Johan den Dunnen
+/. - c.1009G>C r.(?) p.(Asp337His) - pathogenic (recessive) g.17451887G>C g.17341078G>C - - GTPBP3_000021 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.1009G>C - p.Asp337His - NA g.17451887G>C g.17341078G>C - - GTPBP3_000021 functional analysis by expression cloning HEK293 cells; reduced stability, yeast complementation, normal hMTO1 interaction PubMed: Peng 2021 - - In vitro (cloned) - - - - - Johan den Dunnen
+/. - c.1121del r.(?) p.(Asn374ThrfsTer26) - pathogenic g.17451999del g.17341190del GTPBP3(NM_133644.3):c.1217delA (p.N406Tfs*26) - GTPBP3_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. - c.1193G>A r.(?) p.(Cys398Tyr) ACMG likely pathogenic (recessive) g.17452071G>A g.17341262G>A NM_133644.3:c.1289G>A (Cys430Tyr) - GTPBP3_000009 - PubMed: Wang 2022 - - Germline/De novo (untested) - - - - - Johan den Dunnen
+/. - c.1291dup r.(?) p.(Arg431ProfsTer87) - pathogenic (recessive) g.17452324dup g.17341515dup 1291dupC - GTPBP3_000022 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.1291dup r.(?) p.(Arg431ProfsTer87) - pathogenic (recessive) g.17452324dup g.17341515dup 1291dupC - GTPBP3_000022 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.1375G>A r.(?) p.(Glu459Lys) - pathogenic (recessive) g.17452408G>A g.17341599G>A 1291dupC - GTPBP3_000023 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.1375G>A r.(?) p.(Glu459Lys) - pathogenic (recessive) g.17452408G>A g.17341599G>A 1291dupC - GTPBP3_000023 - PubMed: Kopajtich 2014 - - Germline - - - - - Johan den Dunnen
+/. - c.1375G>A - p.Glu459Lys - NA g.17452408G>A g.17341599G>A - - GTPBP3_000023 functional analysis by expression cloning HEK293 cells; normal stability, 0.90 GTPase activityreduced stability, no yeast complementation, normal hMTO1 interaction PubMed: Peng 2021 - - In vitro (cloned) - - - - - Johan den Dunnen
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