Unique variants in the MBTPS1 gene

Information The variants shown are described using the NM_003791.2 transcript reference sequence.

32 entries on 1 page. Showing entries 1 - 32.
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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.(?_-43941)_(*56332_?)del r.0 p.0 ACMG pathogenic (recessive) g.(?_84031722)_(84193956_?)del g.(?_83998117)_(84160351_?)del 84031722_84193956del - MBTPS1_000014 - PubMed: Lucas-Castro 2026, Journal: Lucas-Castro 2026 - - Germline - - - - - Elsa Lucas Castro
?/. 1 - c.129G>C r.(?) p.(Leu43Phe) - VUS g.84135260C>G - MBTPS1(NM_003791.4):c.129G>C (p.(Leu43Phe)) - MBTPS1_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 2 c.163G>A r.(?) p.(Glu55Lys) - VUS g.84135226C>T g.84101621C>T - - MBTPS1_000026 - PubMed: Chen 2023 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.285dup r.285dup p.Asp96Ter - pathogenic (recessive) g.84132794dup g.84099189dup 285dupT - MBTPS1_000020 - PubMed: Kondo 2018 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.569C>T r.(?) p.(Pro190Leu) - VUS g.84129263G>A - MBTPS1(NM_003791.4):c.569C>T (p.(Pro190Leu)) - MBTPS1_000019 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.736+7_736+9delinsTTT r.(=) p.(=) - likely benign g.84127307_84127309delinsAAA - MBTPS1(NM_003791.4):c.736+7_736+9delinsTTT - MBTPS1_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 - c.774C>T r.737_846del p.Leu247IlefsTer37 - likely pathogenic (recessive) g.84126865G>A g.84093260G>A - - MBTPS1_000024 variant predicted to affect ESE site; effect confirmed by mini-gene splicing assay PubMed: Yuan 2023 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.932C>T r.(?) p.(Pro311Leu) - VUS g.84125368G>A - MBTPS1(NM_003791.4):c.932C>T (p.(Pro311Leu)) - MBTPS1_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.1032-3T>C r.spl? p.? - likely benign g.84121068A>G - MBTPS1(NM_003791.4):c.1032-3T>C - MBTPS1_000017 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 - c.1064T>G r.(?) p.(Val355Gly) - likely pathogenic (recessive) g.84121033A>C g.84087428A>C - - MBTPS1_000027 - PubMed: Chen 2022 - - Germline - - - - - Johan den Dunnen
+/., +?/. 3 9 c.1094A>G r.(spl), r.1094_1134del, r.spl? p.(?), p.Asp365GlyfsTer12 - likely pathogenic (recessive), pathogenic (recessive) g.84121003T>C g.84087398T>C A1094G - MBTPS1_000001 variant created splice donor site PubMed: Kondo 2018, PubMed: Meyer 2020, PubMed: Raggio 2024 RCV000767393.1 rs1226321681 Germline - - - - - Johan den Dunnen
-?/. 1 - c.1134+8C>G r.(=) p.(=) - likely benign g.84120955G>C - MBTPS1(NM_003791.4):c.1134+8C>G - MBTPS1_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.1214G>C r.(?) p.(Gly405Ala) - likely benign g.84118660C>G - MBTPS1(NM_003791.3):c.1214G>C (p.G405A) - MBTPS1_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.1286+4A>C r.spl? p.? - VUS g.84118584T>G - MBTPS1(NM_003791.4):c.1286+4A>C - MBTPS1_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.1300C>T r.(?) p.(Arg434Cys) - VUS g.84115500G>A - MBTPS1(NM_003791.2):c.1300C>T (p.(Arg434Cys)) - MBTPS1_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 12 c.1589A>G r.(?) p.(Asp530Gly) - VUS g.84108206T>C g.84074601T>C - - MBTPS1_000025 - PubMed: Chen 2023 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.1679G>A r.(?) p.(Gly560Asp) - VUS g.84104296C>T - MBTPS1(NM_003791.4):c.1679G>A (p.(Gly560Asp)) - MBTPS1_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 15i c.2072-2A>T r.(2072_2228del) p.(Thr692SerfsTer15) - likely pathogenic (recessive) g.84101430T>A g.84067825T>A - - MBTPS1_000030 effect on splicing predicted from mini-gene splicing assay PubMed: Chen 2022 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.2276A>G r.(?) p.(Asn759Ser) - VUS g.84100171T>C - MBTPS1(NM_003791.4):c.2276A>G (p.N759S) - MBTPS1_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/. 1 17i c.2353+3A>G r.2229_2353del p.Arg743SerTer39 ACMG pathogenic (recessive) g.84100091T>C g.84066486T>C - - MBTPS1_000013 1 more item PubMed: Lucas-Castro 2026, Journal: Lucas-Castro 2026 - - Germline - - - - - Elsa Lucas Castro
+/. 1 - c.2355del r.(?) p.(Met785Ilefs*18) - pathogenic (recessive) g.84099371del g.84065766del 2355delG - MBTPS1_000031 - PubMed: Raggio 2024 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.2522T>C r.(?) p.(Ile841Thr) - VUS g.84096960A>G - MBTPS1(NM_003791.4):c.2522T>C (p.(Ile841Thr)) - MBTPS1_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.2628G>A r.(?) p.(Pro876=) - likely benign g.84094363C>T - MBTPS1(NM_003791.3):c.2628G>A (p.P876=) - MBTPS1_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.2634C>A r.(?) p.(Ser878Arg) - VUS g.84094357G>T g.84060752G>T - - MBTPS1_000022 - PubMed: Alotaibi 2022 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.2656C>T r.(?) p.(Gln886Ter) ACMG likely pathogenic (recessive) g.84094335G>A g.84060730G>A - - MBTPS1_000023 ACMG PVS1,  PM2 PubMed: Yuan 2023 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.2941G>A r.(?) p.(Gly981Ser) - likely benign g.84089631C>T - MBTPS1(NM_003791.2):c.2941G>A (p.(Gly981Ser)) - MBTPS1_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 22 c.2948G>A r.(?) p.(Trp983Ter) - pathogenic (recessive) g.84089624C>T g.84056019C>T - - MBTPS1_000021 - PubMed: Carvalho 2020 - - Germline - - - - - Johan den Dunnen
-/., -?/. 2 - c.2950G>A r.(?) p.(Asp984Asn) - benign, likely benign g.84089622C>T g.84056017C>T MBTPS1(NM_003791.2):c.2950G>A (p.(Asp984Asn)), MBTPS1(NM_003791.3):c.2950G>A (p.D984N) - MBTPS1_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
+?/. 1 - c.3007C>T r.3007C>T p.Pro1003Ser - VUS (!) g.84088206G>A g.84054601G>A - - MBTPS1_000032 variant reported as associated to the phenotype PubMed: Schweitzer 2019 - - De novo - - - - - Johan den Dunnen
?/. 1 - c.3029C>A r.(?) p.(Ala1010Asp) - VUS g.84088184G>T - MBTPS1(NM_003791.4):c.3029C>A (p.(Ala1010Asp)) - MBTPS1_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 - c.3157T>C r.(?) p.(Ter1053ArgextTer51) - likely pathogenic (recessive) g.84088056A>G g.84054451A>G (Ter1053Arg) - MBTPS1_000028 - PubMed: Chen 2022 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.3159A>T r.(?) p.(Ter1053CysextTer51) - likely pathogenic (recessive) g.84088054T>A g.84054449T>A (Ter1053Cys) - MBTPS1_000029 - PubMed: Chen 2022 - - Germline - - - - - Johan den Dunnen
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