Unique variants in the ZMPSTE24 gene

This database is one of the gene variant databases from the Leiden Muscular Dystrophy pages
Information The variants shown are described using the NM_005857.4 transcript reference sequence.

67 entries on 1 page. Showing entries 1 - 67.
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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 c.-1269C>G r.(=) p.(=) - benign g.40722675C>G g.40257003C>G - - ZMPSTE24_000026 - PubMed: Halaschek-Wiener 2009 - rs7548758 Germline - 9/94 chromosomes - - - Johan den Dunnen
-/. 1 _1 c.-1149G>A r.(=) p.(=) - benign g.40722795G>A g.40257123G>A - - ZMPSTE24_000025 - PubMed: Halaschek-Wiener 2009 - rs926830 Germline - 30/94 chromosomes - - - Johan den Dunnen
-/. 1 _1 c.-954G>C r.(=) p.(=) - benign g.40722990G>C g.40257318G>C - - ZMPSTE24_000035 - PubMed: Halaschek-Wiener 2009 - rs147867849 Germline - 1/94 chromosomes - - - Johan den Dunnen
-/. 1 _1 c.-522C>G r.(=) p.(=) - benign g.40723422C>G g.40257750C>G - - ZMPSTE24_000031 - PubMed: Halaschek-Wiener 2009 - rs11811211 Germline - 1/94 chromosomes - - - Johan den Dunnen
-?/. 1 _1 c.-370G>T r.(=) p.(=) - likely benign g.40723574G>T g.40257902G>T - - ZMPSTE24_000050 not in 100 control chromosomes PubMed: Gaudy-Marqueste 2009 - rs312262683 Germline - 1/54 chromosomes - - - Johan den Dunnen
-/. 3 _1 c.-326G>A r.(=) p.(=) - benign g.40723618G>A g.40257946G>A - - ZMPSTE24_000028 - PubMed: Gaudy-Marqueste 2009, PubMed: Halaschek-Wiener 2009 - rs3765483 Germline - 2/54 chromosomes, 20/100 controls, 9/94 chromosomes - - - Johan den Dunnen
-/. 1 1 c.-194A>G r.(=) p.(=) - benign g.40723750A>G g.40258078A>G - - ZMPSTE24_000027 - PubMed: Halaschek-Wiener 2009 - rs187549487 Germline - 1/94 chromosomes - - - Johan den Dunnen
-?/. 1 - c.8T>C r.(?) p.(Met3Thr) - likely benign g.40723951T>C - ZMPSTE24(NM_005857.4):c.8T>C (p.(Met3Thr)) - ZMPSTE24_000059 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 - c.33G>A r.(?) p.(Trp11*) - likely pathogenic g.40723976G>A - - - ZMPSTE24_000068 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 3 1 c.50del r.(?) p.(Lys17Serfs*21) - pathogenic g.40723993del g.40258321del - - ZMPSTE24_000021 - PubMed: Smigiel 20120, Smigiel ESHG2009 P02.101 - rs281875360 Germline yes - - - - Johan den Dunnen
+/. 3 1 c.54dup r.(?), r.54dup p.(Ile19Tyrfs*28), p.Ile19Tyrfs*28 - pathogenic g.40723997dup g.40258325dup - - ZMPSTE24_000007 not in 100 control chromosomes CNavarro, submitted, PubMed: Moulson 2006, PubMed: Miner 2010, OMIM:var0007 - rs281875361 Germline - - - - - Johan den Dunnen, Claire Navarro
+/. 3 1 c.121C>T r.(?) p.(Glu41*), p.Glu41* - pathogenic g.40724064C>T g.40258392C>T - - ZMPSTE24_000015 cloned in pRS416GPD, yeast STE24 complementation no activity, not in 200 control chromosomes PubMed: Miyoshi 2008, PubMed: Miyoshi 2008, OMIM:var0004 - rs121908094 Germline - - - - - Johan den Dunnen
-?/. 1 - c.123+8T>C r.(=) p.(=) - likely benign g.40724074T>C - ZMPSTE24(NM_005857.3):c.123+8T>C - ZMPSTE24_000064 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+?/. 1 1 c.124-2A>C r.spl? p.? - likely pathogenic g.40726509A>C g.40260837A>C - - ZMPSTE24_000010 - - - rs312262684 Germline - - - - - Tom Winder
-?/. 1 - c.179A>G r.(?) p.(Asp60Gly) - likely benign g.40726566A>G - ZMPSTE24(NM_005857.4):c.179A>G (p.(Asp60Gly)) - ZMPSTE24_000066 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 2 c.207_208del r.(?) p.(Tyr70Serfs*4) - pathogenic g.40726594_40726595del g.40260922_40260923del 207_208delCT - ZMPSTE24_000052 de novo, in patient PubMed: Cunningham 2010 - rs281875362 De novo yes - - - - Johan den Dunnen
+/. 2 2 c.209_210del r.(?) p.(Tyr70Serfs*4) - pathogenic g.40726596_40726597del g.40260924_40260925del 209_210delAT - ZMPSTE24_000038 - CNavarro, submitted - rs281875363 Germline - - - - - Claire Navarro
+/. 1 2i_5i c.271-1446_628-127del r.271_627del p.Leu91_Leu209del - pathogenic g.40732012_40737439del g.40266340_40271767del - - ZMPSTE24_000003 - PubMed: Navarro 2004, PubMed: Navarro 2005 - - Germline - - - - - Johan den Dunnen
+/. 3 3 c.281T>C r.(?) p.(Leu94Pro) - pathogenic g.40733468T>C g.40267796T>C - - ZMPSTE24_000054 not in 200 control chromosomes PubMed: BenYaou 2011 - rs281875364 Germline yes - BslI+ - - Johan den Dunnen
+/. 1 3 c.296del r.(?) p.(Pro99Leufs*38) - pathogenic g.40733483del g.40267811del - - ZMPSTE24_000005 - PubMed: Navarro 2005 - rs281875365 Germline - - - - - Johan den Dunnen
-/. 1 3i c.357+114A>G r.(=) p.(=) - benign g.40733658A>G g.40267986A>G - - ZMPSTE24_000029 - PubMed: Halaschek-Wiener 2009 - rs7516571 Germline - 30/94 chromosomes - - - Johan den Dunnen
+?/. 1 4 c.401del r.(?) p.(Ser134Metfs*3) - likely pathogenic g.40734134del g.40268462del - - ZMPSTE24_000009 - - - rs281875366 Germline - - - - - Tom Winder
-?/. 1 - c.453A>G r.(?) p.(Glu151=) - likely benign g.40734186A>G - ZMPSTE24(NM_005857.3):c.453A>G (p.E151=) - ZMPSTE24_000065 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/. 1 - c.474G>C r.(?) p.(Gln158His) - VUS g.40734207G>C - ZMPSTE24(NM_005857.3):c.474G>C (p.Q158H) - ZMPSTE24_000062 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-/., ?/. 4 4i c.474+83A>C r.(=) p.(=) - benign, VUS g.40734290A>C g.40268618A>C - - ZMPSTE24_000030 no interpretation available; 58 heterozygous, no homozygous; Clinindb (India) PubMed: Gaudy-Marqueste 2009, PubMed: Halaschek-Wiener 2009, PubMed: Narang 2020, Journal: Narang 2020 - rs75470795 Germline - 2/54 chromosomes, 3/100 controls, 3/94 chromosomes, 58/2779 individuals - - - Johan den Dunnen, Mohammed Faruq
+/. 1 4i c.475-2A>G r.spl p.(Val210Aspfs*21) - pathogenic g.40735645A>G g.40269973A>G - - ZMPSTE24_000043 - CNavarro, submitted - rs312262685 Germline - - - - - Claire Navarro
-?/. 1 - c.535T>C r.(?) p.(Leu179=) - likely benign g.40735707T>C - ZMPSTE24(NM_005857.3):c.535T>C (p.L179=) - ZMPSTE24_000063 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/. 3 5 c.591dup r.591dup p.Ile198Tyrfs*20 - pathogenic g.40735763dup g.40270091dup - - ZMPSTE24_000006 not in 100 control chromosomes PubMed: Moulson 2006, OMIM:var0008 - rs281875367 Germline - - - - - Johan den Dunnen
+/. 3 5 c.592_593del r.(?) p.(Ile198Leufs*19) - pathogenic g.40735764_40735765del g.40270092_40270093del 584_585delAT - ZMPSTE24_000020 - PubMed: Smigiel 20120, Smigiel ESHG2009 P02.101 - rs281875368 Germline yes - - - - Johan den Dunnen
+/. 4 5i c.627+1G>C r.475_627del p.Thr159_Leu209del - pathogenic g.40735800G>C g.40270128G>C - - ZMPSTE24_000016 not in 180 control chromosomes (Dutch); RNA blood PubMed: Sander 2008 - - Germline - - DdeI+ - - Johan den Dunnen
-/. 5 5i c.627+18T>G r.(=) p.(=) - benign g.40735817T>G g.40270145T>G - - ZMPSTE24_000032 - PubMed: Gaudy-Marqueste 2009, PubMed: Halaschek-Wiener 2009 - rs16827109 Germline - 1/100 controls, 14/100 controls, 2/54 chromosomes, 8/94 chromosomes - - - Johan den Dunnen
-/. 2 5i c.628-50T>C r.(=) p.(=) - benign g.40737516T>C g.40271844T>C - - ZMPSTE24_000051 - PubMed: Gaudy-Marqueste 2009 - rs6677717 Germline - 3/54 chromosomes, 4/100 controls - - - Johan den Dunnen
-/. 1 5i c.628-50T>G r.(=) p.(=) - benign g.40737516T>G g.40271844T>G - - ZMPSTE24_000033 - PubMed: Halaschek-Wiener 2009 - rs6677717 Germline - 3/94 chromosomes - - - Johan den Dunnen
+/. 1 5i c.628-2A>G r.(?) p.(Thr159_Leu209del) - pathogenic g.40737564A>G g.40271892A>G - - ZMPSTE24_000041 - CNavarro, submitted - rs312262687 Germline - - - - - Claire Navarro
-/. 7 6 c.651T>C r.(?) p.(=) - benign g.40737589T>C g.40271917T>C 615T>C (D217D), Asp217Asp - ZMPSTE24_000017 - PubMed: Gaudy-Marqueste 2009, PubMed: Halaschek-Wiener 2009, PubMed: Miyoshi 2008 - rs2076697 Germline - 1/100 controls, 22/100 controls, 5/54 chromosomes, 8/94 chromosomes - - - Johan den Dunnen
+/. 8 6 c.691G>T r.(?) p.(Glu231*) - pathogenic g.40737629G>T g.40271957G>T - - ZMPSTE24_000014 - PubMed: Jagadeesh 2009 - rs281875369 Germline - - - - - Parag Tamhankar
+/., +?/. 3 6 c.709G>T r.(?) p.(Glu237*) - likely pathogenic, pathogenic g.40737647G>T g.40271975G>T - - ZMPSTE24_000011 - PubMed: Saxena 2010 - rs281875370 Germline yes - MboII- - - Renu Saxena, Tom Winder
+/. 3 6 c.715G>T r.(?) p.(Glu239*) - pathogenic g.40737653G>T g.40271981G>T - - ZMPSTE24_000049 - PubMed: Chen 2009, PubMed: Chen 2009, OMIM:var0006 - rs267607181 Germline yes - - - - Johan den Dunnen
+/., ?/. 6 6 c.743C>T r.(?) p.(Pro248Leu), p.Pro248Leu - pathogenic, VUS g.40737681C>T g.40272009C>T - - ZMPSTE24_000012 cloned in pRS416GPD, yeast STE24 complementation normal activity, not in 200 control chromosomes PubMed: Ahmad 2010, PubMed: Ahmad 2010, OMIM:var0005, PubMed: Miyoshi 2008, 1 more item - rs121908095 Germline yes - - - - Johan den Dunnen, Tom Winder
-/. 1 6i c.769+4192C>T r.(=) p.(=) - benign g.40741899C>T g.40276227C>T - - ZMPSTE24_000034 - PubMed: Halaschek-Wiener 2009 - rs6676644 Germline - 8/94 chromosomes - - - Johan den Dunnen
-/. 1 6i c.770-131T>C r.(=) p.(=) - benign g.40746884T>C g.40281212T>C 770-131C>T - ZMPSTE24_000045 - - - rs78985808 Unknown - - - - - Claire Navarro
+/., +?/. 6 7 c.794A>G r.(?) p.(Asn265Ser), p.Asn265Ser - likely pathogenic, pathogenic g.40747039A>G g.40281367A>G - - ZMPSTE24_000018 yeast STE24 complementation slightly reduced activity CNavarro, submitted, PubMed: Agarwal 2006, PubMed: Cunningham 2010, PubMed: Shackleton 2005 - rs281875371 Germline yes - - - - Johan den Dunnen, Claire Navarro
+/. 1 7 c.826C>T r.(?) p.(Arg276*) - pathogenic g.40747071C>T g.40281399C>T - - ZMPSTE24_000039 - CNavarro, submitted - rs138606746 Germline - - - - - Claire Navarro
+/. 1 7i c.954+2T>A r.spl p.(Gly256Glufs*2) - pathogenic g.40747201T>A g.40281529T>A - - ZMPSTE24_000044 - CNavarro, submitted - rs312262688 Germline - - - - - Claire Navarro
-?/. 1 - c.954+3G>A r.spl? p.? - likely benign g.40747202G>A - ZMPSTE24(NM_005857.3):c.954+3G>A - ZMPSTE24_000060 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-/., -?/. 2 8 c.990C>T r.(?) p.(=) - benign, likely benign g.40751632C>T g.40285960C>T ZMPSTE24(NM_005857.3):c.990C>T (p.L330=) - ZMPSTE24_000046 VKGL data sharing initiative Nederland - - rs116284141 CLASSIFICATION record, Germline - - - - - Claire Navarro, VKGL-NL_Utrecht
?/. 1 - c.997C>A r.(?) p.(Leu333Ile) - VUS g.40751639C>A - ZMPSTE24(NM_005857.5):c.997C>A (p.(Leu333Ile)) - ZMPSTE24_000069 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/., +?/. 2 8 c.1018T>C r.(?) p.(Trp340Arg), p.Trp340Arg - likely pathogenic, pathogenic g.40751660T>C g.40285988T>C - - ZMPSTE24_000002 not in 200 control chromosomes, yeast STE24 complementation slightly reduced activity PubMed: Agarwal 2003, OMIM:var0002 - rs121908093 Germline - - HpaII+ - - Johan den Dunnen
+/. 3 8 c.1020G>A r.(?) p.(Trp340*) - pathogenic g.40751662G>A g.40285990G>A - - ZMPSTE24_000053 - PubMed: Ahmad 2012 - rs281875372 Germline yes - - - - Johan den Dunnen
-/. 1 8 c.1035A>G r.(?) p.(=) - benign g.40751677A>G g.40286005A>G - - ZMPSTE24_000047 - - - rs116496835 Germline - - - - - Claire Navarro
-/. 1 8i c.1059+61G>A r.(=) p.(=) - benign g.40751762G>A g.40286090G>A - - ZMPSTE24_000024 - PubMed: Halaschek-Wiener 2009 - rs188058932 Germline - 1/94 chromosomes - - - Johan den Dunnen
+/. 63 9 c.1085dup r.(?), r.1085dup p.(Leu362Phefs*19), p.(Leu362PhefsTer19), p.Leu362Phefs*19 - pathogenic g.40756551dup g.40290879dup 1085_1086insT, 1 more item - ZMPSTE24_000001, ZMPSTE24_000057 not in 200 control chromosomes, variants on different intron 4 alleles, 1 more item CNavarro, submitted, Daumer-Haas ESHG2008 P03.072, PubMed: Agarwal 2003, OMIM:var0001, PubMed: Li 2009, 9 more items - rs137854889 CLASSIFICATION record, Germline yes - - - - Johan den Dunnen, Tom Winder, Claire Navarro, VKGL-NL_Leiden, VKGL-NL_Groningen, VKGL-NL_Utrecht
+?/. 2 9 c.1105C>T r.(?) p.(Arg369*) - likely pathogenic g.40756571C>T g.40290899C>T - - ZMPSTE24_000040 - CNavarro, submitted - rs281875373 Germline - - - - - Claire Navarro
-?/. 1 - c.1165T>C r.(?) p.(Leu389=) - likely benign g.40756631T>C - ZMPSTE24(NM_005857.3):c.1165T>C (p.L389=) - ZMPSTE24_000061 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. 1 - c.1203+7T>C r.(=) p.(=) - likely benign g.40756676T>C g.40291004T>C ZMPSTE24(NM_005857.4):c.1203+7T>C (p.(=)) - ZMPSTE24_000058 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.1203+10G>A r.(=) p.(=) - likely benign g.40756679G>A - ZMPSTE24(NM_005857.5):c.1203+10G>A - ZMPSTE24_000067 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 2 9i_10 c.1204-5_1210del r.1204_1225del, r.spl? p.(fs*?), p.Val402Serfs*2 - pathogenic g.40758112_40758123del g.40292440_40292451del 1204-5_1210del12 - ZMPSTE24_000008 saves lethality LMNA:c.1960C>T PubMed: Denecke 2006 - rs312262689 Germline - - - - - Johan den Dunnen, Tom Winder
+/. 1 9i c.1204-1G>A r.spl p.(Val362Phefs*6) - pathogenic g.40758116G>A g.40292444G>A - - ZMPSTE24_000042 - CNavarro, submitted - rs61751009 Germline - - - - - Claire Navarro
+/. 1 10 c.1249C>T r.1249c>u p.Gln417* - pathogenic g.40758162C>T g.40292490C>T - - ZMPSTE24_000004 - PubMed: Navarro 2005 - rs281875374 Germline - - - - - Johan den Dunnen
+?/. 1 10 c.1263dup r.(?) p.(Ala422Cysfs*8) - likely pathogenic g.40758176dup g.40292504dup - - ZMPSTE24_000037 - - - rs281875375 Germline - - - - - Tom Winder
?/. 1 - c.1301C>T r.(?) p.(Ala434Val) - VUS g.40758214C>T - ZMPSTE24(NM_005857.5):c.1301C>T (p.(Ala434Val)) - ZMPSTE24_000070 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 10 c.1312C>T r.(?) p.(Leu438Phe) - likely pathogenic g.40758225C>T g.40292553C>T - - ZMPSTE24_000055 not in 240 control chromosomes PubMed: Dutour 2011 - rs116771294 Germline yes - - - - Johan den Dunnen
+/. 3 10 c.1349G>A r.(?) p.(Trp450*) - pathogenic g.40758262G>A g.40292590G>A - - ZMPSTE24_000013 - PubMed: Ahmad 2010, OMIM:var0009 - rs281875376 Germline yes - - - - Johan den Dunnen, Tom Winder
-/. 1 10 c.1356C>G r.(?) p.(Phe452Leu) - benign g.40758269C>G g.40292597C>G - - ZMPSTE24_000048 - - - rs281875377 Germline - - - - - Claire Navarro
+/. 2 10 c.1385T>G r.(?) p.(Leu462Arg) - pathogenic g.40758298T>G g.40292626T>G - - ZMPSTE24_000019 - PubMed: Thil 2008 - rs281875378 Germline - - TspRI - - Johan den Dunnen
-/. 1 10 c.*723C>T r.(?) p.(=) - benign g.40759064C>T g.40293392C>T - - ZMPSTE24_000022 - PubMed: Halaschek-Wiener 2009 - rs10489431 Germline - 9/94 chromosomes - - - Johan den Dunnen
-/. 1 10 c.*890A>G r.(?) p.(=) - benign g.40759231A>G g.40293559A>G - - ZMPSTE24_000023 - PubMed: Halaschek-Wiener 2009 - rs10489432 Germline - 9/94 chromosomes - - - Johan den Dunnen
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