Unique variants in the MICU1 gene

This database is one of the gene variant databases from the "Leiden Muscular Dystrophy pages" (LMDp)
Information The variants shown are described using the NM_001195518.2 transcript reference sequence.

39 entries on 1 page. Showing entries 1 - 39.
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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.-2094_-2+683del r.0? p.0 - pathogenic (recessive) g.74385085_74387860del g.72625327_72628102del del ex1 - MICU1_000042 2755bp deletion PubMed: Lewis-Smith 2016, Journal: Lewis-Smith 2016 - - Germline yes - - - - Johan den Dunnen
+/. 1 1i_2i c.(-2+1_-1-19)_(161+21_162-1)del r.-1_161del p.0? ACMG pathogenic (recessive) g.(74322822_74326370)_(74326571_74385767)del g.(72563064_72566612)_(72566813_72626009)del g. (?_74326370)_(74326571_?), del ex2 - MICU1_000037 ACMG PVS1, PM2 PubMed: Sharova 2022, Journal: Sharova 2022 - - Germline - - - - - Johan den Dunnen
+/. 1 1i_2i c.(-2+1_-1-1)_(161+1_162-1)del r.(-1_161del) p.0? - pathogenic (recessive) g.(74322822_74326390)_(74326553_74385767)del g.(72563064_72566632)_(72566795_72626009)del del ex2 - MICU1_000040 - PubMed: Kohlschmidt 2021, Journal: Kohlschmidt 2021 - - Germline - - - - - Johan den Dunnen
+?/. 1 8i_10i c.(933+1_934-69_1180+129_1181-1)dup r.? p.? - likely pathogenic (recessive) g.(74135631_74167558)_(74183198_74234857)dup g.(72375873_72407800)_(72423440_72475099)dup - (74167558_74183198)x3 mat, dup ex9-10 MICU1_000041 - PubMed: Musa 2019, Journal: Musa 2019 - - Germline - - - - - Johan den Dunnen
+/. 1 2 c.1A>G r.(?) p.0? - pathogenic g.74326551T>C g.72566793T>C - - MICU1_000014 - PubMed: O'Grady 2016 - rs747832014 Germline yes - - - - Sandra Cooper
+/. 1 2 c.40del r.(?) p.(Ala14LeufsTer20) - pathogenic (recessive) g.74326513del g.72566755del - - MICU1_000044 - PubMed: Cherot 2017 - rs749124658 Germline - - - - - Johan den Dunnen
+?/. 1 - c.52C>T r.(?) p.(Arg18Ter) - likely pathogenic (recessive) g.74326500G>A g.72566742G>A - - MICU1_000039 - PubMed: Kohlschmidt 2021, Journal: Kohlschmidt 2021 - - Germline - - - - - Johan den Dunnen
?/. 1 2 c.53G>A r.(?) p.(Gln18Arg) - VUS g.74326499C>T g.72566741C>T - - MICU1_000002 found once in 8309 European Americans, not in 3898 African Americans Exome Variant Server - - Unknown - 1/12207 controls - - - Johan den Dunnen
?/. 1 2 c.77C>G r.(?) p.(Pro26Arg) - VUS g.74326475G>C g.72566717G>C - - MICU1_000003 not in 3898 African Americans Exome Variant Server - - Unknown - 2/12236 controls - - - Johan den Dunnen
?/. 1 2 c.83A>G r.(?) p.(Gln83Arg) - VUS g.74326469T>C g.72566711T>C - - MICU1_000004 - Exome Variant Server - - Unknown - 1/12225 controls - - - Johan den Dunnen
?/. 1 2 c.114C>G r.(?) p.(Phe38Leu) - VUS g.74326438G>C g.72566680G>C - - MICU1_000005 - Exome Variant Server - - Unknown - 1/12225 controls - - - Johan den Dunnen
?/. 1 - c.119G>A r.(?) p.(Gly40Glu) - VUS g.74326433C>T g.72566675C>T MICU1(NM_001363513.1):c.119G>A (p.G40E) - MICU1_000025 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 2 - c.156G>A r.(?) p.(Trp52Ter) ACMG pathogenic (recessive) g.74326396C>T g.72566638C>T - - MICU1_000046 ACMG PVS1,  PM2_Supporting, MP3 Journal: Li 2025 - - Germline yes - - - - Johan den Dunnen
?/. 1 2 c.160A>G r.(?) p.(Arg54Gly) - VUS g.74326392T>C g.72566634T>C - - MICU1_000006 - Exome Variant Server - - Unknown - 1/12175 controls - - - Johan den Dunnen
+/., +?/. 4 2i c.161+1G>A r.spl p.0?, p.? - likely pathogenic, pathogenic (recessive) g.74326390C>T g.72566632C>T - - MICU1_000007 - Exome Variant Server, PubMed: Klee 2021, Journal: Klee 2021, PubMed: Saneto 2022, Journal: Saneto 2022, 1 more item - rs375502236 Germline, Unknown -, yes 1/12179 controls - - - Johan den Dunnen
?/. 1 3 c.211G>C r.(?) p.(Gly71Arg) - VUS g.74322772C>G g.72563014C>G - - MICU1_000008 - Exome Variant Server - - Unknown - 1/11925 controls - - - Johan den Dunnen
+?/. 2 - c.235G>T r.(?) p.(Glu79Ter) ACMG likely pathogenic (recessive) g.74322748C>A g.72562990C>A - - MICU1_000047 ACMG PVS1,  PM2_Supporting Journal: Li 2025 - - Germline yes - - - - Johan den Dunnen
+?/. 2 3i_8i c.(330+1_331-1)_(933+1_934-1)del r.? p.? ACMG likely pathogenic (recessive) g.(74183130_74234857)_(74311100_74322652)del g.(72423372_72475099)_(72551342_72562894)del - - MICU1_000049 ACMG PVS1,  PM2_Supporting Journal: Li 2025 - - Germline yes - - - - Johan den Dunnen
?/. 1 - c.355C>G r.(?) p.(Arg119Gly) - VUS g.74311075G>C g.72551317G>C MICU1(NM_001195518.1):c.355C>G (p.(Arg119Gly)) - MICU1_000027 VKGL data sharing initiative Nederland - - rs538329212 CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 - c.355C>T r.(?) p.(Arg119Ter) - pathogenic (recessive) g.74311075G>A g.72551317G>A - - MICU1_000045 - PubMed: Beheshti 2025, Journal: Beheshti 2025 - rs538329212 Germline yes - - - - Johan den Dunnen
+/., +?/. 2 4 c.385C>T r.(?) p.(Arg129*), p.(Arg129Ter) ACMG likely pathogenic, pathogenic (recessive) g.74311045G>A g.72551287G>A - - MICU1_000009 ACMG PVS1, PM3, PS4, PM2, PP3 Exome Variant Server, PubMed: Bitarafan 2021, Journal: Bitarafan 2021 - rs369069489 Germline, Unknown -, yes 1/11961 controls - - - Johan den Dunnen
+/., +?/. 4 4 c.386G>C r.(?) p.(Arg129Pro) - likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.74311044C>G g.72551286C>G - - MICU1_000013 - PubMed: Klee 2021, Journal: Klee 2021, PubMed: O'Grady 2016, 2 more items - rs375664373 Germline yes - - - - Johan den Dunnen, Sandra Cooper
+/., +?/. 2 4i c.493+1G>A r.(331_493del), r.spl p.(Val111ThrfsTer7), p.? - likely pathogenic, pathogenic (recessive) g.74310936C>T g.72551178C>T - - MICU1_000011 - Exome Variant Server, PubMed: Beheshti 2025, Journal: Beheshti 2025 - rs375775506 Germline, Unknown -, yes 2/11780 controls - - - Johan den Dunnen
+?/. 1 - c.508C>T r.(?) p.(Gln170Ter) - likely pathogenic (recessive) g.74293533G>A g.72533775G>A - - MICU1_000035 - PubMed: Beheshti 2025, Journal: Beheshti 2025 - rs779752327 Germline yes - - - - Johan den Dunnen
+?/. 1 5 c.513T>A r.(?) p.(Tyr171Ter) ACMG pathogenic g.74293528A>T g.72533770A>T - - MICU1_000028 - - - - Germline - - - - - Gautham Arunachal
+/. 5 - c.547C>T r.(?) :p.(Gln183Ter), p.(Gln183Ter) ACMG pathogenic, pathogenic (recessive) g.74268018G>A g.72508260G>A NM_006077.3:c.553C>T - MICU1_000022 ACMG PVS1,PM2,PP1, variants reported seperately, unknown if mono-allelic or bi-allelic PubMed: Alfares 2017, Journal: Alfares 2017, PubMed: Alkelai 2023, PubMed: Anazi 2017, 2 more items - rs777327250 Germline, Unknown yes - - - - Johan den Dunnen
+/. 13 - c.553C>T r.(?) p.(Arg185Ter) - pathogenic (recessive) g.74268012G>A g.72508254G>A Q185* - MICU1_000038 - PubMed: Beheshti 2025, Journal: Beheshti 2025, PubMed: Finsterer 2024, Journal: Finsterer 2024, 2 more items - rs755651388 Germline yes - - - - Johan den Dunnen
+?/. 1 7 c.638_639del r.spl p.(Thr213AsnfsTer11) - likely pathogenic g.74267928_74267929del g.72508170_72508171del NM_006077.3:c.644_645del - MICU1_000010 found 19x in European Americans, 13x in African Americans Exome Variant Server - - Unknown - 32/11324 controls - - - Johan den Dunnen
+/., +?/., ?/. 8 8i c.735+1G>A r.735_736ins[A;735+2_735+155], r.spl, r.spl? p.?, p.Val246ThrfsTer9 ACMG likely pathogenic, pathogenic, pathogenic (recessive), VUS g.74236931C>T g.72477173C>T NM_006077.3:c.741+1G>A, NM_006077.3:c.741 + 1G>A - MICU1_000001, MICU1_000026 ACMG PVS1, PS4_MOD, PM2_SUP, PM3_SUP, gene implicated in limb-girdle muscular dystrophy (LGMD), 4 more items PMID:24336167, Exome Variant Server, PubMed: Logan 2014, OMIM:var0002, 1 more item VCV000101046.9 rs369915689 Germline, Unknown -, ?, yes 1/10233 controls - - - Johan den Dunnen, Marjolein Kriek, Andreas Laner
+?/. 1 8i c.736-1G>A r.spl? p.? - likely pathogenic g.74235056C>T g.72475298C>T MICU1(NM_001195518.2):c.736-1G>A - MICU1_000033 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 - c.931G>T r.(?) p.(Glu311*) - pathogenic g.74234860C>A g.72475102C>A - - MICU1_000024 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.981T>G r.(?) p.(Phe327Leu) - VUS g.74183082A>C g.72423324A>C MICU1(NM_001195518.2):c.981T>G (p.(Phe327Leu)) - MICU1_000031 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 - c.1042C>T r.(?) p.(Gln348Ter) - pathogenic (recessive) g.74183021G>A g.72423263G>A NM_006077.3:c.1048C>T - MICU1_000043 - PubMed: Cherot 2017 - rs1135401814 Germline - - - - - Johan den Dunnen
+/., +?/. 14 10i c.1072-1G>C r.1071_1072ins[1072-34_1072-2;C], r.spl p.?, p.Gly358ValfsTer28 ACMG pathogenic, pathogenic (recessive) g.74167796C>G g.72408038C>G NM_006077.3:c.1078-1G>C, NM_006077:c.1078-1G>C - MICU1_000012 exome sequencing, homozygosity mapping, homozygosity mapping, exome sequencing PubMed: Fevga 2022, Journal: Fevga 2022, PubMed: Logan 2014, OMIM:var0001, 1 more item - rs754639936 Germline yes - - - - Johan den Dunnen, Gautham Arunachal
+?/. 1 - c.1226C>G r.(?) p.(Ser409Ter) - likely pathogenic (recessive) g.74135585G>C g.72375827G>C - - MICU1_000034 - PubMed: Beheshti 2025, Journal: Beheshti 2025 - - Germline yes - - - - Johan den Dunnen
-?/. 1 - c.1270+9G>A r.(=) p.(=) - likely benign g.74135532C>T g.72375774C>T MICU1(NM_001195518.2):c.1270+9G>A - MICU1_000032 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 2 - c.1289del r.(1289del) p.(Asn430IlefsTer8) ACMG pathogenic (recessive) g.74128096del g.72368338del NM_006077.3:1295delA - MICU1_000036 ACMG PVS1, PM2, PM4, PP3, PP4 PubMed: Mojbafan 2020, Journal: Mojbafan 2020 - - Germline yes - - - - Johan den Dunnen
?/. 1 - c.1319G>A r.(?) p.(Arg440Gln) - VUS g.74128065C>T g.72368307C>T MICU1(NM_001195518.2):c.1319G>A (p.(Arg440Gln)) - MICU1_000030 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 2 - c.1372C>T r.(?) p.(Gln458Ter) ACMG VUS g.74128012G>A g.72368254G>A - - MICU1_000048 ACMG PVS1_Moderate, PM2_Supporting, MP3 Journal: Li 2025 - - Germline yes - - - - Johan den Dunnen
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