All variants in the LBR gene

Information The variants shown are described using the NM_002296.3 transcript reference sequence.

78 entries on 1 page. Showing entries 1 - 78.
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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     
-/? 1i c.-15+90C>G r.(=) p.(=) - benign g.225614814G>C - -13838C>G - LBR_000002 83 homozygous individuals Variant Error [EMISMATCH]: This variant seems to mismatch; the genomic and the transcript variant seems to not belong together. Please fix this entry and then remove this message. PubMed: Gaudy-Marqueste 2009 - - Germline - 200/254 chromosomes - - - LOVD
?/? 1i c.-15+90C>G r.(=) p.(=) - VUS g.225614814G>C - - - LBR_000002 Variant Error [EMISMATCH]: This variant seems to mismatch; the genomic and the transcript variant seems to not belong together. Please fix this entry and then remove this message. - - rs4653636 Unknown - - - - - LOVD
?/. - c.-14-9A>G r.(=) p.(=) - VUS g.225611800T>C - LBR(NM_194442.2):c.-14-9A>G - LBR_000033 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/? - c.? r.(=) p.(=) - benign g.225620987G>C g.225433285G>C NM_194442.2:c.-4594C>G - LBR_000001 no homozygous individuals PubMed: Gaudy-Marqueste 2009 - - Germline - 27/254 chromosomes - - - LOVD
-?/. - c.44G>A r.(?) p.(Arg15Gln) - likely benign g.225611734C>T - LBR(NM_002296.3):c.44G>A (p.(Arg15Gln)) - LBR_000054 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/? 2 c.90C>T r.(?) p.(=) - benign g.225611688G>A g.225423986G>A H30H - LBR_000004 - PubMed: Gaudy-Marqueste 2009 - - Germline - 1/254 chromosomes - - - LOVD
?/? 2 c.90C>T r.(?) p.(=) - VUS g.225611688G>A g.225423986G>A H30H - LBR_000004 - - - rs2230416 Unknown - - - - - LOVD
-/. - c.117G>A r.(?) p.(Val39=) - benign g.225611661C>T g.225423959C>T LBR(NM_002296.4):c.117G>A (p.V39=) - LBR_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-/? 2 c.117G>A r.(?) p.(=) - benign g.225611661C>T g.225423959C>T V39V - LBR_000003 46 homozygous individuals PubMed: Gaudy-Marqueste 2009 - - Germline - 142/254 chromosomes - - - LOVD
?/? 2 c.117G>A r.(?) p.(=) - VUS g.225611661C>T g.225423959C>T V39V - LBR_000003 - - - rs1056607 Unknown - - - - - LOVD
-?/? 2i c.165+74C>T r.(=) p.(=) - likely benign g.225611539G>A g.225423837G>A - - LBR_000005 not in 200 control chromosomes PubMed: Gaudy-Marqueste 2009 - - Germline - 1/27 patients - - - LOVD
-/? 2i c.166-261A>G r.(=) p.(=) - benign g.225610240T>C g.225422538T>C - - LBR_000006 no homozygous individuals PubMed: Gaudy-Marqueste 2009 - - Germline - 37/254 chromosomes - - - LOVD
?/. - c.208A>T r.(?) p.(Ser70Cys) - VUS g.225609937T>A - LBR(NM_002296.3):c.208A>T (p.(Ser70Cys)) - LBR_000053 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.236G>A r.(?) p.(Arg79Gln) - VUS g.225609909C>T - LBR(NM_002296.3):c.236G>A (p.(Arg79Gln)) - LBR_000052 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/. - c.261T>C r.(?) p.(Pro87=) - benign g.225609884A>G g.225422182A>G LBR(NM_002296.4):c.261T>C (p.P87=) - LBR_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-/? 3 c.261T>C r.(?) p.(=) - benign g.225609884A>G g.225422182A>G P87P - LBR_000007 73 homozygous individuals PubMed: Gaudy-Marqueste 2009 - - Germline - 184/254 chromosomes - - - LOVD
?/? 3 c.261T>C r.(?) p.(=) - VUS g.225609884A>G g.225422182A>G P87P - LBR_000007 - - - rs1056608 Unknown - - - - - LOVD
-?/. - c.312C>T r.(?) p.(Ala104=) - likely benign g.225609833G>A - LBR(NM_194442.2):c.312C>T (p.A104=) - LBR_000032 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. - c.356C>T r.(?) p.(Pro119Leu) - VUS g.225609789G>A - - - LBR_000041 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. - c.366+1G>T r.spl? p.? - likely pathogenic g.225609778C>A g.225422076C>A LBR(NM_002296.4):c.366+1G>T - LBR_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-/. - c.366+16C>T r.(=) p.(=) - benign g.225609763G>A - - - LBR_000040 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/? 3i c.366+16G>A r.(=) p.(=) - benign g.225609763C>T - - - LBR_000008 no homozygous individuals Variant Error [EREF/EREF]: This genomic variant does not match the reference sequence; the transcript variant does not match the reference sequence either. Please fix this entry and then remove this message. PubMed: Gaudy-Marqueste 2009 - - Germline - 24/254 chromosomes - - - LOVD
?/? 3i c.366+16G>A r.(=) p.(=) - VUS g.225609763C>T - - - LBR_000008 Variant Error [EREF/EREF]: This genomic variant does not match the reference sequence; the transcript variant does not match the reference sequence either. Please fix this entry and then remove this message. - - rs12410357 Unknown - - - - - LOVD
+/. - c.367-2A>C r.spl? p.? - pathogenic g.225607502T>G g.225419800T>G - - LBR_000025 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. - c.398A>T r.(?) p.(Asn133Ile) - VUS g.225607469T>A - LBR(NM_002296.4):c.398A>T (p.(Asn133Ile)) - LBR_000059 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/. - c.461G>A r.(?) p.(Ser154Asn) - benign g.225607144C>T g.225419442C>T LBR(NM_002296.4):c.461G>A (p.S154N) - LBR_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-/? 5 c.461G>A r.(?) p.(Ser154Asn) - benign g.225607144C>T g.225419442C>T - - LBR_000009 84 homozygous individuals PubMed: Gaudy-Marqueste 2009 - - Germline - 204/254 chromosomes - - - LOVD
?/? 5 c.461G>A r.(?) p.(Ser154Asn) - VUS g.225607144C>T g.225419442C>T - - LBR_000009 - - - rs2230419 Unknown - - - - - LOVD
?/. - c.512G>A r.(?) p.(Arg171Lys) - VUS g.225607093C>T - LBR(NM_194442.2):c.512G>A (p.R171K) - LBR_000031 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.562G>A r.(?) p.(Val188Ile) - likely benign g.225607043C>T g.225419341C>T LBR(NM_002296.3):c.562G>A (p.(Val188Ile)) - LBR_000024 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.607C>T r.(?) p.(Arg203Trp) - VUS g.225606998G>A - LBR(NM_002296.4):c.607C>T (p.(Arg203Trp)) - LBR_000058 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. - c.640+233C>G r.(=) p.(=) - likely benign g.225606732G>C - LBR(NM_002296.4):c.640+233C>G - LBR_000057 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.655A>G r.(?) p.(Met219Val) - VUS g.225605868T>C - LBR(NM_002296.3):c.655A>G (p.(Met219Val)) - LBR_000051 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.715A>T r.(?) p.(Ser239Cys) - VUS g.225605808T>A - LBR(NM_002296.3):c.715A>T (p.(Ser239Cys)) - LBR_000034 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.724A>G r.(?) p.(Asn242Asp) - VUS g.225605799T>C - LBR(NM_194442.2):c.724A>G (p.N242D) - LBR_000028 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. - c.743C>T r.(?) p.(Pro248Leu) - VUS g.225605780G>A - LBR(NM_002296.3):c.743C>T (p.(Pro248Leu)), LBR(NM_194442.2):c.743C>T (p.P248L) - LBR_000027 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. - c.743C>T r.(?) p.(Pro248Leu) - VUS g.225605780G>A - LBR(NM_002296.3):c.743C>T (p.(Pro248Leu)), LBR(NM_194442.2):c.743C>T (p.P248L) - LBR_000027 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. - c.837+4C>T r.spl? p.? - likely benign g.225605682G>A g.225417980G>A LBR(NM_002296.3):c.837+4C>T (p.?) - LBR_000023 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/? 7i c.838-69G>A r.(=) p.(=) - benign g.225603103C>T g.225415401C>T - - LBR_000010 91 homozygous individuals PubMed: Gaudy-Marqueste 2009 - - Germline - 213/254 chromosomes - - - LOVD
?/? 7i c.838-69G>A r.(=) p.(=) - VUS g.225603103C>T g.225415401C>T - - LBR_000010 - - - rs6702433 Unknown - - - - - LOVD
-/. - c.893-5del r.spl? p.? - benign g.225600367del g.225412665del LBR(NM_002296.3):c.893-5del (p.?), LBR(NM_002296.4):c.893-5delT - LBR_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.893-5del r.spl? p.? - likely benign g.225600367del g.225412665del LBR(NM_002296.3):c.893-5del (p.?), LBR(NM_002296.4):c.893-5delT - LBR_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/. - c.893-5del r.spl? p.? - benign g.225600367del - LBR(NM_002296.3):c.893-5del (p.?), LBR(NM_002296.4):c.893-5delT - LBR_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. - c.893-5dup r.spl? p.? - likely benign g.225600367dup g.225412665dup LBR(NM_002296.3):c.893-5dup (p.?) - LBR_000022 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.899A>G r.(?) p.(Tyr300Cys) - VUS g.225600341T>C g.225412639T>C LBR(NM_002296.3):c.899A>G (p.(Tyr300Cys)), LBR(NM_194442.2):c.899A>G (p.Y300C) - LBR_000026 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.899A>G r.(?) p.(Tyr300Cys) - likely benign g.225600341T>C - LBR(NM_002296.3):c.899A>G (p.(Tyr300Cys)), LBR(NM_194442.2):c.899A>G (p.Y300C) - LBR_000026 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. - c.959A>G r.(?) p.(His320Arg) - likely benign g.225600281T>C g.225412579T>C LBR(NM_002296.3):c.959A>G (p.(His320Arg)) - LBR_000021 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.991C>T r.(?) p.(Leu331Phe) - VUS g.225600249G>A - LBR(NM_002296.3):c.991C>T (p.(Leu331Phe)) - LBR_000050 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.1027T>C r.(?) p.(Tyr343His) - VUS g.225600213A>G - LBR(NM_002296.4):c.1027T>C (p.(Tyr343His)) - LBR_000048 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.1040G>A r.(?) p.(Arg347His) - VUS g.225600200C>T - LBR(NM_002296.4):c.1040G>A (p.(Arg347His)) - LBR_000047 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. - c.1057C>T r.(?) p.(Arg353Trp) - likely benign g.225600183G>A - LBR(NM_194442.2):c.1057C>T (p.R353W) - LBR_000038 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/? 8 c.1071G>A r.(?) p.(=) - benign g.225600169C>T g.225412467C>T S357S - LBR_000011 no homozygous individuals PubMed: Gaudy-Marqueste 2009 - - Germline - 7/254 chromosomes - - - LOVD
?/. - c.1114C>T r.(?) p.(Arg372Cys) - VUS g.225599113G>A g.225411411G>A LBR(NM_002296.3):c.1114C>T (p.(Arg372Cys)) - LBR_000019 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. - c.1114C>T r.(?) p.(Arg372Cys) - likely pathogenic g.225599113G>A g.225411411G>A - - LBR_000019 4 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs200180113 Germline - 4/2794 individuals - - - Mohammed Faruq
?/. - c.1115G>A r.(?) p.(Arg372His) - VUS g.225599112C>T - LBR(NM_002296.4):c.1115G>A (p.(Arg372His)) - LBR_000046 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 9 c.1174G>A r.(?) p.(Gly392Arg) - pathogenic (recessive) g.225599053C>T - NM_002296.3:c.1174G>A - LBR_000036 - PubMed: Zhang-2019 - - Germline - - - - - LOVD
-?/. - c.1188+4T>C r.spl? p.? - likely benign g.225599035A>G - LBR(NM_002296.3):c.1188+4T>C (p.?) - LBR_000037 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.1384G>T r.(?) p.(Val462Leu) - VUS g.225594465C>A - LBR(NM_002296.4):c.1384G>T (p.(Val462Leu)) - LBR_000056 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. - c.1399A>T r.(?) p.(Ile467Phe) - likely benign g.225594450T>A - LBR(NM_194442.2):c.1399A>T (p.I467F) - LBR_000030 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. - c.1432C>G r.(?) p.(His478Asp) - likely pathogenic g.225594417G>C - - - LBR_000039 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. - c.1483C>T r.(?) p.(Leu495Phe) - likely benign g.225594366G>A - LBR(NM_002296.3):c.1483C>T (p.(Leu495Phe)) - LBR_000049 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. - c.1483+1G>A r.spl? p.? - pathogenic g.225594365C>T - - - LBR_000043 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. - c.1528G>A r.(?) p.(Ala510Thr) - benign g.225592364C>T g.225404662C>T LBR(NM_002296.4):c.1528G>A (p.A510T) - LBR_000020 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. - c.1534C>T r.(?) p.(Arg512Trp) - pathogenic g.225592358G>A - LBR(NM_002296.4):c.1534C>T (p.(Arg512Trp)) - LBR_000045 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 12 c.1535G>A r.(?) p.(Arg512Gln) - pathogenic (recessive) g.225592357C>T - NM_002296.3:c.1535G>A - LBR_000035 - PubMed: Zhang-2019 - - Germline - - - - - LOVD
?/. - c.1561G>T r.(?) p.(Ala521Ser) - VUS g.225592331C>A - LBR(NM_002296.4):c.1561G>T (p.(Ala521Ser)) - LBR_000055 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.1631G>A r.(?) p.(Arg544His) - VUS g.225592162C>T - LBR(NM_002296.4):c.1631G>A (p.(Arg544His)) - LBR_000044 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. - c.1639A>G r.(?) p.(Asn547Asp) ACMG likely pathogenic (recessive) g.225592154T>C g.225404452T>C - - LBR_000042 ACMG: PM3_STR, PS3_MOD, PP3_MOD, PM2_SUP PMID: 21327084, 18382993, 27830109, 27336722 VCV000100900.8 - Germline ? - - - - Andreas Laner
+/. - c.1672T>C r.(?) p.(Trp558Arg) - likely pathogenic (recessive) g.225592121A>G g.225404419A>G NM_002296.3:c.1672T>C:p.(Trp558Arg) - LBR_000029 - PubMed: Maddirevula 2018 - - Germline - - - - - LOVD
?/. - c.1771T>C r.(?) p.(Cys591Arg) - VUS g.225591082A>G - LBR(NM_002296.4):c.1771T>C (p.C591R) - DNAH14_000028 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. - c.1801A>G r.(?) p.(Lys601Glu) - likely benign g.225591052T>C - LBR(NM_002296.3):c.1801A>G (p.(Lys601Glu)) - DNAH14_000061 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.1828A>C r.(?) p.(Ile610Leu) - VUS g.225591025T>G - LBR(NM_002296.4):c.1828A>C (p.I610L) - DNAH14_000062 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+?/. - c.1834C>A r.(?) p.(Pro612Thr) - likely pathogenic g.225591019G>T - - - DNAH14_000027 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/? 14 c.*22A>C r.(?) p.(=) - likely benign g.? - - - LBR_000012 not in 200 control chromosomes PubMed: Gaudy-Marqueste 2009 - - Germline - 1/27 patients - - - LOVD
-?/. - c.*4034T>A r.(=) p.(=) - likely benign g.225586971A>T g.225399269A>T DNAH14(NM_001373.1):c.13548A>T (p.(*4516Tyrext*?)) - DNAH14_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. - c.*4104G>T r.(=) p.(=) - likely benign g.225586901C>A - DNAH14(NM_001367479.1):c.13784C>A (p.(Ser4595Ter)) - DNAH14_000049 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. - c.*4198C>T r.(=) p.(=) - likely benign g.225586807G>A g.225399105G>A DNAH14(NM_001373.1):c.13384G>A (p.(Ala4462Thr)) - DNAH14_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. - c.*4267dup r.(?) p.(=) - likely benign g.225586751dup - DNAH14(NM_001367479.1):c.13639-5dup - DNAH14_000048 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
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