All variants in the KLHL40 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_152393.3 transcript reference sequence.

71 entries on 1 page. Showing entries 1 - 71.
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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.25G>T r.(?) p.(Glu9*) - VUS g.42727135G>T - KLHL40(NM_152393.4):c.25G>T (p.(Glu9Ter)) - KLHL40_000047 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/+? 1 c.46C>T r.(?) p.(Gln16*) - pathogenic g.42727156C>T g.42685664C>T - - KLHL40_000025 - PubMed: Todd 2015 - - Germline - - - - - Gianina Ravenscroft
+/+ 1 c.100G>C r.(?) p.(Asp34His) - pathogenic g.42727210G>C g.42685718G>C - - KLHL40_000001 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
?/. - c.134C>T r.(?) p.(Pro45Leu) - VUS g.42727244C>T - KLHL40(NM_152393.4):c.134C>T (p.(Pro45Leu)) - KLHL40_000048 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/+ 1 c.134del r.(?) p.(Pro45Argfs*19) - pathogenic g.42727244del g.42685752del - - KLHL40_000002 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
?/. - c.238G>C r.(?) p.(Asp80His) - VUS g.42727348G>C g.42685856G>C KLHL40(NM_152393.3):c.238G>C (p.D80H) - KLHL40_000029 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/+ 1 c.257T>C r.(?) p.(Leu86Pro) - pathogenic g.42727367T>C g.42685875T>C - - KLHL40_000017 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 1 c.270C>G r.(?) p.(Tyr90*) - pathogenic g.42727380C>G g.42685888C>G - - KLHL40_000003 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
?/. - c.313T>C r.(?) p.(Phe105Leu) - VUS g.42727423T>C g.42685931T>C KLHL40(NM_152393.3):c.313T>C (p.(Phe105Leu)) - KLHL40_000035 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. - c.360C>A r.(?) p.(Cys120Ter) - pathogenic g.42727470C>A g.42685978C>A KLHL40(NM_152393.4):c.360C>A (p.C120*) - KLHL40_000030 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+?/. - c.515T>C r.(?) p.(Leu172Pro) - likely pathogenic g.42727625T>C g.42686133T>C - - KLHL40_000041 - PubMed: Gonzalez-Quereda 2020 - - Germline - - - - - Johan den Dunnen
+/+ 1 c.581T>A r.(?) p.(Val194Glu) - pathogenic g.42727691T>A g.42686199T>A - - KLHL40_000004 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+?/+? 1 c.601T>C r.(?) p.(Trp201Arg) - likely pathogenic g.42727711T>C g.42686219T>C - - KLHL40_000026 - - - - Germline - - - - - Tom Winder
+/+ 1 c.602G>A r.(?) p.(Trp201*) - pathogenic g.42727712G>A g.42686220G>A - - KLHL40_000006 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/. - c.602G>A r.(?) p.(Trp201*) ACMG pathogenic (recessive) g.42727712G>A g.42686220G>A - - KLHL40_000006 - PubMed: Ravenscroft 2020, Journal: Ravenscroft 2020 - - Germline - - - - - Gianina Ravenscroft
+/+ 1 c.602G>T r.(?) p.(Trp201Leu) - pathogenic g.42727712G>T g.42686220G>T - - KLHL40_000005 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+?/. - c.604del r.(?) p.(Ala202ArgfsTer56) ACMG pathogenic (recessive) g.42727714del g.42686222del 604delG - KLHL40_000042 ACMG PVS1, PM2, PM3 PubMed: Natera-de Benito 2021 - - Germline - - - - - Johan den Dunnen
-?/. - c.648G>T r.(?) p.(Ala216=) - likely benign g.42727758G>T g.42686266G>T KLHL40(NM_152393.4):c.648G>T (p.A216=) - KLHL40_000040 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/+ 1 c.790del r.(?) p.(Arg264Alafs*59) - pathogenic g.42727900del g.42686408del - - KLHL40_000007 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
?/? 1 c.931C>A r.(?) p.(Arg311Ser) - VUS g.42728041C>A g.42686549C>A - - KLHL40_000028 - PubMed: Todd 2015 - - Germline yes - - - - Gianina Ravenscroft
+/+ 1 c.932G>T r.(?) p.(Arg311Leu) - pathogenic (recessive) g.42728042G>T g.42686550G>T - - KLHL40_000008 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
-/. 1 c.1034A>G r.(?) p.(Asn345Ser) - benign g.42728144A>G g.42686652A>G - - KLHL40_000033 - - - rs6805421 Unknown ? 0.8 controls - - - Florian Erger
+?/. - c.1174T>C r.(?) p.(Trp392Arg) - likely pathogenic g.42729655T>C g.42688163T>C - - KLHL40_000043 - PubMed: Gonzalez-Quereda 2020 - - Germline - - - - - Johan den Dunnen
+/? 2 c.1190C>T r.(?) p.(Pro397Leu) - pathogenic g.42729671C>T g.42688179C>T - - KLHL40_000009 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
?/. - c.1204C>T r.(?) p.(Arg402Cys) - VUS g.42729685C>T - KLHL40(NM_152393.4):c.1204C>T (p.(Arg402Cys)) - HHATL_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.1246G>A r.(?) p.(Val416Met) - VUS g.42729727G>A - KLHL40(NM_152393.4):c.1246G>A (p.(Val416Met)) - HHATL_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/+ 2 c.1270_1272delinsAGATCAAGGT r.(?) p.(Asp424Argfs*23) - pathogenic g.42729751_42729753delinsAGATCAAGGT g.42688259_42688261delinsAGATCAAGGT - - KLHL40_000010 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
?/. - c.1275C>T r.(?) p.(=) - VUS g.42729756C>T - KLHL40(NM_152393.4):c.1275C>T (p.(Gly425=)) - HHATL_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/+ 2 c.1281_1294del r.(?) p.(Cys428Hisfs*12) - pathogenic g.42729762_42729775del g.42688270_42688283del 1281_1294delCTGCCTGGACTCGG - KLHL40_000011 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 3 c.1364A>G r.(?) p.(His455Arg) - pathogenic g.42730152A>G g.42688660A>G - - KLHL40_000012 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
?/. - c.1405G>A r.(?) p.(Gly469Ser) ACMG VUS g.42730193G>A g.42688701G>A - - KLHL40_000045 - PubMed: Gurgel-Giannetti 2022 - - Germline - - - - - Johan den Dunnen
?/. - c.1405G>A r.(?) p.(Gly469Ser) ACMG VUS g.42730193G>A g.42688701G>A - - KLHL40_000045 - PubMed: Gurgel-Giannetti 2022 - - Germline - - - - - Johan den Dunnen
+/+ 3 c.1405G>T r.(?) p.(Gly469Cys) - pathogenic g.42730193G>T g.42688701G>T - - KLHL40_000013 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 3 c.1405G>T r.(?) p.(Gly469Cys) - pathogenic g.42730193G>T g.42688701G>T - - KLHL40_000013 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+?/+? 3i c.1421+1G>T r.spl p.? - likely pathogenic g.42730210G>T g.42688718G>T - - KLHL40_000027 - - - - Germline - - - - - Tom Winder
-?/. - c.1421+9G>A r.(=) p.(=) - likely benign g.42730218G>A - KLHL40(NM_152393.4):c.1421+9G>A - HHATL_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/. - c.1427G>A r.(?) p.(Cys476Tyr) - VUS g.42730366G>A - KLHL40(NM_152393.4):c.1427G>A (p.(Cys476Tyr)) - HHATL_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. - c.1495G>A r.(?) p.(Ala499Thr) - likely benign g.42730434G>A g.42688942G>A - - KLHL40_000038 1 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs148921329 Germline - 1/2795 individuals - - - Mohammed Faruq
?/. - c.1498C>T r.(?) p.(Arg500Cys) ACMG VUS g.42730437C>T g.42688945C>T - - KLHL40_000046 - PubMed: Gurgel-Giannetti 2022 - - Germline - - - - - Johan den Dunnen
?/. - c.1498C>T r.(?) p.(Arg500Cys) ACMG VUS g.42730437C>T g.42688945C>T - - KLHL40_000046 - PubMed: Gurgel-Giannetti 2022 - - Germline - - - - - Johan den Dunnen
+/. - c.1513G>C r.(?) p.(Ala505Pro) ACMG VUS g.42730452G>C g.42688960G>C - - KLHL40_000044 ACMG PM2, PM3, PP3 PubMed: Natera-de Benito 2021 - - Germline - - - - - Johan den Dunnen
+/+ 4 c.1516A>C r.(?) p.(Thr506Pro) - pathogenic (recessive) g.42730455A>C g.42688963A>C - - KLHL40_000022 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/. 4 c.1516A>C r.(?) p.(Thr506Pro) ACMG pathogenic (recessive) g.42730455A>C g.42688963A>C - - KLHL40_000022 ACMG PM3_VS, PM2, PP3 PubMed: Mao 2025 - - Germline - - - - - Johan den Dunnen
-?/. - c.1540G>A r.(?) p.(Val514Met) - likely benign g.42730479G>A - KLHL40(NM_152393.4):c.1540G>A (p.V514M) - HHATL_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/+ 4 c.1582G>A r.(?) p.(Glu528Lys) - pathogenic g.42730521G>A g.42689029G>A - - KLHL40_000014 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 4 c.1582G>A r.(?) p.(Glu528Lys) - pathogenic g.42730521G>A g.42689029G>A - - KLHL40_000014 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 4 c.1582G>A r.(?) p.(Glu528Lys) - pathogenic g.42730521G>A g.42689029G>A - - KLHL40_000014 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 4 c.1582G>A r.(?) p.(Glu528Lys) - pathogenic g.42730521G>A g.42689029G>A - - KLHL40_000014 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 4 c.1582G>A r.(?) p.(Glu528Lys) - pathogenic g.42730521G>A g.42689029G>A - - KLHL40_000014 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 4 c.1582G>A r.(?) p.(Glu528Lys) - pathogenic g.42730521G>A g.42689029G>A - - KLHL40_000014 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 4 c.1582G>A r.(?) p.(Glu528Lys) - pathogenic g.42730521G>A g.42689029G>A - - KLHL40_000014 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 4 c.1582G>A r.(?) p.(Glu528Lys) - pathogenic g.42730521G>A g.42689029G>A - - KLHL40_000014 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 4 c.1582G>A r.(?) p.(Glu528Lys) - pathogenic g.42730521G>A g.42689029G>A - - KLHL40_000014 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 4 c.1582G>A r.(?) p.(Glu528Lys) - pathogenic g.42730521G>A g.42689029G>A - - KLHL40_000014 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 4 c.1582G>A r.(?) p.(Glu528Lys) - pathogenic g.42730521G>A g.42689029G>A - - KLHL40_000014 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 4 c.1582G>A r.(?) p.(Glu528Lys) - pathogenic g.42730521G>A g.42689029G>A - - KLHL40_000014 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 4 c.1582G>A r.(?) p.(Glu528Lys) - pathogenic g.42730521G>A g.42689029G>A - - KLHL40_000024 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 4 c.1582G>A r.(?) p.(Glu528Lys) - pathogenic g.42730521G>A g.42689029G>A - - KLHL40_000014 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 4 c.1582G>A r.(?) p.(Glu528Lys) - pathogenic g.42730521G>A g.42689029G>A - - KLHL40_000014 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 4i c.1608-1G>A r.spl p.? - pathogenic g.42732350G>A g.42690858G>A - - KLHL40_000015 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
+/+ 5 c.1612G>C r.(?) p.(Ala538Pro) - pathogenic g.42732355G>C g.42690863G>C - - KLHL40_000016 - PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
?/. - c.1709C>T r.(?) p.(Thr570Met) - VUS g.42732452C>T g.42690960C>T - - KLHL40_000039 1 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs139268932 Germline - 1/2795 individuals - - - Mohammed Faruq
-?/. - c.1738C>A r.(?) p.(Leu580Ile) - likely benign g.42732481C>A g.42690989C>A KLHL40(NM_152393.4):c.1738C>A (p.L580I) - KLHL40_000031 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. - c.1755G>A r.(?) p.(=) - likely benign g.42733374G>A - KLHL40(NM_152393.4):c.1755G>A (p.(Arg585=)) - HHATL_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/-? 6 c.1762G>A r.(?) p.(Glu588Lys) - pathogenic g.42733381G>A g.42691889G>A - - KLHL40_000023 variant classification changed based on frequency an present of unaffected homozygous individual (ExAC database) PubMed: Ravenscroft 2013 - - Germline - - - - - Gianina Ravenscroft
?/. - c.1792C>G r.(?) p.(Arg598Gly) - VUS g.42733411C>G g.42691919C>G KLHL40(NM_152393.3):c.1792C>G (p.R598G) - KLHL40_000032 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 6 c.1849T>C r.(?) p.(Cys617Arg) - benign g.42733468T>C g.42691976T>C - - KLHL40_000034 - - - rs123509 Unknown ? 0.85 controls - - - Florian Erger
+?/. - c.*152G>T r.(=) p.(=) - likely pathogenic g.42733637G>T - KLHL40(NM_152393.4):c.*152G>T - HHATL_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. - c.*152G>T r.(=) p.(=) - likely pathogenic g.42733637G>T - KLHL40(NM_152393.4):c.*152G>T - HHATL_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. - c.*346T>G r.(=) p.(=) - likely benign g.42733831T>G - KLHL40(NM_152393.4):c.*346T>G - HHATL_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/. - c.*1131T>C r.(=) p.(=) - VUS g.42734616T>C g.42693124T>C HHATL(NM_020707.3):c.1343A>G (p.(Asn448Ser)) - HHATL_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
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