Unique variants in the LONP1 gene

Information The variants shown are described using the NM_004793.3 transcript reference sequence.

71 entries on 1 page. Showing entries 1 - 71.
Legend   How to query  

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.-631G>C r.(?) p.(=) - likely benign g.5720774C>G g.5720763C>G CATSPERD(NM_152784.3):c.26C>G (p.(Ala9Gly)) - CATSPERD_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.-86G>A r.(?) p.(=) - likely benign g.5720229C>T g.5720218C>T LONP1(NM_001276479.1):c.-86G>A (p.(=)) - CATSPERD_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 - c.44G>C r.(?) p.(Arg15Pro) - pathogenic (recessive) g.5720100C>G g.5720089C>G - - LONP1_000031 - PubMed: Patel 2017 - - Germline yes - - - - Johan den Dunnen
-?/. 1 - c.57G>C r.(?) p.(Leu19=) - likely benign g.5720087C>G g.5720076C>G LONP1(NM_004793.3):c.57G>C (p.L19=) - CATSPERD_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.70C>A r.(?) p.(Leu24Met) - likely benign g.5720074G>T g.5720063G>T LONP1(NM_001276479.1):c.70C>A (p.(Leu24Met)) - CATSPERD_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.70C>G r.(?) p.(Leu24Val) - likely benign g.5720074G>C g.5720063G>C LONP1(NM_001276479.1):c.70C>G (p.(Leu24Val)) - CATSPERD_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.79G>C r.(?) p.(Ala27Pro) - VUS g.5720065C>G g.5720054C>G - - LONP1_000015 - PubMed: Taylor 2014 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.121C>G r.(?) p.(Arg41Gly) - likely benign g.5720023G>C - LONP1(NM_001276479.1):c.121C>G (p.(Arg41Gly)) - CATSPERD_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.233C>T r.(?) p.(Ala78Val) - likely benign g.5719911G>A g.5719900G>A LONP1(NM_004793.3):c.233C>T (p.(Ala78Val)) - CATSPERD_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.297C>T r.(?) p.(Gly99=) - likely benign g.5719847G>A g.5719836G>A LONP1(NM_004793.3):c.297C>T (p.G99=) - CATSPERD_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.312G>A r.(?) p.(=) - likely benign g.5719832C>T - LONP1(NM_001276479.2):c.125-5G>A - CATSPERD_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.356A>G r.(?) p.(Asp119Gly) - VUS g.5719788T>C - LONP1(NM_004793.3):c.356A>G (p.(Asp119Gly)) - CATSPERD_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.496G>A r.(?) p.(Val166Ile) - likely benign g.5714216C>T - LONP1(NM_001276479.1):c.304G>A (p.(Val102Ile)) - LONP1_000027 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.684G>C r.(?) p.(Glu228Asp) - likely benign g.5711968C>G g.5711957C>G LONP1(NM_004793.3):c.684G>C (p.(Glu228Asp)) - LONP1_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/. 1 - c.730_732del r.(?) p.(Lys244del) - benign g.5711923_5711925del g.5711912_5711914del LONP1(NM_004793.3):c.730_732delAAG (p.K244del) - LONP1_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 2 - c.742G>A r.(?) p.(Asp248Asn) - likely benign g.5711910C>T g.5711899C>T LONP1(NM_001276479.1):c.550G>A (p.D184N, p.(Asp184Asn)) - LONP1_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
-?/. 1 - c.870+7C>T r.(=) p.(=) - likely benign g.5711775G>A g.5711764G>A LONP1(NM_001276479.1):c.678+7C>T (p.(=)) - LONP1_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.870+8C>T r.(=) p.(=) - likely benign g.5711774G>A - LONP1(NM_001276479.1):c.678+8C>T (p.(=)) - LONP1_000026 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.1010C>T r.(?) p.(Ala337Val) - VUS g.5707760G>A - LONP1(NM_001276479.1):c.818C>T (p.(Ala273Val)) - LONP1_000028 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.1208_1210del r.(?) p.(Ile403del) - VUS g.5705944_5705946del g.5705933_5705935del LONP1(NM_001276479.1):c.1016_1018del (p.(Ile339del)) - LONP1_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.1264C>T r.(?) p.(Arg422Trp) - VUS g.5705886G>A g.5705875G>A LONP1(NM_001276479.1):c.1072C>T (p.(Arg358Trp)) - LONP1_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.1299C>T r.(?) p.(His433=) - likely benign g.5705851G>A - LONP1(NM_004793.3):c.1299C>T (p.H433=) - LONP1_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/., ?/. 2 - c.1309G>A r.(?) p.(Val437Ile) - likely benign, VUS g.5705841C>T - LONP1(NM_001276479.1):c.1117G>A (p.(Val373Ile)), LONP1(NM_004793.3):c.1309G>A (p.V437I) - LONP1_000023 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
-?/. 1 - c.1368-4G>A r.spl? p.? - likely benign g.5700942C>T g.5700931C>T LONP1(NM_004793.3):c.1368-4G>A - LONP1_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.1402A>G r.(?) p.(Ile468Val) - likely benign g.5700904T>C - LONP1(NM_001276479.1):c.1210A>G (p.(Ile404Val)) - LONP1_000022 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.1477G>A r.(?) p.(Gly493Ser) - VUS g.5700829C>T - LONP1(NM_004793.4):c.1477G>A (p.G493S) - LONP1_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
?/. 1 - c.1600C>T r.(?) p.(Arg534Cys) - VUS g.5699123G>A - LONP1(NM_004793.3):c.1600C>T (p.(Arg534Cys)) - LONP1_000032 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.1607T>C r.(?) p.(Ile536Thr) - VUS g.5699116A>G - LONP1(NM_004793.4):c.1607T>C (p.(Ile536Thr)) - LONP1_000034 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 - c.1612C>T r.(?) p.(Arg538Cys) - pathogenic (recessive) g.5699111G>A g.5699100G>A - - LONP1_000030 - PubMed: Patel 2017 - - Germline yes - - - - Johan den Dunnen
-?/. 1 - c.1632C>T r.(?) p.(Tyr544=) - likely benign g.5699091G>A - LONP1(NM_004793.3):c.1632C>T (p.Y544=) - LONP1_000017 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.1661A>T r.(?) p.(Asp554Val) ACMG VUS g.5699062T>A g.5699051T>A LONP1 c.1661A>T p.(Asp554Val) hom - LONP1_000021 homozygous PubMed: Lenassi 2020 - - Germline ? - - - - LOVD
?/. 1 - c.1705A>C r.(?) p.(Met569Leu) - VUS g.5696749T>G - LONP1(NM_004793.4):c.1705A>C (p.(Met569Leu)) - LONP1_000033 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.1716G>A r.(?) p.(Lys572=) - likely benign g.5696738C>T - LONP1(NM_004793.3):c.1716G>A (p.K572=) - LONP1_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.1773+9G>A r.(=) p.(=) - likely benign g.5696672C>T g.5696661C>T LONP1(NM_004793.3):c.1773+9G>A - RPL36_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.1815G>A r.(?) p.(=) - likely benign g.5696341C>T - LONP1(NM_001276479.1):c.1623G>A (p.(Ser541=)) - RPL36_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.1826A>G r.(?) p.(Glu609Gly) - VUS g.5696330T>C - LONP1(NM_004793.4):c.1826A>G (p.(Glu609Gly)) - RPL36_000023 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 - c.1892C>A r.(?) p.(Ser631Tyr) - pathogenic g.5696264G>T g.5696253G>T - - LONP1_000002 - PubMed: Strauss 2015, Journal: Strauss 2015 - - Germline yes - - - - Johan den Dunnen
?/. 1 - c.1897G>C r.(?) p.(Val633Leu) - VUS g.5696181C>G - LONP1(NM_004793.4):c.1897G>C (p.(Val633Leu)) - RPL36_000022 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.1909T>G r.(?) p.(Cys637Gly) - VUS g.5696169A>C - LONP1(NM_001276479.1):c.1717T>G (p.(Cys573Gly)) - RPL36_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.1914G>A r.(?) p.(=) - likely benign g.5696164C>T - LONP1(NM_004793.4):c.1914G>A (p.(Thr638=)) - RPL36_000021 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.1921G>A r.(?) p.(Val641Ile) - likely benign g.5696157C>T - LONP1(NM_001276479.1):c.1729G>A (p.(Val577Ile)) - RPL36_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.1967T>C r.(?) p.(Ile656Thr) - VUS g.5696111A>G - LONP1(NM_004793.3):c.1967T>C (p.(Ile656Thr)) - RPL36_000019 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 2 - c.1985T>G r.(?) p.(Val662Gly) - VUS g.5696093A>C g.5696082A>C LONP1(NM_004793.3):c.1985T>G (p.V662G), LONP1(NM_004793.4):c.1985T>G (p.(Val662Gly)) - RPL36_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
+/. 4 - c.2014C>T r.(?) p.(Arg672Cys) - pathogenic (recessive) g.5694912G>A g.5694901G>A - - LONP1_000029 candidate disease gene; variant absent in 352 controls PubMed: Khan 2015, PubMed: Patel 2017 - - Germline yes - - - - Johan den Dunnen
-?/. 1 - c.2023G>C r.(?) p.(Val675Leu) - likely benign g.5694903C>G - LONP1(NM_001276479.1):c.1831G>C (p.(Val611Leu)) - RPL36_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/., ?/. 3 - c.2026C>T r.(?) p.(Pro676Ser) - pathogenic, VUS g.5694900G>A g.5694889G>A LONP1(NM_004793.4):c.2026C>T (p.P676S) - LONP1_000001 VKGL data sharing initiative Nederland PubMed: Strauss 2015, Journal: Strauss 2015 - - CLASSIFICATION record, Germline yes - - - - Johan den Dunnen, VKGL-NL_Nijmegen, VKGL-NL_VUmc
-?/. 1 - c.2038G>A r.(?) p.(Ala680Thr) - likely benign g.5694888C>T g.5694877C>T LONP1(NM_001276479.1):c.1846G>A (p.(Ala616Thr)) - RPL36_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.2045G>C r.(?) p.(Cys682Ser) - likely benign g.5694881C>G g.5694870C>G LONP1(NM_001276479.1):c.1853G>C (p.(Cys618Ser)) - RPL36_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.2060G>A r.(?) p.(Ser687Asn) - likely benign g.5694866C>T - LONP1(NM_001276479.1):c.1868G>A (p.(Ser623Asn)) - RPL36_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.2083G>A r.(?) p.(Val695Met) - VUS g.5694843C>T - LONP1(NM_004793.4):c.2083G>A (p.(Val695Met)) - RPL36_000020 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.2155-7C>T r.(=) p.(=) - likely benign g.5694570G>A g.5694559G>A LONP1(NM_001276479.1):c.1963-7C>T (p.(=)) - RPL36_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/., ./., ?/. 7 - c.2161C>G r.(?) p.(Arg721Gly) - pathogenic g.5694557G>C g.5694546G>C - - LONP1_000003 - PubMed: Strauss 2015, Journal: Strauss 2015 - - Germline yes - - - - Johan den Dunnen
+/. 1 - c.2171C>T r.(?) p.(Ala724Val) - pathogenic g.5694547G>A g.5694536G>A - - LONP1_000004 - PubMed: Strauss 2015, Journal: Strauss 2015 - - Germline yes - - - - Johan den Dunnen
+?/. 1 - c.2213C>T r.(?) p.(Thr738Met) - likely pathogenic g.5694505G>A g.5694494G>A - - LONP1_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.2254A>G r.(?) p.(Thr752Ala) - VUS g.5694464T>C g.5694453T>C LONP1(NM_004793.3):c.2254A>G (p.T752A) - RPL36_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.2256C>T r.(?) p.(Thr752=) - VUS g.5694462G>A g.5694451G>A LONP1(NM_004793.3):c.2256C>T (p.T752=) - RPL36_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 - c.2282C>T r.(?) p.(Pro761Leu) - pathogenic g.5694436G>A g.5694425G>A - - LONP1_000008 - - - - Germline yes - - - - Grace Yoon
+?/. 1 - c.2290G>A r.(?) p.(Val764Met) - likely pathogenic g.5694428C>T g.5694417C>T LONP1(NM_004793.2):c.2392G>A(p.G798S)/c.2290G>A(p.V764M) - LONP1_000025 - PubMed: Sun 2018 - - Germline/De novo (untested) ? 182 - - - LOVD
-?/. 1 - c.2314G>T r.(?) p.(Ala772Ser) - likely benign g.5694404C>A - LONP1(NM_001276479.1):c.2122G>T (p.(Ala708Ser)) - RPL36_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 - c.2392G>A r.(?) p.(Gly798Ser) - likely pathogenic g.5693709C>T g.5693698C>T LONP1(NM_004793.2):c.2392G>A(p.G798S)/c.2290G>A(p.V764M) - LONP1_000024 - PubMed: Sun 2018 - - Germline/De novo (untested) ? 182 - - - LOVD
+?/., ?/. 2 - c.2461G>A r.(?) p.(Ala821Thr) - likely pathogenic, VUS g.5693640C>T - LONP1(NM_004793.3):c.2461G>A (p.(Ala821Thr)) - RPL36_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Nijmegen
-/. 1 - c.2463C>T r.(?) p.(=) - benign g.5693638G>A - LONP1(NM_001276479.1):c.2271C>T (p.(Ala757=)) - RPL36_000017 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/., ?/. 2 - c.2485G>A r.(?) p.(Ala829Thr) - likely benign, VUS g.5693616C>T g.5693605C>T LONP1(NM_001276479.1):c.2293G>A (p.(Ala765Thr)) - RPL36_000002 VKGL data sharing initiative Nederland PubMed: Taylor 2014 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Leiden
?/. 1 - c.2603C>T r.(?) p.(Ala868Val) - VUS g.5693409G>A - LONP1(NM_004793.3):c.2603C>T (p.(Ala868Val)) - C19orf70_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.2716G>T r.(?) p.(Gly906Trp) - VUS g.5692207C>A - LONP1(NM_004793.4):c.2716G>T (p.G906W) - C19orf70_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. 1 - c.2731G>A r.(?) p.(Val911Ile) - likely benign g.5692192C>T g.5692181C>T LONP1(NM_001276479.1):c.2539G>A (p.(Val847Ile)) - C19orf70_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.2830G>C r.(?) p.(Asp944His) - likely benign g.5692093C>G - LONP1(NM_001276479.1):c.2638G>C (p.(Asp880His)) - C19orf70_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.*507G>A r.(=) p.(=) - VUS g.5691536C>T g.5691525C>T RPL36(NM_015414.3):c.229-7C>T (p.(=)) - RPL36_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.*512G>A r.(=) p.(=) - likely benign g.5691531C>T - RPL36(NM_015414.3):c.229-12C>T (p.(=)) - C19orf70_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.*3890C>T r.(=) p.(=) - likely benign g.5688153G>A g.5688142G>A HSD11B1L(NM_001267868.1):c.*197G>A (p.(=)) - C19orf70_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.*4230C>T r.(=) p.(=) - likely benign g.5687813G>A - HSD11B1L(NM_001267868.1):c.859G>A (p.(Val287Met)) - C19orf70_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
Legend   How to query  


Screenscraping/webscraping (downloading large amounts of data using scripts) is strictly prohibited.
Use our APIs to retrieve data.