Unique variants in the ECHS1 gene

Information The variants shown are described using the NM_004092.3 transcript reference sequence.

33 entries on 1 page. Showing entries 1 - 33.
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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 - c.-194098_*12025561dup - - - pathogenic g.123150811_135380935dup - - - ACADSB_000015 increased gene dosage PubMed: DDDS 2015, Journal: DDDS 2015 - - De novo - - - - - Johan den Dunnen
+/. 1 - c.2T>G r.? p.0? - pathogenic (recessive) g.135186836A>C g.133373332A>C - - ECHS1_000018 - PubMed: Sakai 2015 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.5C>T r.(?) p.(Ala2Val) - pathogenic (recessive) g.135186833G>A g.133373329G>A - - ECHS1_000019 - PubMed: Sakai 2015 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.32T>C r.(?) p.(Val11Ala) - likely benign g.135186806A>G - ECHS1(NM_004092.4):c.32T>C (p.V11A) - ECHS1_000032 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. 1 - c.88+5G>A r.spl? p.? - pathogenic g.135186745C>T - ECHS1(NM_004092.4):c.88+5G>A - ECHS1_000033 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.97T>C r.(?) p.(Phe33Leu) - VUS g.135184253A>G - - - ECHS1_000026 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 1 - c.160C>A r.(?) p.(Arg54Ser) - likely pathogenic g.135184190G>T g.133370686G>T - - ECHS1_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 1 - c.176A>G r.(?) p.(Asn59Ser) - pathogenic (recessive) g.135184174T>C g.133370670T>C - - ECHS1_000020 - PubMed: Yamada 2015 - - Germline yes - - - - Johan den Dunnen
-/., -?/. 2 - c.224C>T r.(?) p.(Thr75Ile) - benign, likely benign g.135184126G>A g.133370622G>A ECHS1(NM_004092.4):c.224C>T (p.T75I) - ECHS1_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen, VKGL-NL_Nijmegen
?/. 1 - c.229G>C r.(?) p.(Glu77Gln) - VUS g.135184121C>G - ECHS1(NM_004092.4):c.229G>C (p.E77Q) - ECHS1_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.243C>T r.(?) p.(Ala81=) - likely benign g.135184107G>A g.133370603G>A ECHS1(NM_004092.3):c.243C>T (p.A81=) - ECHS1_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.244G>A r.(?) p.(Val82Met) - VUS g.135184106C>T g.133370602C>T - - ECHS1_000028 - - - - Germline - - - - - Min Peng
+/. 1 - c.310C>G r.(?) p.(Gln104Glu) - pathogenic (recessive) g.135183512G>C g.135183512G>C - - ECHS1_000023 - PubMed: Wu 2020 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.404A>G r.(?) p.(Asn135Ser) - VUS g.135183418T>C - ECHS1(NM_004092.4):c.404A>G (p.(Asn135Ser)) - ECHS1_000031 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 - c.413C>T r.(?) p.(Ala138Val) - pathogenic (recessive) g.135183409G>A g.133369905G>A - - ECHS1_000021 - PubMed: Yamada 2015 - - Germline yes - - - - Johan den Dunnen
+/. 1 3i c.414+3G>C r.[376_414del,=] p.[Val126_Ala138del,=] - pathogenic (recessive) g.135183405C>G g.133369901C>G - - ECHS1_000015 mRNA with 0.80 deleted product PubMed: Peters 2014 - - Germline yes - - - - Johan den Dunnen
+/. 1 3i c.414+5G>A r.376_414del p.Val126_Ala138del - pathogenic (recessive) g.135183403C>T g.133369899C>T - - ECHS1_000022 - PubMed: Wu 2020 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.473C>A r.473c>a p.Ala158Asp - pathogenic (recessive) g.135182468G>T g.133368964G>T - - ECHS1_000014 - PubMed: Peters 2014 - - Germline yes - - - - Johan den Dunnen
+/. 1 - c.476A>G r.(?) p.(Gln159Arg) - pathogenic g.135182465T>C g.133368961T>C - - ECHS1_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 8 - c.489G>A r.(?) p.(=), p.(Pro163=) - likely benign (!), pathogenic, VUS g.135182452C>T g.133368948C>T - - ECHS1_000017 VKGL data sharing initiative Nederland, 1 more item PubMed: Abdenur 2020, Journal: Abdenur 2020 - rs140410716 CLASSIFICATION record, Germline -, yes 0.17 (439 alleles in 1284 Samoan participants) TOPMED data, 2 more items - - - Mariella Simon, VKGL-NL_Nijmegen, Min Peng
+/., ?/. 2 - c.518C>T r.(?) p.(Ala173Val) - pathogenic (recessive), VUS g.135180494G>A g.133366990G>A - - ECHS1_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record, Germline - - - - - VKGL-NL_Nijmegen, Min Peng
?/. 1 - c.519G>A r.(?) p.(=) - VUS g.135180493C>T - ECHS1(NM_004092.4):c.519G>A (p.(Ala173=)) - ECHS1_000030 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 2 - c.563C>T r.(?) p.(Ala188Val) - VUS g.135180449G>A - ECHS1(NM_004092.3):c.563C>T (p.A188V), ECHS1(NM_004092.4):c.563C>T (p.A188V) - ECHS1_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_VUmc
?/. 1 - c.571A>G r.(?) p.(Met191Val) - VUS g.135180441T>C g.133366937T>C - - ECHS1_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. 1 - c.582T>C r.(?) p.(Thr194=) - benign g.135180430A>G g.133366926A>G - - ECHS1_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.583G>A r.(?) p.(Gly195Ser) - VUS g.135180429C>T g.133366925C>T ECHS1(NM_004092.3):c.583G>A (p.G195S) - ECHS1_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 2 - c.664G>A r.(?) p.(Ala222Thr) - VUS g.135179555C>T - ECHS1(NM_004092.4):c.664G>A (p.(Ala222Thr), p.A222T) - ECHS1_000024 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Groningen
+/. 1 - c.759_762del r.(?) p.(Gly255Valfs*21) - likely pathogenic (recessive) g.135178207_135178210del g.133364703_133364706del - - ECHS1_000027 - - - - Germline - - - - - Min Peng
+?/. 1 7i c.807+5G>A r.767_807del p.Ser256Argfs*2 ACMG likely pathogenic (recessive) g.135178157C>T g.133364653C>T - - ECHS1_000029 - - - - Germline yes - - - - Xiaomei Luo
+/. 1 - c.817A>G r.(?) p.(Lys273Glu) - pathogenic g.135176428T>C g.133362924T>C - - ECHS1_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 1 - c.830C>T r.(?) p.(Thr277Ile) - likely pathogenic g.135176415G>A - - - ECHS1_000025 - - - rs775650144 Unknown - - - - - MobiDetails
+/. 5 - c.832G>A r.(?) p.(Ala278Thr) - pathogenic (recessive) g.135176413C>T g.133362909C>T - - ECHS1_000016 Decreased ECHS1 proteinstability confirmed via Western blot, results in unstable ECHS1 protein, 1 more item PubMed: Abdenur 2020, Journal: Abdenur 2020 - - Germline yes - - - - Mariella Simon
?/. 1 - c.833C>T r.(?) p.(Ala278Val) - VUS g.135176412G>A g.133362908G>A - - ECHS1_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
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