SCN5A Variant E418G

Summary of observed carriers, functional annotations, and structural context for SCN5A E418G. Data combine curated literature, international cohorts, and gnomAD observations.

Estimated LQT3 penetrance

8%

0/10 effective observations

Estimated BrS1 penetrance

11%

1/10 effective observations

Total carriers

0

0 BrS1 · 0 LQT3 · 0 unaffected

E418G has not been reported in gnomAD. This residue resides in a Non_Hotspot region for Brugada syndrome and a Non_Hotspot region for LQT3.

Variant features alone are equivalent to phenotyping 1 individuals for Brugada syndrome and 0 individuals for LQT3.

In silico predictors

Variant-level computational predictors.
PROVEAN PolyPhen-2 BLAST-PSSM REVEL Penetrance Density BrS (%) Penetrance Density LQT3 (%)
NA NA NA 0.875 6 6

PROVEAN scores below -2 suggest deleterious impact. REVEL scores above 0.5–0.75 are often interpreted as likely pathogenic. PolyPhen-2 scores above 0.85 are typically pathogenic. Penetrance density summarises neighbouring residue risk (Kroncke et al. 2019).

Reported carrier data

Observed carriers by publication or cohort.
Source Year Carriers Unaffected LQT3 BrS1 Other Other Disease
Literature, cohort, and gnomAD 0 0 0 0
Variant features alone 15 14 0 1

Totals may differ from individual publications due to duplicate patients removed during curation.

Nearby variants

Neighbouring residues within 15 Ångström provide structural context. Variants listed in the right-most column have been observed clinically or in gnomAD.

Previously observed variants near E418G.
Neighbour residue Distance (Å) Observed variants
414 6 M414V,
939 14 L939F,
937 14
1778 14
249 9 K249X,
247 14 V247L, V247L,
240 13 V240M,
1777 15 V1777M, V1777L, V1777L,
418 0 E418K,
250 14
409 14 L409V, L409P,
237 13
417 6
933 13
246 10
1779 10 T1779M,
412 10 V412D,
245 8 Q245K,
1776 12
244 12
415 5 A415T,
940 13 S940N,
420 8
248 13
241 10
419 5 Q419X,
423 9
239 12 I239V, I239V ,
410 12 A410V,
1780 10 E1780G
242 9 A242D,
416 8 Y416C,
413 9 A413T, A413E,
1783 11
936 12
238 11
422 7
1775 13 F1775V, p.F1775LfsX15,
421 7
411 10 V411M,
243 12
1782 13