SCN5A Variant D1792Y

Summary of observed carriers, functional annotations, and structural context for SCN5A D1792Y. Data combine curated literature, international cohorts, and gnomAD observations.

Estimated LQT3 penetrance

11%

0/11 effective observations

Estimated BrS1 penetrance

10%

1/11 effective observations

Total carriers

1

0 BrS1 · 0 LQT3 · 1 unaffected

D1792Y is present in 1 alleles in gnomAD. This residue resides in a Non_Hotspot region for Brugada syndrome and a Mild_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 (%)
-5.53 1 -4.11 0.904 2 16

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 1 1 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 D1792Y.
Neighbour residue Distance (Å) Observed variants
1850 13 C1850S, C1850S,
1785 14
1794 8
1778 14
1853 15 I1853V
1795 7 Y1795N, Y1795H, p.Y1795_E1796insD, Y1795C,
1635 13
1652 14 M1652T, M1652R,
1634 15 L1634P,
1824 14 P1824A,
1820 14 A1820T, A1820V,
1504 8 K1504E,
1641 12
1851 14 M1851V, M1851I, M1851I, M1851I,
1501 12 p.L1501_K1505del, L1501V,
1857 15
1639 14 G1639A,
1507 9 p.Q1507_P1509del,
1505 12 p.K1505_Q1507del, K1505N, K1505N,
1858 13
1509 15 P1509T,
1787 8 S1787N,
1786 10 c.5356_5357delCT, L1786Q, L1786R,
1648 10
1649 14 A1649V,
1821 12
1644 10 R1644C, R1644H, R1644L,
1798 12 W1798X,
1496 15
1854 11
1825 11 L1825P,
1797 10 I1797V,
1800 13
1793 6 M1793K,
1781 14 E1781G, E1781D, E1781D,
1789 5
1817 14
1645 12 T1645M,
1796 6
1799 11
1788 5 c.5361_5364delTGAG,
1638 9 R1638X, R1638Q,
1651 12
1500 10 p.K1500del,
1791 6
1637 13
1792 0 D1792N, D1792Y, D1792V,
1823 15 E1823K, p.E1823HfsX10,
1508 14
1502 13 G1502S, G1502A,
1497 13
1790 7 D1790N, D1790G, p.D1790del,
1506 11 P1506T, P1506S,
1647 14
1503 11 S1503Y,
1822 11 c.5464_5467delTCTG, c.5464-5467delTCTG,
1782 15