Review Article
Cardiac Ion Channelopathies and the Sudden Infant Death Syndrome
Table 6
Prevalence of cardiac ion channel-related mutations in SIDS cohorts‡.
| | | Number of SIDS cases | | Gene | Total | with mutation | Functionally significant |
| related genes | | | | SCN5A | 542 | 28 (5.2%) | 26 (4.8%) | SCN1B | 292 | 0 | 0 | SCN1Bb | 292 | 1 (0.3%) | 1 (0.3%) | SCN2B | 292 | 0 | 0 | SCN3B | 292 | 2 (0.7%) | 2 (0.7%) | SCN4B | 292 | 1 (0.3%) | 1 (0.3%) | CAV3 | 335 | 6 (1.8%) | 5 (1.5%) | GPD1-L | 221 | 2 (0.9%) | 2 (0.9%) | SNTA1 | 292 | 8 (2.7%) | 3 (1.0%) | related genes | | | | KCND3 | 292 | 1 (0.3%) | 0 | related genes | | | | KCNQ1 | 409 | 9 (2.2%) | 4 (1.0%) | KCNE1 | 367 | 1 (0.3%) | 0 | related genes | | | | KCNH2 | 409 | 8 (2.0%) | 2 (0.5%) | KCNE2 | 367 | 2 (0.5%) | 1 (0.3%) | related genes | | | | KCNJ2 | 201 | 0 | 0 | related genes | | | | KCNJ8 | 292 | 2 (0.7%) | 2 (0.7%) | Intracellular calcium cycling related genes | | | | RYR2 | 134 | 2 (1.5%) | 2 (1.5%) | Gap junctional current related genes | | | | GJA1 | 292 | 2 (0.7%) | 1 (0.3%) |
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: ATP-sensitive potassium current; : inward rectifier potassium current; : rapid delayed rectifier potassium current; : slow delayed rectifier potassium current; : fast sodium current; : transient outward potassium current. ‡Based on data listed in Table 5.
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