TABLE 2

Main causative genes of hypertrophic cardiomyopathy (HCM)

Sarcomeric proteinsGeneGene prevalence in HCM probands
Myosin-binding protein CMYPBC315%
Beta myosin heavy chainMYH715%
Cardiac troponin TTNNT2  7%
Alpha-tropomyosinTPM1  7%
Regulatory myosin light chainMYL2< 5%
Essential myosin light chainMYL3< 5%
Cardiac troponin ITNNI3< 5%
Nonsarcomeric proteinsGeneInheritanceAssociated phenotype
TransthyretinTTRDominant; 1%–10%Amyloidosis
Lysosome-associated membrane glycoprotein 2LAMP2X-linked; rareDanon disease
Alpha-galactosidase AGLAX-linked; 1%–2% of malesFabry disease
Lysosomal alpha-glucosidaseGAARecessive; rarePompe disease
FrataxinFXNRecessive; rareFriedrich ataxia
  • Based on information in reference 3.