Start Date

2021

Description

  • Hypertrophic cardiomyopathy (HCM) is a highly morbid myocardial disease occurring in 1:200 people in the general population
  • Most common genetic cause of HCM are mutations in MYBPC3, encoding for MyBP-C, a critical protein for regulating cardiac muscle contractility
  • Limited treatments available primarily focus on symptomatic management and cannot reverse cardiomyopathy
  • Haploinsufficiency - one allele is unable to produce sufficient protein to maintain normal cardiac function
  • Adeno-associated virus (AAV) mediated gene replacement therapy recently emerged as a therapeutic option for genetic diseases due to loss of function variants causing haploinsufficiency

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Genetics Commons

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Jan 1st, 12:00 AM

Efficacy of AAV-mediated gene replacement therapy for MYBPC3- associated HCM in iPSCMs

  • Hypertrophic cardiomyopathy (HCM) is a highly morbid myocardial disease occurring in 1:200 people in the general population
  • Most common genetic cause of HCM are mutations in MYBPC3, encoding for MyBP-C, a critical protein for regulating cardiac muscle contractility
  • Limited treatments available primarily focus on symptomatic management and cannot reverse cardiomyopathy
  • Haploinsufficiency - one allele is unable to produce sufficient protein to maintain normal cardiac function
  • Adeno-associated virus (AAV) mediated gene replacement therapy recently emerged as a therapeutic option for genetic diseases due to loss of function variants causing haploinsufficiency