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Preclinical Advances of Therapies for Laminopathies.

Louise BenarrochEnzo CohenAntonio AtalaiaRabah Ben YaouGisèle BonneAnne T Bertrand
Published in: Journal of clinical medicine (2021)
Laminopathies are a group of rare disorders due to mutation in LMNA gene. Depending on the mutation, they may affect striated muscles, adipose tissues, nerves or are multisystemic with various accelerated ageing syndromes. Although the diverse pathomechanisms responsible for laminopathies are not fully understood, several therapeutic approaches have been evaluated in patient cells or animal models, ranging from gene therapies to cell and drug therapies. This review is focused on these therapies with a strong focus on striated muscle laminopathies and premature ageing syndromes.
Keyphrases
  • copy number
  • induced apoptosis
  • genome wide
  • gene expression
  • cell therapy
  • skeletal muscle
  • type diabetes
  • insulin resistance
  • metabolic syndrome
  • oxidative stress
  • cell death
  • transcription factor
  • drug induced
  • pi k akt