Carboxylic acid derivatives of histone deacetylase inhibitors induce full length SMN2 transcripts: a promising target for spinal muscular atrophy therapeutics.


Dayangac-Erden D. , Bora-Tatar G. , DALKARA S. , DEMİR A. G. , Erdem-Yurter H.

Archives of medical science : AMS, vol.7, no.2, pp.230-4, 2011 (Journal Indexed in SCI Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 7 Issue: 2
  • Publication Date: 2011
  • Doi Number: 10.5114/aoms.2011.22072
  • Title of Journal : Archives of medical science : AMS
  • Page Numbers: pp.230-4
  • Keywords: histone deacetylase inhibitors, SMN2 gene, therapy, SURVIVAL MOTOR-NEURON, FACTOR-KAPPA-B, DETERMINING GENE, IN-VITRO, INCREASES, CURCUMIN, CELLS, EXPRESSION, PROTEIN, MECHANISMS

Abstract

Introduction: Proximal spinal muscular atrophy (SMA) is a common autosomal recessively inherited neuromuscular disorder. It is caused by homozygous absence of the survival motor neuron 1 (SMN1) gene. SMN2, which modulates the severity of the disease, represents a major target for therapy. The aim of this study was to investigate whether SMN2 expression can be increased by caffeic acid, chlorogenic acid and curcumin, which are designed by modifications of the carboxylic acid class of histone deacetylase (HDAC) inhibitors.