Improved Growth Patterns in Cystic Fibrosis Mice after Loss of Histone Deacetylase 6

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Sharon M Rymut1, Deborah A Corey1, Dana M Valerio1

  • 1Department of Pediatrics, Case Western Reserve University, Cleveland, Ohio, USA.

Scientific Reports
|June 18, 2017
PubMed

Insights

Targeting histone deacetylase-6 (HDAC6) improved growth in cystic fibrosis (CF) mouse models. This suggests HDAC6 inhibition is a potential therapeutic strategy for CF-related growth failure.

Area of Science:

  • Biomedical research
  • Genetics
  • Molecular biology

Background:

  • Growth failure is a significant complication in cystic fibrosis (CF) patients, correlating with adverse disease outcomes.
  • Reduced tubulin acetylation has been observed in CF models, and this defect is reversible by inhibiting histone deacetylase-6 (HDAC6).

Purpose of the Study:

  • To investigate whether the loss of HDAC6 can ameliorate the growth phenotype in a mouse model of cystic fibrosis.
  • To explore the potential of HDAC6 as a therapeutic target for CF-related growth deficits.

Main Methods:

  • Generation of F508del/Hdac6 (CF/HDA) mice by crossing Hdac6 knockout mice with F508del (CF) mice.
  • Analysis of growth parameters, fat deposits, survival rates, and bioelectric measurements in the generated mouse models.

Main Results:

  • CF/HDA mice exhibited significant improvements in body length and weight compared to CF mice, despite no restoration of CFTR function.
  • Increased insulin-like growth factor 1 (Igf1) levels were observed, potentially explaining the enhanced length and fertility.
  • Weight gain in CF/HDA mice was associated with increased fat deposits, possibly due to enhanced adipocyte differentiation.

Conclusions:

  • Inhibition of HDAC6 can improve growth deficits associated with cystic fibrosis.
  • HDAC6 represents a promising therapeutic target for addressing growth failure in CF patients.

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