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Lack of Pur-alpha alters postnatal brain development and causes megalencephaly

Suvi Hokkanen1, Heidi M Feldmann, Haiyan Ding

  • 1Center of Neuropathology and Prion Research, Ludwig-Maximilians-Universität and German Center for Neurodegenerative Diseases (DZNE) Munich, 81377 Munich, Germany.

Human Molecular Genetics
|October 20, 2011
PubMed

Insights

Pur-alpha (Purα) deficiency prolongs neuronal precursor cell proliferation in mice, leading to tremors, megalencephaly, and neurofilament abnormalities. This Purα(-/-) mouse model aids in understanding neurodegenerative mechanisms.

Area of Science:

  • Neuroscience
  • Developmental Biology
  • Genetics

Background:

  • Pur-alpha (Purα) is crucial for cellular processes like proliferation and transcriptional regulation.
  • Its specific role in brain development and mature function remains incompletely understood.

Purpose of the Study:

  • To investigate the function of Pur-alpha in postnatal brain development using a Purα-deficient mouse model.
  • To elucidate the impact of Purα deficiency on neuronal precursor cell proliferation, neuronal development, and associated neurological functions.

Main Methods:

  • Generation and analysis of Purα-deficient (Purα(-/-)) mice up to six months of age.
  • Assessment of neuronal precursor cell proliferation in the hippocampus and cerebellum.
  • Evaluation of MAP2 protein expression and distribution.
  • Clinical and histopathological examination for neurological deficits, including tremor, megalencephaly, axonal swelling, and neurofilament phosphorylation.

Main Results:

  • Purα(-/-) mice exhibited prolonged postnatal proliferation of neuronal precursor cells without altering mature neuron numbers.
  • Alterations in the expression and distribution of the dendritic protein MAP2 were observed.
  • Purα(-/-) mice developed persistent tremors from 2 weeks of age, megalencephaly, axonal swellings, and hyperphosphorylated neurofilaments in adulthood.

Conclusions:

  • Pur-alpha is essential for regulating neuronal precursor cell proliferation during postnatal brain development.
  • Purα plays a role in controlling the expression and distribution of key axonal and dendritic proteins.
  • The Purα(-/-) mouse model offers valuable insights into Purα's role in neurodevelopment and neurodegenerative conditions like fragile X tremor/ataxia syndrome.

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