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Absence of Ndn, encoding the Prader-Willi syndrome-deleted gene necdin, results in congenital deficiency of central respiratory drive in neonatal mice

  1. Author:
    Ren, J.
    Lee, S.
    Pagliardini, S.
    Gerard, M.
    Stewart, C. L.
    Greer, J. J.
    Wevrick, R.

  2. Author Address

    Univ Alberta, Dept Physiol, Ctr Neurosci, Edmonton, AB T6G 2M7, Canada Univ Alberta, Dept Physiol, Ctr Neurosci, Edmonton, AB T6G 2M7, Canada Univ Alberta, Dept Med Genet, Edmonton, AB T6G 2M7, Canada CEA Saclay, Div Biochem & Mol Genet, F-91191 Gif Sur Yvette, France NCI, Lab Canc & Dev Biol, Frederick Canc Res & Dev Ctr, Frederick, MD 21702 USA Greer JJ Univ Alberta, Dept Physiol, Ctr Neurosci, Edmonton, AB T6G 2M7, Canada
    1. Year: 2003
  2. Journal: Journal of Neuroscience
    1. 23
    2. 5
    3. Pages: 1569-1573
  4. Type of Article: Article
  1. Abstract:

    necdin (Ndn) is one of a cluster of genes deleted in the neurodevelopmental disorder Prader-Willi syndrome. necdin is upregulated during neuronal differentiation and is thought to play a role in cell cycle arrest in terminally differentiated neurons. Most necdin-deficient Ndn(tm2Stw) mutant pups carrying a targeted replacement of Ndn with a lacZ reporter gene die in the neonatal period of apparent respiratory insufficiency. We now demonstrate that the defect can be explained by abnormal neuronal activity within the putative respiratory rhythm- generating center, the pre-Botzinger complex. Specifically, the rhythm is unstable with prolonged periods of depression of respiratory rhythmogenesis. These observations suggest that the developing respiratory center is particularly sensitive to loss of necdin activity and may reflect abnormalities of respiratory rhythm-generating neurons or conditioning neuromodulatory drive. We propose that necdin deficiency maycontribute to observed respiratory abnormalities in individuals with Prader- Willi syndrome through a similar suppression of central respiratory drive.

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