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Neuropathology in respiratory-related motoneurons in young Pompe (Gaa(-/-)) mice.

Publication ,  Journal Article
Turner, SMF; Hoyt, AK; ElMallah, MK; Falk, DJ; Byrne, BJ; Fuller, DD
Published in: Respir Physiol Neurobiol
June 15, 2016

Respiratory and/or lingual dysfunction are among the first motor symptoms in Pompe disease, a disorder resulting from absence or dysfunction of the lysosomal enzyme acid α-glucosidase (GAA). Here, we histologically evaluated the medulla, cervical and thoracic spinal cords in 6 weeks old asymptomatic Pompe (Gaa(-/-)) mice to determine if neuropathology in respiratory motor regions has an early onset. Periodic acid-Schiff (PAS) staining indicated glycogen accumulation was exclusively occurring in Gaa(-/-) hypoglossal, mid-cervical and upper thoracic motoneurons. Markers of DNA damage (Tunel) and ongoing apoptosis (Cleaved Caspase 3) did not co-localize with PAS staining, but were prominent in a medullary region which included the nucleus tractus solitarius, and also in the thoracic spinal dorsal horn. We conclude that respiratory-related motoneurons are particularly susceptible to GAA deficiency and that neuronal glycogen accumulation and neurodegeneration may occur independently in early stage disease. The data support early therapeutic intervention in Pompe disease.

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Published In

Respir Physiol Neurobiol

DOI

EISSN

1878-1519

Publication Date

June 15, 2016

Volume

227

Start / End Page

48 / 55

Location

Netherlands

Related Subject Headings

  • Thoracic Vertebrae
  • Spinal Cord
  • Physiology
  • Neuroimmunomodulation
  • Motor Neurons
  • Microfilament Proteins
  • Mice, Knockout
  • Mice, 129 Strain
  • Medulla Oblongata
  • Glycogen Storage Disease Type II
 

Citation

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Turner, S. M. F., Hoyt, A. K., ElMallah, M. K., Falk, D. J., Byrne, B. J., & Fuller, D. D. (2016). Neuropathology in respiratory-related motoneurons in young Pompe (Gaa(-/-)) mice. Respir Physiol Neurobiol, 227, 48–55. https://doi.org/10.1016/j.resp.2016.02.007
Turner, Sara M. F., Aaron K. Hoyt, Mai K. ElMallah, Darin J. Falk, Barry J. Byrne, and David D. Fuller. “Neuropathology in respiratory-related motoneurons in young Pompe (Gaa(-/-)) mice.Respir Physiol Neurobiol 227 (June 15, 2016): 48–55. https://doi.org/10.1016/j.resp.2016.02.007.
Turner SMF, Hoyt AK, ElMallah MK, Falk DJ, Byrne BJ, Fuller DD. Neuropathology in respiratory-related motoneurons in young Pompe (Gaa(-/-)) mice. Respir Physiol Neurobiol. 2016 Jun 15;227:48–55.
Turner, Sara M. F., et al. “Neuropathology in respiratory-related motoneurons in young Pompe (Gaa(-/-)) mice.Respir Physiol Neurobiol, vol. 227, June 2016, pp. 48–55. Pubmed, doi:10.1016/j.resp.2016.02.007.
Turner SMF, Hoyt AK, ElMallah MK, Falk DJ, Byrne BJ, Fuller DD. Neuropathology in respiratory-related motoneurons in young Pompe (Gaa(-/-)) mice. Respir Physiol Neurobiol. 2016 Jun 15;227:48–55.
Journal cover image

Published In

Respir Physiol Neurobiol

DOI

EISSN

1878-1519

Publication Date

June 15, 2016

Volume

227

Start / End Page

48 / 55

Location

Netherlands

Related Subject Headings

  • Thoracic Vertebrae
  • Spinal Cord
  • Physiology
  • Neuroimmunomodulation
  • Motor Neurons
  • Microfilament Proteins
  • Mice, Knockout
  • Mice, 129 Strain
  • Medulla Oblongata
  • Glycogen Storage Disease Type II