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Neuropediatrics 1999; 30(1): 34-37
DOI: 10.1055/s-2007-973454
Original articles

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The Early Induction of Cyclooxygenase 2 Associated with Neurofibrillary Degeneration in Brains of Patients with Fukuyama-Type Congenital Muscular Dystrophy

Akira Oka1 , 2 , M. Itoh1 , S. Takashima1
  • 1Department of Mental Retardation and Birth Defect Research, National Institute of Neuroscience, National Center for Neurology and Psychiatry, 4-1-1 Ogawahigashimachi, Kodaira, Tokyo 187-8502, Japan,
  • 2Division of Child Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, 36-1, Nishimachi, Yonago, Tottori 683-8504, Japan
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Publikationsdatum:
12. März 2007 (online)

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Abstract

Brains of thirteen patients with Fukuyama-type congenital muscular dystrophy (FCMD) were evaluated regarding the expression of cyclooxygenase 2 (COX2), an enzyme involved in the synthetic pathway of prostaglandins and thromboxanes, as well as neurofibrillary tangles (NFT). The neuronal induction of COX2 was demonstrated with immunohistochemistry and Western blotting confirmed the up-regulation. Preceded by COX2 immunoreactivity, NFT-containing neurons appeared in the majority of FCMD patients without β-amyloid deposition or senile plaques. The hippocampus did not demonstrate neurodegeneration, while, in other areas, neurons with NFT spread in a similar manner to Alzheimer's disease. NFT-bearing neurons were concomitantly shown to be immunoreactive to COX2. The precedent induction of COX2, therefore, may be related to the formation of NFT in this genetic disorder.

Key words

Cyclooxygenase 2 - Fukuyama-type congenital muscular dystrophy - Neurofibrillary tangles - Alzheimer disease