Reduced Nerve Growth Factor in Rett Syndrome Postmortem Brain Tissue

To determine whether reduced nerve growth factor (NGF) and/or its high affinity receptor, trkA, play a role in the pathophysiology of Rett syndrome (RS), we used immunohistochemistry in paraffin-embedded human autopsy brain tissue to quantify NGF and trkA levels within the frontal cortex of 9 RS fem...

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Bibliographic Details
Published in:Journal of neuropathology and experimental neurology 2000-10, Vol.59 (10), p.889-895
Main Authors: LIPANI, JOHN D, BHATTACHARJEE, MEENA B, COREY, DAVID M, LEE, DEBORAH A
Format: Article
Language:English
Subjects:
Adult
Antibody Specificity
Astrocytes - chemistry
Astrocytes - pathology
Biological and medical sciences
Brain
Brain - metabolism
Brain - pathology
Brain Chemistry
Child
Child, Preschool
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Female
Glial Fibrillary Acidic Protein - analysis
Gliosis - metabolism
Health aspects
Humans
Medical sciences
Middle Aged
Nerve growth factor
Nerve Growth Factor - analysis
Nerve Growth Factor - immunology
Nerve Growth Factor - metabolism
Neurology
Receptor, trkA - analysis
Receptor, trkA - immunology
Rett syndrome
Rett Syndrome - metabolism
Rett Syndrome - pathology
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Summary:To determine whether reduced nerve growth factor (NGF) and/or its high affinity receptor, trkA, play a role in the pathophysiology of Rett syndrome (RS), we used immunohistochemistry in paraffin-embedded human autopsy brain tissue to quantify NGF and trkA levels within the frontal cortex of 9 RS females and 10 female controls of similar age. The results showed a significant reduction of NGF expression in RS patients (p < 0.001). Specifically, all RS brains exhibited NGF levels at or below the minimum level observed in controls. In 3 RS brains there was no NGF detected. TrkA expression was also reduced in the RS group (p = 0.035). Interestingly, the expression of NGF in the RS group was significantly related to the presence of cortical astrogliosis (r = 0.91) as indicated by immunostaining for glial fibrillary acidic protein (GFAP). This suggests that while the signals for NGF production during injury remain intact, the critical developmental signals required for early NGF production are impaired.
ISSN:0022-3069
1554-6578
DOI:10.1093/jnen/59.10.889