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Aging-associated neuropathology in Werner syndrome
J B Leverenz1, C E Yu, G D Schellenberg
1Department of Neurology, University of Washington School of Medicine, Seattle 98195-6465, USA.
Acta Neuropathologica
|October 31, 1998
Summary
Werner syndrome (WS), a disorder of accelerated aging, appears to affect the central nervous system. Our study found amyloid beta deposition and neurofibrillary pathology in WS brains, suggesting a link to aging neuropathology.
Area of Science:
- Neuropathology
- Gerontology
- Genetics
Background:
- Werner syndrome (WS) is an autosomal recessive disorder characterized by accelerated systemic aging.
- It is generally believed that WS does not affect the central nervous system (CNS).
Purpose of the Study:
- To investigate the presence and extent of neuropathological hallmarks of aging in individuals with Werner syndrome.
- To determine if the WRN gene mutation associated with WS plays a role in CNS aging and related diseases.
Main Methods:
- Examination of brain tissue from two WS cases using Bielschowsky silver stain.
- Immunohistochemistry was employed to detect amyloid beta peptide (A beta) and hyperphosphorylated tau.
Main Results:
- Extensive A beta deposition was observed in the frontal and temporal lobes of the oldest WS case (57 years).
- Restricted neurofibrillary pathology was identified in the medial temporal lobe of both WS cases.
- A beta deposition in the medial temporal lobe of the oldest case was more severe than in control cases.
Conclusions:
- The findings suggest that accelerated aging in Werner syndrome can involve the central nervous system.
- The WRN gene mutation may be implicated in the neuropathology of aging and aging-associated diseases.