Cognitive deficits associated with a recently reported familial neurodegenerative disease: familial encephalopathy with neuroserpin inclusion bodies.

@article{Bradshaw2001CognitiveDA,
  title={Cognitive deficits associated with a recently reported familial neurodegenerative disease: familial encephalopathy with neuroserpin inclusion bodies.},
  author={Charles B Bradshaw and Richard L. Davis and Antony E. Shrimpton and Peter D. Holohan and Charlotte Rea and D Fieglin and Patrick L. Kent and George H. Collins},
  journal={Archives of neurology},
  year={2001},
  volume={58 9},
  pages={1429-34}
}
BACKGROUND We recently discovered an autosomal dominant disease causing a progressive dementia. The disease is caused by a point mutation in the gene coding for the serine protease inhibitor (ie, serpin) neuroserpin. The mutation results in an unstable neuroserpin protein that readily aggregates into intraneuronal inclusions that we identify as Collins bodies. The bodies are distributed throughout the cerebral hemispheres but are significantly more numerous in the cortex and the substantia… CONTINUE READING

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Polymerisation underlies alpha1-antitrypsin deficiency, dementia and other serpinopathies.

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References

Publications referenced by this paper.
Showing 1-10 of 22 references

An autosomal dominant epilepsy with dementia and intraneuronal inclusion bodies [ abstract ]

MS Yerby, AE Shrimpton, RL Davis, PD Holohan, Shaw E-M
2000

An autosomal dominant epilepsy with dementia and intraneuronal inclusion bodies [abstract]. Neurology. 2000;54(suppl 3):A356

MS Yerby, AE Shrimpton, RL Davis, PD Holohan, E-M. Shaw
2000

Bond J

UCLA Statistics Department
Power calculator for 2 samples with unequal variances. Available at: http://ebook.stat.ucla.edu/calculators/powercalc/. Accessed February 14, • 2000
View 2 Excerpts

Collins bodies and atypical myoclonus bodies: the neuroserpin connection [abstract

RL Davis, M Yerby, C Shaw
Brain Pathol • 2000

Familial encephalopathy with neuroserpin inclusion bodies.

The American journal of pathology • 1999
View 2 Excerpts

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