Perry, Elaine K

Publication Detail

The publication detail shows the title, authors (with indicators showing other profiled authors), information on the publishing organization, abstract and a link to the article in PubMed. This abstract is what is used to create the fingerprint of the publication. If any grants are referenced by the publication, they will be listed here as well.

Hereditary diffuse leukoencephalopathy with spheroids: ultrastructural and immunoelectron microscopic studies.

Wen-Lang Lin; Zbigniew K Wszolek; Dennis W Dickson (Profiled Author: Dickson, Dennis)

Department of Neuroscience, Mayo Clinic, Jacksonville, Florida 32224, USA.
International journal of clinical and experimental pathology 2010;3(7):665-74.

Hereditary diffuse leukoencephalopathy with spheroids (HDLS) is a rare autosomal dominant disorder characterized by cerebral white matter degeneration with myelin loss and axonal swellings (spheroids) leading to progressive cognitive and motor dysfunction. Histopathology of HDLS has been well characterized, but ultrastructural details are lacking. Here we report ultrastructural and immunoelectron microscopic characterization of spheroids and capillary basal lamina in white matter of HDLS brains. Spheroids had thin or discontinuous or no myelin sheaths. They contained various combinations of aggregated neurofilaments (NF), cytoplasmic organelles, dense bodies, and laminated figures. Aggregated filaments labeled with antibodies to phosphorylated NF (pNF), non-pNF and amyloid precursor protein. The gliotic white matter had many reactive astrocytes, and lipid-laden macrophages with membranous and fingerprint-like bodies. The basal laminas (BL) of many capillaries were dilated, and the enlarged space was heavily deposited with banded collagen type I and III. Some BL had focal thickenings and duplications. Fibronectin, not collagen IV, was found associated with banded collagen. The various types of axonal spheroids and changes in capillary basal lamina have not been emphasized previously. It remains to be determined if they are a reactive process or a primary mechanism of white matter degeneration in HDLS.

4 Originating Grant

• 1.

  Hardy, John A

  GENETICS AND MOLECULAR BIOLOGY OF PARKINSONISM

  30 September 1999 - 31 July 2004

  NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE

  Total Funding: $ 3,886,270

• 2.

  Dickson, Dennis William

  Genetics and Molecular Biology of Parkinsonism

  30 September 1999 - 31 August 2009

  NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE

  Total Funding: $ 8,904,777

• 3.

  Dickson, Dennis W

  GENETICS AND MOLECULAR BIOLOGY OF PARKINSONISM

  30 September 1999 - 29 September 2004

  NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE

  Total Funding: $ 2,454,521

• 4.

  PETERSEN, RONALD C

  Mayo Alzheimer's Disease Research Center

  1 May 1999 - 30 April 2014

  NATIONAL INSTITUTE ON AGING

  Total Funding: $ 25,743,859

Scientific Context

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