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Progranulin (PGRN) expression in ALS: an immunohistochemical study.

D Irwin¹, C F Lippa, A Rosso

¹Department of Neurology, Drexel University College of Medicine, Philadelphia, PA 19102, USA.

Journal of the Neurological Sciences

|October 14, 2008

Summary

Progranulin (PGRN) is elevated in affected brain regions of amyotrophic lateral sclerosis (ALS) patients, suggesting a role in motor neuron degeneration and cognitive decline within the ALS-Frontotemporal Dementia spectrum.

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Area of Science:

Neuroscience
Neuropathology
Genetics

Background:

Mutations in progranulin (PGRN) are linked to frontotemporal dementia with ubiquitin-positive inclusions (FTLD-U).
TDP-43 is a component of ubiquitinated inclusions in FTLD-U and ALS with dementia (ALS-D).
Shared pathology between ALS and FTLD-U warrants investigation into common mechanisms.

Purpose of the Study:

To investigate PGRN immunoexpression in post-mortem tissues from ALS patients.
To explore the potential association between PGRN and the neuropathology observed in ALS and FTLD-U.

Main Methods:

Immunohistochemical analysis of brain and spinal cord sections from eight ALS patients and eighteen age-matched controls.
Staining with anti-PGRN antibodies to assess PGRN expression patterns.

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Main Results:

Increased PGRN staining was observed in motor tracts with vacuolar degeneration and glial cells in ALS spinal cord and brainstem sections compared to controls.
Variable PGRN expression was noted in upper motor neurons and reactive glia in ALS motor cortex.
The ALS-dementia case exhibited PGRN immunoexpression in non-motor cortical areas, unlike control tissues.

Conclusions:

A pattern of increased PGRN expression in areas of active degeneration in ALS has been identified.
The findings suggest a potential role for PGRN in the variable motor and cognitive deficits seen in the ALS-FTD spectrum.
Further research is needed to elucidate the precise role of PGRN in ALS pathogenesis.