350 Brain Research, 579 (1992) 350-352
© 1992 Elsevier Science Publishers B.V. All rights reserved. 0006-8993/92/$05.00
BRES 25166
Basic fibroblast growth factor binds to filamentous inclusions of
neurodegenerative diseases
George Perry, Peggy Richey, Sandra L. Siedlak, Pamela Galloway, Mitsuru Kawai and
Patrick Cras
Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, OH 44106-4901 (USA)
(Accepted 29 January 1992)
Key words: Alzheimer disease; Heparan sulfate proteoglycan; Pick disease; Parkinson disease: Progressive supranuclear palsy; Diffuse
Lewy body disease
The extracellular matrix protein heparan sulfate proteoglycans (HSPG) has been found in the neurofibrillary pathology of Alzheimer dis-
ease. This study was performed to determine if similar proteoglycans might be present in the fibrillary inclusions of other neurodegenerative
diseases. Basic fibroblast growth factor (bFGF) binding to heparinase sensitive sites was used as an assay for HSPGs. We found that the
inclusions of Pick and Parkinson diseases as well as progressive supranuclear palsy contained heparinase sensitive bFGF binding sites while
the inclusions of diffuse Lewy body disease lacked bFGF binding sites. These findings indicate that HSPG's interactions and possible role in
the formation of intraneuronal inclusions are not limited to Alzheimer disease.
Insolubility in denaturants has hindered direct deter-
mination of the composition of the filamentous neuronal
inclusions of Alzheimer disease 9'12, progressive supranu-
clear palsy (PSP) 16, Pick disease 11 and Parkinson dis-
ease z. Nevertheless intense interest in the origin of the
filamentous structures has led to a proliferation of indi-
rect approaches primarily utilizing immunochemical
methodologies. Immunochemistry has been used to show
the involvement of the neuronal cytoskeleton in the for-
mation of the filaments 1'2'9't1'16. Complementing these
studies has been the isolation of a soluble form of the
paired helical filaments characteristic of Alzheimer dis-
ease which is analyzable by direct biochemical analysis
and composed of the microtubule associated protein r 4'5.
Yet, in recent studies a heparan sulfate proteoglycan
(HSPGs) has also been reported in the intraneuronal fil-
aments of Alzheimer disease 1°A3'14 indicating that the
structural rearrangement leading to the neurofibrillary
tangles (NFT) may not be restricted to cytoskeletal ele-
ments. The present study was undertaken to determine
if HSPGs were present in the inclusions of other neuro-
degenerative diseases.
Sections from the locus coeruleus of 5 cases of idio-
pathic Parkinson disease (age 53-70 years) and from the
pons of 4 cases of PSP (age 76-83 years) were examined.
Frontal and temporal cortex of 3 cases of Pick disease
(age 62-68 years) and 3 cases of diffuse Lewy body dis-
ease (age 75-82 years) were also examined. All tissue
had been fixed in buffered formalin and embedded in
paraffin prior to sectioning (6/zm thick). HSPGs were
detected by basic fibroblast growth factor (bFGF)which
avidly binds to heparinase sensitive sites in NFT of Alz-
heimer disease 1°'13. The method, as previously described
in detail 13, involves incubating sections with 5 ~g/ml of
recombinant human bFGF (Calbio) in 20 mM EDTA,
0.1 M Tris, pH 7.6 for 2 h at 37°C followed by immu-
nostaining with a monoclonal antibody to bFGF (48.1,
Calbio). Affinity purified antiserum to r s and ubiquitin 7
were used on adjacent sections as an aid in identifying
inclusions. Immunostains were developed with the per-
oxidase-antiperoxidase procedure 15 with 3'3' diami-
nobenzidine as co-substrate.
In Pick disease, intraneuronal Pick bodies bound
bFGF (Fig. 1A), but extraneuronal round structures were
more frequently seen. The extraneuronal structures are
consistent with the persistence of Pick bodies in the ex-
tracellular space since they are the same size as Pick
bodies, occurred in the same regions, are fibrillar and
are distinct in distribution from corpora amylacea. In
PSP numerous NFT were seen in the pontine nuclei (Fig.
1C). The number of NFT of PSP binding bFGF was far
greater than recognized by the antiserum to r or ubiq-
uitin and the vast majority were extracellular.
In Parkinson disease only some of the Lewy bodies
Correspondence." G. Perry, Division of Neuropathology, Institute of Pathology, Case Western Reserve University, 2085 Adelbert Road,
Cleveland, OH 44106-4901 USA. Fax: (1)(216) 368-8964.