Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations.

Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations. Research Abstract Details 

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Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations. Abstract Text:

Keith A Josephs,Zeshan Ahmed,Omi Katsuse,Joseph F Parisi,Bradley F Boeve,David S Knopman,Ronald C Petersen,Peter Davies,Ranjan Duara,Neill R Graff-Radford,Ryan J Uitti,Rosa Rademakers,Jennifer Adamson,Matthew Baker,Michael L Hutton,Dennis W Dickson,

Frontotemporal lobar degeneration is heterogeneous; cases with tau- and synuclein-negative, ubiquitin-positive neuronal inclusions are the most common, and some have mutations in the gene for progranulin (PGRN). The purpose of this study was to determine whether there were distinctive clinical and neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with PGRN mutations. A retrospective review of medical records and semiquantitative neuropathologic analysis was performed on 18 PGRN(+) and 24 PGRN(-) cases. Clinically, PGRN(+) cases had more frequent language impairment and parkinsonism. Pathologically, PGRN(+) cases had smaller brains, more marked global atrophy, and more frontal atrophy. There was no difference in the frequency of hippocampal sclerosis. The pathology of PGRN(+) cases was relatively homogeneous, whereas PGRN(-) cases were more heterogenous. PGRN(+) cases had greater density of cortical ubiquitin-immunoreactive lesions, especially dystrophic neurites in layer II. Intranuclear inclusions were present in all PGRN(+) and 42% of PGRN(-) cases. The results suggest that frontotemporal lobar degeneration with ubiquitin-positive inclusions due to PGRN mutations has several characteristic features, including ubiquitin-immunoreactive neuritic pathology in superficial cortical layers and neuronal intranuclear inclusions. On the other hand, there is no histopathologic feature or combination of features that is pathognomonic. Neuronal intranuclear inclusions are virtually always present, but they can be detected in PGRN(-) cases.

Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations. Publishing Authors By Initials

KA Josephs,Z Ahmed,O Katsuse,JF Parisi,BF Boeve,DS Knopman,RC Petersen,P Davies,R Duara,NR Graff-Radford,RJ Uitti,R Rademakers,J Adamson,M Baker,ML Hutton,DW Dickson,

For similar proteins: ubiquitins: ubiquitin research abstracts see: proteins: ubiquitins: ubiquitin research

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Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations. Journal Published:

PUBLICATION TYPE: Research Support, Non-U.S. Gov

Journal: Journal of neuropathology and experimental neurolo

VOLUME: 66

Page Numbers: 142-51

Journal Abbreviation: J. Neuropathol. Exp. Neurol.

ISSN: 0022-3069

DAY: 3

MONTH: Feb

YEAR: 2007

Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 2985192

Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations. Keywords Mesh Terms:

KEYWORDS: Ubiquitin

MESH TERMS: metabolism

Chemical & Substance for Abstract: Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations. Information

Substance Name: Ubiquitin

Registry Number: 0

Grant and Affiliation Information for Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations.

AFFILIATION: Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.

Country: United States

AGENCY: United States NIA

GRANT: U01-AG06786

ACRONYM: AG

MEDLINETA: J Neuropathol Exp Neurol

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