Asymmetric pathology in primary progressive aphasia with progranulin mutations and TDP inclusions.

ABSTRACT:

Objective

To investigate quantitative regional distribution and hemispheric asymmetry of TDP-43 (TAR DNA-binding protein 43) inclusions, neurons, and activated microglia in primary progressive aphasia (PPA) with progranulin (GRN) mutations, and to determine concordance between distribution of pathology, clinical phenotype, and known atrophy patterns.

Methods

Antibodies to phospho-TDP-43, NeuN (neuronal nuclei), and HLA-DR were used to visualize inclusions, neurons, and activated microglia in paraffin-embedded tissue sections from 4 participants with PPA: 2 of the agrammatic and 2 of the logopenic subtype. Unbiased stereological counting techniques were used for quantitation of immunoreactive profiles in language- and memory-related cortical areas bilaterally. Patterns of pat

SUBMITTER: Kim G 

PROVIDER: S-EPMC4762414 | biostudies-literature | 2016 Feb

REPOSITORIES: biostudies-literature