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. 2015 Jun 18;130(4):537–555. doi: 10.1007/s00401-015-1450-z

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© The Author(s) 2015

Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.

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Fig. 5 — Regional distribution pattern of neuronal poly-GA, poly-GR and poly-GP inclusion pathology in cases with C9orf72 mutation. Semi-quantitative immunohistochemical analyses for poly-GA (a), poly-GR (b) and poly-GP (c) neuronal cytoplasmic inclusions (NCI), neuronal intranuclear inclusions (NII) and dystrophic neurites (DN) in representative cortical, hippocampal, subcortical, brain stem, cerebellar and spinal cord areas of five C9orf72 mutation patients reveal a predominance of poly-GA aggregates for all types of aggregates in all areas. Highest densities of poly-GA, poly-GR and poly-GP aggregates are seen in cortical areas, hippocampus, amygdaloid nuclei, thalamus and cerebellum. Note that poly-GR-positive DNs are rarely found outside the hippocampus. Categories for semi-quantitative analysis (none, few, some, many, abundant) are explained in detail in the “Materials and methods” section. Acc accumbens nucleus, Am amygdaloid nuclei, BC basal nucleus of Meynert compact part, CA3/4 cornu ammonis fields 3/4, CBLgl cerebellar granular cell layer, CBLml cerebellar molecular cell layer, CBLpcl cerebellar Purkinje cell layer, CCtx cortex of cingulate gyrus, Cd caudate nucleus, DG dentate gyrus, DRN dorsal raphe nuclei, FCtx frontal cortex, IO inferior olive, LC locus coeruleus, MCtx primary motor cortex, N XII hypoglossal nucleus, OCtx occipital cortex, Pa pallidum, PCtx parietal cortex, PN pontine nuclei of pons, preEnt lamina principalis externa of entorhinal cortex, priEnt lamina principalis interna of entorhinal cortex, Pu putamen, SCAc anterior horn of cervical spinal cord, SCAl anterior horn of lumbar spinal cord, SCAs anterior horn of sacral spinal cord, SCAt anterior horn of thoracic spinal cord, SCPc posterior horn of cervical spinal cord, SCPl anterior horn of lumbar spinal cord, SCPs posterior horn of sacral spinal cord, SCPt posterior horn of thoracic spinal cord, SNc substantia nigra compact part, STN subthalamic nucleus, SubCA1/2 subiculum plus cornu ammonis fields 1/2, TCtx temporal cortex, Th thalamus