Neurodegeneration in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9orf72 is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins

Aims A hexanucleotide expansion in C9orf72 is the major genetic cause of inherited behavioural variant Frontotemporal dementia (bvFTD) and motor neurone disease (MND), although the pathological mechanism(s) underlying disease remains uncertain. Methods Using antibodies to poly‐GA, poly‐GP, poly‐GR,...

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Veröffentlicht in:	Neuropathology and applied neurobiology 2016-04, Vol.42 (3), p.242-254
Hauptverfasser:	Davidson, Y., Robinson, A. C., Liu, X., Wu, D., Troakes, C., Rollinson, S., Masuda-Suzukake, M., Suzuki, G., Nonaka, T., Shi, J., Tian, J., Hamdalla, H., Ealing, J., Richardson, A., Jones, M., Pickering-Brown, S., Snowden, J. S., Hasegawa, M., Mann, D. M. A.
Format:	Artikel
Sprache:	eng
Schlagworte:	Aged C9orf72 C9orf72 Protein dipeptide repeat proteins Dipeptides DNA Repeat Expansion DNA-Binding Proteins - metabolism Female frontotemporal lobar degeneration Frontotemporal Lobar Degeneration - genetics Frontotemporal Lobar Degeneration - pathology Humans Immunohistochemistry Inclusion Bodies - metabolism Inclusion Bodies - pathology Male Middle Aged Motor Neuron Disease - genetics Motor Neuron Disease - pathology motor neurone disease Nerve Degeneration - genetics Nerve Degeneration - pathology Neurons - pathology Original Pathology Proteins Proteins - genetics Spinal cord TDP-43
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