TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia

IRA Mackenzie, R Rademakers… - The Lancet Neurology, 2010 - thelancet.com
… form of frontotemporal dementia (FTD), frontotemporal lobar degeneration with ubiquitinated
inclusions (FTLD-U), remained unknown until the TAR DNA binding protein 43 (TDP-43) …

Amyotrophic lateral sclerosis, frontotemporal dementia and beyond: the TDP-43 diseases

F Geser, M Martinez-Lage, LK Kwong, VMY Lee… - Journal of …, 2009 - Springer
TDP-43 related diseases, which shall be reviewed in this article. We focus especially on
the spectrum of the major TDP-43 … significance of the secondary TDP-43 pathology in other …

Neurons selectively targeted in frontotemporal dementia reveal early stage TDP-43 pathobiology

AL Nana, M Sidhu, SE Gaus, JHL Hwang, L Li… - Acta …, 2019 - Springer
… Therefore, we also studied the morphology of nuclear TDP-43-depleted and TDP-43
inclusion-bearing neurons to determine whether the reduction of nuclear TDP-43 alone is …

TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia

JD Rohrer, F Geser, J Zhou, ED Gennatas, M Sidhu… - Neurology, 2010 - AAN Enterprises
TDP-43 subtyping as a biologically meaningful and constructive framework for understanding
FTLD-TDP… schemes had MND and died with TDP-43 pathology too sparse to classify. …

TDP-43 and frontotemporal dementia

WT Hu, M Grossman - Current neurology and neuroscience reports, 2009 - Springer
… This family of disorders, collectively referred to as TDP-43 proteinopathies, is believed to …
system TDP-43 involvement. This review summarizes the recent advances in TDP-43 pro…

TDP-43 pathology in familial frontotemporal dementia and motor neuron disease without Progranulin mutations

H Seelaar, H Jurgen Schelhaas, A Azmani, B Küsters… - Brain, 2007 - academic.oup.com
TDP-43 antibody showed robust staining of neuronal inclusions similar in distribution and
morphology to NCI and NII. Additionally, TDP-43 … The observation of glial TDP-43 positive …

Frontotemporal dementia and amyotrophic lateral sclerosis-associated disease protein TDP-43 promotes dendritic branching

Y Lu, J Ferris, FB Gao - Molecular brain, 2009 - Springer
TDP-43 (dTDP-43) in vivo significantly increases dendritic branching of sensory neurons in
Drosophila larvae. Loss of dTDP-43 … for dTDP-43 in dendrite formation. Moreover, human …

ALS and FTLD: two faces of TDP43 proteinopathy

RM Liscic, LT Grinberg, J Zidar… - European journal of …, 2008 - Wiley Online Library
… Inclusion body myopathy associated with Paget’s disease of bone and frontotemporal
dementia (IBMPFD) is a rare autosomal dominant disorder caused by a mutation in the VCP gene […

Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: a spectrum of TDP43 proteinopathies

F Geser, VMY Lee, JQ Trojanowski - Neuropathology, 2010 - Wiley Online Library
… However, in the spinal cord the predominant p409/410-labeled TDP-43 protein species
is full-length TDP-43, 59 which is consistent with our previous findings that different TDP-43

Advances in understanding the molecular basis of frontotemporal dementia

R Rademakers, M Neumann… - Nature Reviews Neurology, 2012 - nature.com
Frontotemporal dementia (FTD) is a clinical syndrome with a … That same year, TAR
DNA-binding protein 43 (TDP-43) was … and regulation of progranulin and TDP-43, as well as their …