Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: A spectrum of TDP-43 proteinopathies

Geser, Felix; Lee, Virginia M.-Y.; Trojanowski, John Q.

doi:10.1111/j.1440-1789.2009.01091.x

Cited by 195Amyotrophic Lateral Sclerosis ResearchAlzheimer's disease research and treatmentsAmyloidosis: Diagnosis, Treatment, Outcomes

Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: A spectrum of TDP-43 proteinopathies

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Geser, Felix, Lee, Virginia M.-Y., Trojanowski, John Q.·2010·Neuropathology

DOI 10.1111/j.1440-1789.2009.01091.xPMC PMC3131978

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Abstract

It is now established that pathological transactive response DNA-binding protein with a Mr of 43 kD (TDP-43) on sodium dodecyl sulfate-polyacrylamide gel electrophoresis is the major disease protein in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) with ubiquitin-positive inclusions (now known as FTLD-TDP). In fact, the discovery of pathological TDP-43 solidified the idea that these disorders are multi-system diseases and this led to the concept of a TDP-43 proteinopathy as a spectrum of disorders comprised of different clinical and pathological entities extending from ALS to ALS with cognitive impairment/dementia and FTLD-TDP without or with motor neuron disease (FTLD-MND). These align along a broad disease continuum sharing similar pathogenetic mechanisms linked to pathological TDP-43. We here review salient findings in the development of a concept of TDP-43 proteinopathy as a novel group of neurodegenerative diseases similar in concept to alpha-synucleinopathies and tauopathies.

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TDP-43 can be phosphorylated, which is hypothesized to play a role in its toxicity.
“TDP-43 can be phosphorylated, which is hypothesized to play a role in its toxicity (102,103).”
From Functional genomic screen and network analysis reveal novel modifiers of tauopathy dissociated from tau phosphorylation (2011) · cites this paper as [103]
Age-dependent neurodegenerative disorders are clinically diverse but exhibit significant comorbidity and heterogeneous pathology both within and across disorders.
“Age-dependent neurodegenerative disorders are clinically diverse but exhibit significant comorbidity and heterogeneous pathology both within and across disorders”
From The Role of Retrotransposons and Endogenous Retroviruses in Age-Dependent Neurodegenerative Disorders (2024) · cites this paper as Geser et al. 2010

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Lin, W.-L., & Dickson, D. W. (2008) · Acta Neuropathologica · doi:10.1007/s00401-008-0408-9 · Cited by 134 · see citing works
Maturation process of TDP-43-positive neuronal cytoplasmic inclusions in amyotrophic lateral sclerosis with and without dementia
Mori, F., Tanji, K., Zhang, H., Nishihira, Y., Tan, C., Takahashi, H., & Wakabayashi, K. (2008) · Acta Neuropathologica · doi:10.1007/s00401-008-0396-9 · Cited by 133 · see citing works
Evaluation of subcortical pathology and clinical correlations in FTLD-U subtypes
Josephs, K. A., Stroh, A., Dugger, B. N., & Dickson, D. W. (2009) · Acta Neuropathologica · doi:10.1007/s00401-009-0547-7 · Cited by 126 · see citing works
Lack of TDP-43 abnormalities in mutant SOD1 transgenic mice shows disparity with ALS
Robertson, J., Sanelli, T. R., Xiao, S., Yang, W., Horne, P., Hammond, R. R., Pioro, E. P., & Strong, M. J. (2007) · Neuroscience Letters · doi:10.1016/j.neulet.2007.03.066 · Cited by 125 · see citing works
TDP-43: an emerging new player in neurodegenerative diseases
Wang, I., Wu, L., & Shen, C. (2008) · Trends in Molecular Medicine · doi:10.1016/j.molmed.2008.09.001 · Cited by 121 · see citing works
Neuropathology of ALS
Hirano, A. (1996) · Neurology · doi:10.1212/wnl.47.4_suppl_2.63s · Cited by 119 · see citing works
A Novel CpG-free Vertebrate Insulator Silences the Testis-specific SP-10 Gene in Somatic Tissues
Abhyankar, M. M., Urekar, C., & Reddi, P. P. (2007) · Journal of Biological Chemistry · doi:10.1074/jbc.m705811200 · Cited by 108 · see citing works
TARDBP (TDP‐43) sequence analysis in patients with familial and sporadic ALS: identification of two novel mutations
Bo, R. D., Ghezzi, S., Corti, S. P., Pandolfo, M., Ranieri, M., Santoro, D., Ghione, I., Prelle, A. C., Orsetti, V., Mancuso, M., et al. (2009) · European Journal of Neurology · doi:10.1111/j.1468-1331.2009.02574.x · Cited by 106 · see citing works
Chromosome 9p-linked families with frontotemporal dementia associated with motor neuron disease
Ber, I. L., Camuzat, A., Berger, É., Hannequin, D., Laquerrière, A., Golfier, V., Seilhean, D., Viennet, G., Couratier, P., Verpillat, P., et al. (2009) · Neurology · doi:10.1212/wnl.0b013e3181a55f1c · Cited by 98 · see citing works
Clinicopathological characterization of Pick’s disease versus frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions
Yokota, O., Tsuchiya, K., Arai, T., Yagishita, S., Matsubara, O., Mochizuki, A., Tamaoka, A., Kawamura, M., Yoshida, H., Terada, S., et al. (2009) · Acta Neuropathologica · doi:10.1007/s00401-009-0493-4 · Cited by 90 · see citing works
Neuronal inclusion protein TDP-43 has no primary genetic role in FTD and ALS
Gijselinck, I., Sleegers, K., Engelborghs, S., Robberecht, W., Martin, J., Vandenberghe, R. R., Sciot, R., Dermaut, B., Goossens, D., van der Zee, J., et al. (2008) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2007.11.002 · Cited by 85 · see citing works
Severe subcortical TDP-43 pathology in sporadic frontotemporal lobar degeneration with motor neuron disease
Brandmeir, N. J., Geser, F., Kwong, L. K., Zimmerman, E. A., Qian, J., Lee, V. M. ‐., & Trojanowski, J. Q. (2007) · Acta Neuropathologica · doi:10.1007/s00401-007-0315-5 · Cited by 85 · see citing works
Self-citation
Expression of ubiquitin-binding protein p62 in ubiquitin-immunoreactive intraneuronal inclusions in amyotrophic lateral sclerosis with dementia: analysis of five autopsy cases with broad clinicopathological spectrum
Nakano, T., Nakaso, K., Ohama, E., & Nakashima, K. (2004) · Acta Neuropathologica · doi:10.1007/s00401-004-0821-7 · Cited by 78 · see citing works
Survival in two variants of tau-negative frontotemporal lobar degeneration: FTLD-U vs FTLD-MND
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TDP-43 is a culprit in human neurodegeneration, and not just an innocent bystander
Banks, G. T., Kuta, A., Isaacs, A. M., & Fisher, E. (2008) · Mammalian Genome · doi:10.1007/s00335-008-9117-x · Cited by 71 · see citing works
Accumulation of TDP-43 and α-actin in an amyotrophic lateral sclerosis patient with the K17I ANG mutation
Seilhean, D., Cazeneuve, C., Thuriès, V., Russaouen, O., Millecamps, S., Salachas, F., Meininger, V., LeGuern, E., & Duyckaerts, C. (2009) · Acta Neuropathologica · doi:10.1007/s00401-009-0545-9 · Cited by 71 · see citing works
TDP-43 Is Not a Common Cause of Sporadic Amyotrophic Lateral Sclerosis
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Sporadic amyotrophic lateral sclerosis of long duration is associated with relatively mild TDP-43 pathology
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TDP-43 Pathologic Lesions and Clinical Phenotype in Frontotemporal Lobar Degeneration With Ubiquitin-Positive Inclusions
Grossman, M., Wood, E. M., Moore, P., Neumann, M., Kwong, L. K., Forman, M. S., Clark, C. M., McCluskey, L. F., Miller, B. L., Lee, V. M. ‐., et al. (2007) · Archives of Neurology · doi:10.1001/archneur.64.10.1449 · Cited by 60 · see citing works
Self-citation
Amyotrophic Lateral Sclerosis–Plus Syndrome With TAR DNA-Binding Protein-43 Pathology
McCluskey, L. F., Elman, L. B., Martinez‐Lage, M., Deerlin, V. M. V., Yuan, W., Falcone, D., Siderowf, A. D., & Trojanowski, J. Q. (2009) · Archives of Neurology · doi:10.1001/archneur.66.1.121 · Cited by 58 · see citing works
Self-citation
TDP-43 M311V mutation in familial amyotrophic lateral sclerosis
Lemmens, R., Race, V., Hersmus, N., Matthijs, G., Bosch, L. V. D., Damme, P. V., Dubois, B., Boonen, S. E., Goris, A., & Robberecht, W. (2009) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2008.157677 · Cited by 56 · see citing works
Fine structural analysis of the neuronal inclusions of frontotemporal lobar degeneration with TDP-43 proteinopathy
Thorpe, J. R., Tang, H., Atherton, J., & Cairns, N. J. (2008) · Journal of Neural Transmission · doi:10.1007/s00702-008-0137-1 · Cited by 55 · see citing works
Screening for TARDBP mutations in Japanese familial amyotrophic lateral sclerosis
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Evidence That TDP-43 is Not the Major Ubiquitinated Target Within the Pathological Inclusions of Amyotrophic Lateral Sclerosis
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TDP‐43 neuropathology is similar in sporadic amyotrophic lateral sclerosis with or without TDP‐43 mutations
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Phosphorylated Smad2/3 immunoreactivity in sporadic and familial amyotrophic lateral sclerosis and its mouse model
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TDP-43 gene analysis in frontotemporal lobar degeneration
Rollinson, S. J., Snowden, J. S., Neary, D., Morrison, K. E., Mann, D. M. A., & Pickering-Brown, S. M. (2007) · Neuroscience Letters · doi:10.1016/j.neulet.2007.03.044 · Cited by 44 · see citing works
Ubiquitin associated protein 1 is a risk factor for frontotemporal lobar degeneration
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Sporadic amyotrophic lateral sclerosis: Widespread multisystem degeneration with TDP‐43 pathology in a patient after long‐term survival on a respirator
Nishihira, Y., Tan, C., Toyoshima, Y., Yonemochi, Y., Kondo, H., Nakajima, T., & Takahashi, H. (2009) · Neuropathology · doi:10.1111/j.1440-1789.2008.00999.x · Cited by 39 · see citing works
No association of TDP-43 with sporadic frontotemporal dementia
Schumacher, A., Friedrich, P., Diehl‐Schmid, J., Ibach, B., Perneczky, R., Eisele, T., Vukovich, R., Förstl, H., & Riemenschneider, M. J. (2007) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2007.05.022 · Cited by 39 · see citing works
TDP-43 in skeletal muscle of patients affected with amyotrophic lateral sclerosis
Sorarú, G., Orsetti, V., Buratti, E., Baralle, F. E., Cima, V., Volpe, M., D’Ascenzo, C., Palmieri, A., Koutsikos, K., Pegoraro, E., et al. (2010) · Amyotrophic Lateral Sclerosis · doi:10.3109/17482960902810890 · Cited by 30 · see citing works
Neuropathology of Olfactory Ensheathing Cell Transplantation into the Brain of Two Amyotrophic Lateral Sclerosis (ALS) Patients
Giordana, M. T., Grifoni, S., Votta, B., Magistrello, M., Vercellino, M., Pellerino, A., Navone, R., Valentini, C., Calvo, A., & Chiò, A. (2009) · Brain Pathology · doi:10.1111/j.1750-3639.2009.00353.x · Cited by 26 · see citing works
TDP-43 immunoreactivity in neurodegenerative disorders: disease versus mechanism specificity
Dickson, D. W. (2007) · Acta Neuropathologica · doi:10.1007/s00401-007-0323-5 · Cited by 24 · see citing works
TDP-43 inclusions do not protect motor neurons from sporadic ALS
Pamphlett, R., & Jew, S. K. (2008) · Acta Neuropathologica · doi:10.1007/s00401-008-0392-0 · Cited by 22 · see citing works
TAR DNA-binding protein-43 in amyotrophic lateral sclerosis, frontotemporal lobar degeneration, and Alzheimer disease
Bigio, E. H. (2008) · Acta Neuropathologica · doi:10.1007/s00401-008-0405-z · Cited by 16 · see citing works
Motor Neuron Disease and Frontotemporal Lobar Degeneration: A Tale of Two Disorders Linked To TDP-43
Elman, L. B., McCluskey, L. F., & Grossman, M. (2007) · Neurosignals · doi:10.1159/000109762 · Cited by 14 · see citing works
TDP-43 in skeletal muscle of patients affected with amyotrophic lateral sclerosis
Sorarú, G., Orsetti, V., Buratti, E., Baralle, F. E., Cima, V., Volpe, M., D’Ascenzo, C., Palmieri, A., Koutsikos, K., Pegoraro, E., et al. (2009) · Amyotrophic Lateral Sclerosis · doi:10.1080/17482960902810890 · Cited by 3 · see citing works

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[8]Geser F Lee VM-Y Trojanowski JQ Rosenberg RN DiMauro S Paulson H Ptacek L Nestler E Frontotemporal dementias The Molecular and genetic Basis of Neurological and Psychiatric Disease 2007 Philadelphia, PA Lippincott Williams & Wilkins 330 338

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Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration
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Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice
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Chronic traumatic encephalopathy in an Iraqi war veteran with posttraumatic stress disorder who committed suicide
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The Pathobiology of TDP-43 C-Terminal Fragments in ALS and FTLD
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TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia
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Pattern of ubiquilin pathology in ALS and FTLD indicates presence of C9ORF72 hexanucleotide expansion
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Inflammation Induces TDP-43 Mislocalization and Aggregation
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Authors: Geser, F., Lee, V. M.-Y., & Trojanowski, J. Q.
Affiliations: University of Pennsylvania (US)
Corresponding author: Trojanowski, John Q.
ORCID: Geser, Felix
Lee, Virginia M.-Y.
Trojanowski, John Q.
Venue: Neuropathology
Publisher: Wiley
Published: 2010-04
Volume: 30
Issue: 2
Pages: 103-112
ISSN: 0919-6544 (ISSN-L)
1440-1789
DOI: 10.1111/j.1440-1789.2009.01091.x
PMC: PMC3131978
OpenAlex: W2125931517
Cited by: 195
References: 108
Citations by year: 2025: 4 · 2024: 11 · 2023: 15 · 2022: 11 · 2021: 8 · 2020: 8 · 2019: 12 · 2018: 8 · 2017: 10 · 2016: 10 · 2015: 12 · 2014: 10 · 2013: 16 · 2012: 24
FWCI: 3.01
Type: journal article
Language: en
Open access: green
www.ncbi.nlm.nih.gov
Licence: doi.wiley.com/10.1002/tdm_license_1.1 (tdm, 1979 d)
Locations: Neuropathology (publishedVersion)
PubMed (publishedVersion)
PubMed Central (acceptedVersion)
Funders: National Institute on Aging (P30AG010124)
Topics: Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
Keywords: amyotrophic lateral sclerosis, frontotemporal lobar degeneration, TDP-43, TDP-43 proteinopathy, FTLD-TDP, disease continuum, neurodegenerative diseases, synucleinopathies, tauopathy, ubiquitin-positive inclusions
MeSH terms: Amyotrophic Lateral Sclerosis, Brain, DNA-Binding Proteins, Humans, Neurons, Frontotemporal Lobar Degeneration

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Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: A spectrum of TDP-43 proteinopathies

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