Skip to main content

Advertisement

SpringerLink
Log in

Inclusions in frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP) and amyotrophic lateral sclerosis (ALS), but not FTLD with FUS proteinopathy (FTLD-FUS), have properties of amyloid

  • Correspondence
  • Published:
Acta Neuropathologica Aims and scope Submit manuscript

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

References

  1. Cohen TJ, Lee VMY, Trojanowski JQ (2011) TDP-43 functions and pathogenic mechanisms implicated in TDP-43 proteinopathies. Trends Mol Med 17:659–667

    Article  PubMed  CAS  Google Scholar 

  2. Engler H, Santillo AF, Wang SX, Lindau M, Savitcheva I, Nordberg A, Lannfelt L, Langstom B, Kilander L (2008) In vivo amyloid imaging with PET in frontotemporal dementia. Eur J Nucl Med Mol Imaging 35:100–106

    Article  PubMed  Google Scholar 

  3. Guo W, Chen Y, Zhou X, Kar A, Ray P, Chen X, Rao EJ, Yang M, Ye H, Zhu L, Liu J, Zhang D, Buratti E, Baralle FE, Bigio EH, Mesulam M, Xu Q, Shen Y, Fushimi K, Wu JY (2011) An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxocity. Nat Struct Mol Biol 18:822–830

    Article  PubMed  CAS  Google Scholar 

  4. Klunk WE, Engler H, Nordberg A, Wang Y, Blomqvist G, Holt DP, Bergstrom M, Savitcheva I, Huang G, Estrada S, Ausen B, Debnath ML, Barletta J, Price JC, Sandell J, Lopresti BJ, Wall A, Koivisto P, Antoni G, Mathis CA, Langstrom B (2004) Imaging brain amyloid in Alzheimer’s disease with Pittsburgh compound-B. Ann Neurol 55:306–319

    Article  PubMed  CAS  Google Scholar 

  5. Lee EB, Lee VM-Y, Trojanowski JQ (2012) Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration. Nature 13:38–50

    CAS  Google Scholar 

  6. Robinson JL, Geser F, Stieber A, Umoh M, Kwong LK, Van Deerlin VM, Lee VM-Y, Trojanowski JQ (2013) TDP-43 skeins show properties of amyloid in a subset of ALS cases. Acta Neuropathol 125:121–131. doi:10.1007/s00401-012-1055-8

    Article  PubMed  CAS  Google Scholar 

  7. Westermark GT, Johnson KH, Westermark P (1999) Staining methods for identification of amyloid in tissue. Methods Enzymol 309:3–25

    Article  PubMed  CAS  Google Scholar 

  8. Wolozin B (2012) Regulated protein aggregation: stress granules and neurodegeneration. Mol Neurodegener 7:56. doi:10.1186/1750-1326-7-56

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

Support from the following is acknowledged: EHB, MM, CG, RG, and MP, NIA AG 13854; JYW, NIA (RO1AG033004), NINDS (R56NS074763) and ALS Therapy Alliance. H-XD, NIH NS078287, NS081474, NS074366; TS, NS078504, NS082104.

Author information

Authors and Affiliations

  1. Cognitive Neurology and Alzheimer Disease Center, Northwestern University Feinberg School of Medicine, Chicago, IL, 60611, USA

    Eileen H. Bigio, Rakhee Ganti, Melanie Peterson, Changiz Geula & Marsel Mesulam

  2. Department of Pathology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Eileen H. Bigio, Esther N. Bit-Ivan & Qinwen Mao

  3. Department of Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Jane Y. Wu, Han-Xiang Deng, Nailah Siddique, Teepu Siddique & Marsel Mesulam

  4. Center for Genetic Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Jane Y. Wu

  5. Les Turner/Lois Insolia ALS Center, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Han-Xiang Deng, Nailah Siddique & Teepu Siddique

Authors
  1. Eileen H. Bigio
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Jane Y. Wu
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Han-Xiang Deng
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Esther N. Bit-Ivan
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Qinwen Mao
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Rakhee Ganti
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Melanie Peterson
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Nailah Siddique
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Changiz Geula
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. Teepu Siddique
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. Marsel Mesulam
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Eileen H. Bigio.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Bigio, E.H., Wu, J.Y., Deng, HX. et al. Inclusions in frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP) and amyotrophic lateral sclerosis (ALS), but not FTLD with FUS proteinopathy (FTLD-FUS), have properties of amyloid. Acta Neuropathol 125, 463–465 (2013). https://doi.org/10.1007/s00401-013-1089-6

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00401-013-1089-6

Keywords

Search

Navigation