TDP-43-negative FTLD-U is a significant new clinico-pathological subtype of FTLD

Acta Neuropathologica

Volumn 116, Issue 2, 2008, Pages 147-157

  Roeber, Sigrun ^a   Mackenzie, Ian R A ^b   Kretzschmar, Hans A ^a   Neumann, Manuela ^a  

^a UNIVERSITY OF MUNICH   (Germany)

^b UNIVERSITY OF BRITISH COLUMBIA   (Canada)

Author keywords

Frontotemporal dementia; Frontotemporal lobar degeneration; Intranuclear inclusions; p62; TDP 43; Ubiquitin

Indexed keywords

NUCLEAR PROTEIN; PROTEIN P62; TRANSACTIVE RESPONSE DNA BINDING PROTEIN 43; UBIQUITIN;

ADULT; ARTICLE; BEHAVIOR CHANGE; BRAIN STEM; CELL INCLUSION; CLINICAL ARTICLE; CONTROLLED STUDY; DISEASE CLASSIFICATION; FEMALE; FRONTOTEMPORAL DEMENTIA; FRONTOTEMPORAL LOBAR DEGENERATION WITH UBIQUITIN POSITIVE INCLUSIONS; HIPPOCAMPUS; HISTOPATHOLOGY; HUMAN; HUMAN TISSUE; IMMUNOFLUORESCENCE; IMMUNOHISTOCHEMISTRY; IMMUNOREACTIVITY; MALE; NEOCORTEX; PERSONALITY DISORDER; PHENOTYPE; PRIORITY JOURNAL; PROTEIN ANALYSIS; SPINAL CORD;

ADULT; BRAIN; DEMENTIA; DNA-BINDING PROTEINS; FEMALE; FLUORESCENT ANTIBODY TECHNIQUE; HUMANS; IMMUNOHISTOCHEMISTRY; INCLUSION BODIES; MALE; MIDDLE AGED; UBIQUITIN;

EID: 47949123961     PISSN: 00016322     EISSN: None     Source Type: Journal    
DOI: 10.1007/s00401-008-0395-x     Document Type: Article

References (23)

1. Amador-Ortiz C, Lin WL, Ahmed Z, Personett D, Davies P, Duara R et al (2007) TDP-43 immunoreactivity in hippocampal sclerosis and Alzheimer's disease. Ann Neurol 61:435-445
2. Arai T, Hasegawa M, Akiyama H, Ikeda K, Nonaka T, Mori H et al (2006) TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem Biophys Res Commun 351:602-611. doi: 10.1016/j.bbrc.2006.10.093
3. Cairns NJ, Bigio EH, Mackenzie IR, Neumann M, Lee VM, Hatanpaa KJ et al (2007) Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: Consensus of the consortium for frontotemporal lobar degeneration. Acta Neuropathol 114:5-22. doi: 10.1007/s00401-007-0237-2
4. Cairns NJ, Neumann M, Bigio EH, Holm IE, Troost D, Hatanpaa KJ et al (2007) TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusions. Am J Pathol 171:227-240. doi: 10.2353/ ajpath.2007.070182
5. Davidson Y, Kelley T, Mackenzie IR, Pickering-Brown S, Du Plessis D, Neary D et al (2007) Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43. Acta Neuropathol 113:521-533. doi: 10.1007/s00401-006-0189-y
6. Giannakopoulos P, Hof PR, Bouras C (1995) Dementia lacking distinctive histopathology: Clinicopathological evaluation of 32 cases. Acta Neuropathol 89:346-355. doi: 10.1007/BF00309628
7. Grossman M (2002) Frontotemporal dementia: A review. J Int Neuropsychol Soc 8:566-583. doi: 10.1017/S1355617702814357
8. Hatanpaa KJ, Bigio EH, Cairns NJ, Womack KB, Weintraub S, Morris JC et al (2008) TAR DNA-binding protein 3 immunohistochemistry reveals extensive neuritic pathology in FTLD-U: A midwest-southwest consortium for FTLD study. J Neuropathol Exp Neurol 67:271-279. doi: 10.1097/ NEN.0b013e31816a12a6
9. Johnson JK, Diehl J, Mendez MF, Neuhaus J, Shapira JS, Forman M et al (2005) Frontotemporal lobar degeneration: Demographic characteristics of 353 patients. Arch Neurol 62:925-930. doi: 10.1001/archneur.62.6.925
10. Kahle PJ, Neumann M, Ozmen L, Muller V, Jacobsen H, Schindzielorz A et al (2000) Subcellular localization of wild-type and Parkinson's disease-associated mutant alpha-synuclein in human and transgenic mouse brain. J Neurosci 20:6365-6373
11. Knopman DS, Mastri AR, Frey WH, Sung JH, Rustan T (1990) Dementia lacking distinctive histologic features: A common non-Alzheimer degenerative dementia. Neurology 40:251-256
12. Kuusisto E, Salminen A, Alafuzoff I (2001) Ubiquitin-binding protein p62 is present in neuronal and glial inclusions in human tauopathies and synucleinopathies. Neuroreport 12:2085-2090. doi: 10.1097/ 00001756-200107200-00009
13. Lipton AM, White CL 3rd, Bigio EH (2004) Frontotemporal lobar degeneration with motor neuron disease-type inclusions predominates in 76 cases of frontotemporal degeneration. Acta Neuropathol 108:379-385. doi: 10.1007/s00401-004-0900-9
14. Mackenzie IR, Foti D, Woulfe J, Hurwitz TA (2008) Atypical frontotemporal lobar degeneration with ubiquitin-positive, TDP-43-negative neuronal inclusions. Brain 131:1282-1293. doi: 10.1093/ brain/awn061
15. Mackenzie IR, Shi J, Shaw CL, Duplessis D, Neary D, Snowden JS et al (2006) Dementia lacking distinctive histology (DLDH) revisited. Acta Neuropathol 112:551-559. doi: 10.1007/s00401-006-0123-3
16. Neary D, Snowden JS, Gustafson L, Passant U, Stuss D, Black S et al (1998) Frontotemporal lobar degeneration: A consensus on clinical diagnostic criteria. Neurology 51:1546-1554
17. Neumann M, Igaz LM, Kwong LK, Nakashima-Yasuda H, Kolb SJ, Dreyfuss G et al (2007) Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degeneration. Acta Neuropathol 113:543-548. doi: 10.1007/s00401-007-0221-x
18. Neumann M, Kwong LK, Truax AC, Vanmassenhove B, Kretzschmar HA, Van Deerlin VM et al (2007) TDP-43-positive white matter pathology in frontotemporal lobar degeneration with ubiquitin-positive inclusions. J Neuropathol Exp Neurol 66:177-183. doi: 10.1097/ 01.jnen.0000248554.45456.58
19. Neumann M, Sampathu DM, Kwong LK, Truax AC, Micsenyi MC, Chou TT et al (2006) Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 314:130-133. doi: 10.1126/ science.1134108
20. Pickering-Brown SM (2007) The complex aetiology of frontotemporal lobar degeneration. Exp Neurol 206:1-10. doi: 10.1016/j.expneurol.2007.03.017
21. Pikkarainen M, Hartikainen P, Alafuzoff I (2008) Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions visualized with ubiquitin-binding protein p62 immunohistochemistry. J Neuropathol Exp Neurol 67:280-298
22. Probst A, Taylor KI, Tolnay M (2007) Hippocampal sclerosis dementia: A reappraisal. Acta Neuropathol 114:335-345. doi: 10.1007/s00401-007-0262-1
23. Shi J, Shaw CL, Du Plessis D, Richardson AM, Bailey KL, Julien C et al (2005) Histopathological changes underlying frontotemporal lobar degeneration with clinicopathological correlation. Acta Neuropathol 110:501-512. doi: 10.1007/s00401-005-1079-4