N.B. This page is in the initial phases of construction, so I suggest you follow the external links below for more on this disease.

 (external site links in italics )

See:  Spillantini et al. (1998): Downloadable Review

and the 'Institut National de la Sante et de la Recherche Medical' website for more detail on FTDP-17. 

	Type:  FTDP-17 is a group of familial (inherited) tauopathies.
	Regions affected:  Include those involved in cognitive, executive and motor functions and the different variants result from varying burdens of pathology in these regions.
	Tau pathology: Neuronal:  Abundant filamentous tau pathology. Glial   :  In some variants. Neuropil threads :
	Other pathology: some beta-amyloid in a minority of cases.

Some Related References

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Arai, T, Nonaka, T, Hasegawa, M, Akiyama, H, Yoshida, M, Hashizume, Y, Tsuchiya, K, Oda, T, Ikeda, K (2003) Neuronal and glial inclusions in frontotemporal dementia with or without motor neuron disease are immunopositive for p62. NEUROSCIENCE LETTERS 342: 41-44  

Bigio, EH, Lipton, AM, White, CL, Dickson, DW, Hirano, A (2003) Frontotemporal and motor neurone degeneration with neurofilament inclusion bodies: additional evidence for overlap between FTD and ALS. NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY 29: 239-253

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Chang, HT, Cortez, S, Vonsattel, JP, Stopa, EG, Schelper, RL (2004) Familial frontotemporal dementia: a report of three cases of severe cerebral atrophy with rare inclusions that are negative for tau and synuclein, but positive for ubiquitin. ACTA NEUROPATHOLOGICA 108: 10-16

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Curcio, SAM, Kawarai, T, Paterson, AD, Maletta, RG, Puccio, G, Perri, M, Di Natale, M, Palermo, S, Foncin, JF, Hyslop, PHS, Bruni, AC (2002) A large Calabrian kindred segregating frontotemporal dementia. JOURNAL OF NEUROLOGY 249: 911-92

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Delobel, P, Flament, S, Hamdane, M, Jakes, R, Rousseau, A, Delacourte, A, Vilain, JP, Goedert, M, Buee, L (2002) Functional characterization of FTDP-17 tau gene mutations through their effects on Xenopus oocyte maturation. JOURNAL OF BIOLOGICAL CHEMISTRY 277: 9199-9205

de Silva, R, Hardy, J, Crook, J, Khan, N, Graham, EA, Morris, CM, Wood, NW, Lees, AJ (2002) The tau locus is not significantly associated with pathologically confirmed sporadic Parkinson's disease . NEUROSCIENCE LETTERS 330: 201-203

DeTure, M, Ko, LW, Easson, C, Yen, SH (2002) tau assembly in inducible transfectants expressing wild-type or FTDP-17 tau . AMERICAN JOURNAL OF PATHOLOGY 161: 1711-1722

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Gydesen, S, Brown, JM, Brun, A, Chakrabarti, L, Gade, A, Johannsen, P, Rossor, M, Thusgaard, T, Grove, A, Yancopoulou, D, Spillantini, MG, Fisher, EMC, Collinge, J, Sorensen, SA (2002) Chromosome 3 linked frontotemporal dementia (FTD-3) . NEUROLOGY 59: 1585-1594  

Hamilton, RL (2003) The other dementias: The neuropathology of the non-Alzheimer's disease dementias. REVISTA DE NEUROLOGIA 37: 130-139

Hutton, M (2001) Missense and splice site mutations in tau associated with FTDP-17: Multiple pathogenic mechanisms. NEUROLOGY 56: S21-S25

Hutton, M.; Lendon, C. L.; Rizzu, P.; Baker, M.; Froelich, S.; Houlden, H.; Pickering-Brown, S.; Chakraverty, S.; Isaacs, A.; Grover, A.; Hackett, J.; Adamson, J.; and 39 others: (1998) Association of missense and 5-prime-splice-site mutations in tau with the inherited dementia FTDP-17. Nature 393: 702-705

Ingram, EM, Spillantini, MG (2002) Tau gene mutations: dissecting the pathogenesis of FTDP-17 . TRENDS IN MOLECULAR MEDICINE 8: 555-562

Jiang, ZH, Tang, H, Havlioglu, N, Zhang, XC, Stamm, S, Yan, RQ, Wu, JY (2003) Mutations in tau gene exon 10 associated with FTDP-17 alter the activity of an exonic splicing enhancer to interact with Tra2 beta. JOURNAL OF BIOLOGICAL CHEMISTRY 278: 18997-19007

Johansson, A, Hampel, H, Faltraco, F, Buerger, K, Minthon, L, Bogdanovic, N, Sjogren, M, Zetterberg, H, Forsell, L, Lilius, L, Wahlund, LO, Rymo, L, Prince, JA, Blennow, K (2003) Increased frequency of a new polymorphism in the cycle 2 (cdc2) gene in patients with Alzheimer's disease frontotemporal dementia. NEUROSCIENCE LETTERS 340: 69-73

Kertesz, A (2003) Pick complex: An integrative approach to frontotemporal dementia - Primary progressive aphasia, corticobasal degeneration, and progressive supranuclear palsy. NEUROLOGIST 9: 311-317

Kertesz, A, Munoz, DG (2002) Frontotemporal dementia. MEDICAL CLINICS OF NORTH AMERICA 86: 501-520

Kobayashi, T, Ota, S, Tanaka, K, Ito, Y, Hasegawa, M, Umeda, Y, Motoi, Y, Takanashi, M, Yasuhara, M, Anno, M, Mizuno, Y, Mori, H (2003) A novel L266V mutation of the tau gene causes frontotemporal dementia with a unique tau pathology. ANNALS OF NEUROLOGY 53: 133-137

Kosik, KS, Ahn, J, Stein, R, Yeh, LA (2002) Discovery of compounds that will prevent tau pathology . JOURNAL OF MOLECULAR NEUROSCIENCE 19: 261-266

Kowalska, A (2002) Molecular genetics of frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) . FOLIA NEUROPATHOLOGICA 40: 111-118

Kowalska, A, Takahashi, K, Kozubski, W, Tabira, T (2003) Microtubule associated protein (Tau) gene variability in patients with frontotemporal dementia. FOLIA NEUROPATHOLOGICA 40: 1-5  

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Li, L, von Bergen, M, Mandelkow, EM, Mandelkow, E (2002)  Structure, stability, and aggregation of paired helical filaments from tau protein and FTDP-17 mutants probed by tryptophan scanning mutagenesis . JOURNAL OF BIOLOGICAL CHEMISTRY 277: 41390-41400

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Verpillat, P, Camuzat, A, Hannequin, D, Thomas-Anterion, C, Puel, M, Belliard, S, Dubois, B, Didic, M, Michel, BF, Lacomblez, L, Moreaud, O, Sellal, F, Golfier, V, Campion, D, Clerget-Darpoux, F, Brice, A (2002) Association between the extended tau haplotype and frontotemporal dementia. ARCHIVES OF NEUROLOGY 59: 935-939

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Zhukareva, V, Sundarraj, S, Mann, D, Sjogren, M, Blenow, K, Clark, CM, McKeel, DW, Goate, A, Lippa, CF, Vonsattel, JP, Growdon, JH, Trojanowski, JQ, Lee, VMY (2003) Selective reduction of soluble Tau proteins in sporadic and familial frontotemporal dementias: an international follow-up study. ACTA NEUROPATHOLOGICA 105: 469-476