α-Internexin aggregates are abundant in neuronal intermediate filament inclusion disease (NIFID) but rare in other neurodegenerative diseases

Acta Neuropathologica

Volumn 108, Issue 3, 2004, Pages 213-223

  Cairns, Nigel J ^a   Uryu, Kunihiro ^a   Bigio, Eileen H ^b   Mackenzie, Ian R A ^c   Gearing, Marla ^d   Duyckaerts, Charles ^e   Yokoo, Hideaki ^f   Nakazato, Yoichi ^f   Jaros, Evelyn ^g   Perry, Robert H ^g   Arnold, Steven E ^a   Lee, Virginia M Y ^a   Trojanowski, John Q ^a  

^a UNIVERSITY OF PENNSYLVANIA   (United States)

^b NORTHWESTERN UNIVERSITY   (United States)

^c VANCOUVER GENERAL HOSPITAL   (Canada)

^d EMORY UNIVERSITY SCHOOL OF MEDICINE   (United States)

^e PITIÉ SALPÊTRIÈRE HOSPITAL   (France)

^f GUNMA UNIVERSITY   (Japan)

^g NEWCASTLE GENERAL HOSPITAL   (United Kingdom)

Author keywords

internexin; Frontotemporal dementia; Intermediate filament; Neurofilament; Neuronal intermediate filament inclusion disease

Indexed keywords

ALPHA INTERNEXIN; ALPHA SYNUCLEIN; EPITOPE; INTERMEDIATE FILAMENT PROTEIN; NEUROFILAMENT PROTEIN; PROTEIN SUBUNIT; TAU PROTEIN;

ADULT; AGED; ALZHEIMER DISEASE; ARTICLE; CELL INCLUSION; CONTROLLED STUDY; DEGENERATIVE DISEASE; DEMENTIA; DIFFUSE LEWY BODY DISEASE; EXTRAPYRAMIDAL SYMPTOM; FEMALE; FRONTOTEMPORAL DEMENTIA; HUMAN; HUMAN TISSUE; IMMUNOHISTOCHEMISTRY; MALE; MOTOR NEURON DISEASE; NEUROLOGIC DISEASE; NEURONAL INTERMEDIATE FILAMENT INCLUSION DISEASE; NIEMANN PICK DISEASE; PARKINSON DISEASE; PATHOGENESIS; PHENOTYPE; PRIORITY JOURNAL; PROGRESSIVE SUPRANUCLEAR PALSY; PROTEIN AGGREGATION; PROTEIN ANALYSIS; PYRAMIDAL SIGN;

ADULT; AGED; AGED, 80 AND OVER; BRAIN; CARRIER PROTEINS; FEMALE; HUMANS; IMMUNOHISTOCHEMISTRY; INCLUSION BODIES; INTERMEDIATE FILAMENT PROTEINS; INTERMEDIATE FILAMENTS; MALE; MIDDLE AGED; NEURODEGENERATIVE DISEASES; NEURONS;

EID: 4444255628     PISSN: 00016322     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (38)

1. Al-Chalabi A, Miller CCJ (2003) Neurofilaments and neurological disease. BioEssays 25:346-365
2. Bigio EH, Lipton AM, White CL 3rd, Dickson DW, Hirano A (2003) Frontotemporal dementia and motor neurone degeneration with neurofilament inclusion bodies: additional evidence for overlap between FTD, and ALS. Neuropathol Appl Neurobiol 29:239-253
3. Cairns NJ, Armstrong RA (2003) Clustering of neurofilament inclusions in the temporal lobe in dementia with neurofilament inclusions. Acta Neuropathol 106:125-128
4. Cairns NJ, Perry RH, Jaros E, Burn D, McKeith IG, Lowe JS, Holton J, Rossor MN, Skullerud K, Duyckaerts C, Cruz-Sanchez FF, Lantos PL (2003) Patients with a novel neurofilamentopathy: dementia with neurofilament inclusions. Neurosci Lett 341:177-180
5. Cairns NJ, Zhukareva V, Uryu K, Zhang B, Bigio E, Mackenzie IRA, Gearing M, Duyckaerts C, Yokoo H, Nakazato Y, Jaros E, Perry RH, Lee VM-Y, Trojanowski JQ (2004) α-Internexin is present in the pathological inclusions of neuronal intermediate filament inclusion disease. Am J Pathol (in press)
6. Carden MJ, Trojanowski JQ, Schlaepfer WW, Lee VM-Y (1987) Two-stage expression of neurofilament polypeptides during rat neurogenesis with early establishment of adult phosphorylation patterns. J Neurosci 7:3499-3504
7. Ching GY, Liem RKH (1998) Roles of head and tail domains in α-internexin's self-assembly and coassembly with the neurofilament triplet proteins. J Cell Sci 111:321-333
8. Ching GY, Liem RKH (1991) Structure of the gene for the neuronal intermediate filament protein α-internexin and functional analysis of its promoter. J Biol Chem 29:19459-19468
9. Ching GY, Chien C-L, Flores R, Liem RKH (1999) Overexpression of α-internexin causes abnormal neurofilamentous accumulations and motor coordination deficits in transgenic mice. J Neurosci 19:2974-2986
10. Duda JE, Giasson BI, Mabon ME, Lee VM, Trojanowski JQ (2002) Novel antibodies to synuclein show abundant striatal pathology in Lewy body diseases. Ann Neurol 52:205-210
11. Durham HD (1988) Aggregation of intermediate filaments by 2,5-hexanedione: comparison of effects on neurofilaments, GFAP filaments and vimentin filaments in dissociated cultures of mouse spinal cord dorsal root ganglia. J Neuropathol Exp Neurol 47:432-442
12. Duyckaerts C, Mokhtari K, Fontaine B, Hauw J-J (2003) Maladie de Pick généralisée: une démence mal nommée caractérisée par des inclusions neurofilamentaires. Rev Neurol (Paris) 159: 224-224
13. Fliegner KH, Kaplan MP, Wood TL, Pintar JE, Liem RKH (1994) Expression of the gene for the neuronal intermediate filament protein α-internexin coincides with the onset of neuronal differentiation in the developing rat nervous system. J Comp Neurol 342:161-173
14. Galvin JE, Nakamura M, McIntosh TK, Saatman KE, Sampathu D, Raghupathi R, Lee VM-Y, Trojanowski JQ (2000) Neurofilament-rich intraneuronal inclusions exacerbate neurodegenerative sequelae of brain trauma in NFH/LacZ transgenic mice. Exp Neurol 165:77-89
15. Gearing M, Castellano AA, Hunter SB, Brat DJ, Glass JD (2003) Unusual neuropathologic findings in a case of primary lateral sclerosis. J Neuropathol Exp Neurol 62:555-555
16. Goedert M, Spillantini MG, Serpell LC, Berriman J, Smith MJ, Jakes R, Crowther RA (2001) From genetics to pathology: tau and alpha-synuclein assemblies in neurodegenerative diseases. Philos Trans R Soc Lond B Biol Sci 356:213-227
17. Goldstein E, Sternberger NM, Sternberger LA (1987) Phosphorylation protects neurofilaments against proteolysis. J Neuroimmunol 14:149-160
18. He CZ, Hays AP (2004) Expression of peripherin in ubiquitinated inclusions of amyotrophic lateral sclerosis. J Neurol Sci 217:47-54
19. Hill WD, Lee VM-Y, Hurtig HI, Murray JM, Trojanowski JQ (1991) Epitopes located in spatially separate domains of each neurofilament subunit are present in Parkinson's disease Lewy bodies. J Comp Neurol 309:150-160
20. Ishihara T, Higuchi M, Zhang B, Yoshiyama Y, Hong M, Trojanowski JQ, VM-Y Lee (2001) Attenuated neurodegenerative disease phenotype in tau transgenic mouse lacking neurofilaments. J Neurosci 15:6026-6035
21. Josephs KA, Holton JL, Rossor MN, Braendgaard H, Ozawa T, Fox NC, Petersen RC, Pearl GS, Ganguly M, Rosa P, Laursen H, Parisi JE, Waldemar G, Quinn NP, Dickson DW, Revesz T (2003) Neurofilament inclusion body disease: a new proteinopathy? Brain 126:2291-2303
22. Kaplan MP, Chin SSM, Fliegner KH, Liem RKH (1990) α-Internexin, a novel neuronal intermediate filament protein, precedes the low molecular weight neurofilament protein (NF-L) in the developing brain. J Neurosci 10:2735-2748
23. Lantos PL, Cairns NJ, Khan MN, King A, Revesz T, Janssen JC, Morris H, Rossor MN (2002) Neuropathologic variation in frontotemporal dementia due to the intronic tau 10(+16) mutation. Neurology 58:1169-1175
24. Lee K, Cleveland DW (1996) Neuronal intermediate filaments. Annu Rev Neurosci 19:187-217
25. Lee VM-Y, Carden MJ, Schlaepfer WW, Trojanowski JQ (1987) Monoclonal antibodies distinguish several differentially phosphorylated states of the two largest rat neurofilament subunits (NF-H and NF-M) and demonstrate their existence in the normal nervous system of adult rats. J Neurosci 7:3478-3488
26. Lee VM-Y, Otvos L Jr, Schmidt ML, Trojanowski JQ (1988) Alzheimer disease tangles share immunological similarities with multiphosphorylation repeats in the two large neurofilament proteins. Proc Natl Acad Sci USA 85:7384-7388
27. Lee VM-Y, Goedert M, Trojanowski JQ (2001) Neurodegenerative tauopathies. Annu Rev Neurosci 24:1121-1159
28. Leigh PN, Dodson A, Swash M, Brion J-P, Anderton BH (1989) Cytoskeletal abnormalities in motor neuron disease: an immunohistochemical study. Brain 112:521-535
29. Manetto V, Sternberger NH, Perry G, Sternberger LA, Gambetti P (1988) Phosphorylation of neurofilaments is altered in amyotrophic lateral sclerosis. J Neuropathol Exp Neurol 47:642-653
30. Migheli A, Pezzulo T, Attanasio A, Schiffer D (1993) Peripherin immunoreactive structures in amyotrophic lateral sclerosis. Lab Invest 68:185-191
31. Pawlyk AC, Giasson BI, Sampathu DM, Perez FA, Lim KL, Dawson VL, Dawson TM, Palmiter RD, Trojanowski JQ, Lee VM (2003) Novel monoclonal antibodies demonstrate biochemical variation of brain parkin with age. J Biol Chem 278: 48120-48128
32. Schmidt ML, Carden MJ, Lee VM-Y, Trojanowski JQ (1987) Phosphate dependent and independent neurofilament epitopes in the axonal swellings of patients with motor neuron disease and controls. Lab Invest 56:282-294
33. Schmidt ML, Lee VM-Y, Trojanowski JQ (1990) Relative abundance of tau and neurofilament epitopes in hippocampal neurofibrillary tangles. Am J Pathol 136:1069-1075
34. Schmidt ML, Lee VM-Y, Trojanowski JQ (1991) Comparative epitope analysis of neuronal cytoskeletal proteins in Alzheimer's disease senile plaque neurites and neuropil threads. Lab Invest 64:352-357
35. Schmidt ML, Murray J, Lee VM-Y, Hill WD, Wertkin A, Trojanowski JQ (1991) Epitope map of neurofilament protein domains in cortical and peripheral nervous system Lewy bodies. Am J Pathol 139:53-65
36. Sternberger LA, Sternberger NH (1983) Monoclonal antibodies distinguish phosphorylated and non-phosphorylated forms of neurofilaments. Proc Natl Acad Sci USA 80:6126-6130
37. Trojanowski JQ, Dickson D (2001) Update on the neuropathological diagnosis of frontotemporal dementia. J Neuropathol Exp Neurol 60:1123-1126
38. Yokoo H, Oyama T, Hirato J, Sasaki A, Nakazato Y (1994) A case of Pick's disease with unusual neuronal inclusions. Acta Neuropathol 88:267-272