Can regional spreading of amyotrophic lateral sclerosis motor symptoms be explained by prion-like propagation?

Journal of Neurology, Neurosurgery and Psychiatry

Volumn 83, Issue 7, 2012, Pages 739-745

  Kanouchi, Tadashi ^a   Ohkubo, Takuya ^a   Yokota, Takanori ^a  

^a TOKYO MEDICAL AND DENTAL UNIVERSITY   (Japan)

Author keywords

[No Author keywords available]

Indexed keywords

ALPHA SYNUCLEIN; COPPER ZINC SUPEROXIDE DISMUTASE; HUNTINGTIN; TAR DNA BINDING PROTEIN; TAU PROTEIN;

ALZHEIMER DISEASE; AMYOTROPHIC LATERAL SCLEROSIS; CLINICAL FEATURE; CREUTZFELDT JAKOB DISEASE; DISEASE COURSE; DISEASE SEVERITY; HUMAN; HUNTINGTON CHOREA; MOLECULAR PATHOLOGY; NONHUMAN; PARKINSON DISEASE; PRION; PRIORITY JOURNAL; PROTEIN FOLDING; PROTEIN TRANSPORT; REVIEW; SYMPTOM;

EID: 84861841901     PISSN: 00223050     EISSN: 1468330X     Source Type: Journal    
DOI: 10.1136/jnnp-2011-301826     Document Type: Review

References (45)

1. Kiernan M, Vucic S, Cheah B, et al. Amyotrophic lateral sclerosis. Lancet 2011;377:942-55.
2. Arai T, Hasegawa M, Akiyama H, et al. TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Biochem Biophys Res Commun 2006;351:602-11. (Pubitemid 44708852)
3. Neumann M, Sampathu DM, Kwong LK, et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 2006;314:130-3. (Pubitemid 44547757)
4. Uchida A, Sasaguri H, Kimura N, et al. Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43. Brain 2012;135:833-46.
5. Hasegawa M, Nonaka T, Tsuji H, et al. Molecular dissection of TDP-43 proteinopathies. J Mol Neurosci 2011;45:480-5.
6. Münch C, O'Brien J, Bertolotti A. Prion-like propagation of mutant superoxide dismutase-1 misfolding in neuronal cells. Proc Natl Acad Sci U S A 2011;108:3548-53.
7. Johnson BS, Snead D, Lee JJ, et al. TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity. J Biol Chem 2009;284:20329-39.
8. Furukawa Y, Kaneko K, Watanabe S, et al. A seeding reaction recapitulates intracellular formation of Sarkosyl-insoluble transactivation response element (TAR) DNA-binding protein-43 inclusions. J Biol Chem 2011;286:18664-72.
9. Pesiridis GS, Tripathy K, Tanik S, et al. A "two-hit" hypothesis for inclusion formation by carboxyl-terminal fragments of TDP-43 protein linked to RNA depletion and impaired microtubule-dependent transport. J Biol Chem 2011;286:18845-55.
10. Colombrita C, Zennaro E, Fallini C, et al. TDP-43 is recruited to stress granules in conditions of oxidative insult. J Neurochem 2009;111:1051-61.
11. Grad LI, Guest WC, Yanai A, et al. Intermolecular transmission of superoxide dismutase 1 misfolding in living cells. Proc Natl Acad Sci U S A 2011;108:116398-403.
12. Urushitani M, Sik A, Sakurai T, et al. Chromogranin-mediated secretion of mutant superoxide dismutase proteins linked to amyotrophic lateral sclerosis. Nat Neurosci 2006;9:108-18. (Pubitemid 43011916)
13. Urushitani M, Ezzi SA, Julien JP. Therapeutic effects of immunization with mutant superoxide dismutase in mice models of amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A 2007;104:2495-500. (Pubitemid 46391424)
14. Boillée S, Yamanaka K, Lobsiger CS, et al. Onset and progression in inherited ALS determined by motor neurons and microglia. Science 2006;312:1389-92. (Pubitemid 43839552)
15. Yamanaka K, Chun SJ, Boillee S, et al. Astrocytes as determinants of disease progression in inherited amyotrophic lateral sclerosis. Nat Neurosci 2008;11:251-3. (Pubitemid 351311795)
16. Papadeas ST, Kraig SE, O'Banion C, et al. Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo. Proc Natl Acad Sci U S A 2011;108:17803-8.
17. Haidet-Phillips AM, Hester ME, Miranda CJ, et al. Astrocytes from familial and sporadic ALS patients are toxic to motor neurons. Nat Biotechnol 2011;29:824-8.
18. Brooks BR, Sufit RL, DePaul R, et al. Design of clinical therapeutic trials in amyotrophic lateral sclerosis. In: Rowland LP, ed. Amyotrophic lateral sclerosis and other motor neuron disease. Adv Neurol 1991;56:521-645.
19. Ravits J, Paul P, Jorg C. Focality of upper and lower motor neuron degeneration at the clinical onset of ALS. Neurology 2007;68:1571-5. (Pubitemid 46717980)
20. Körner S, Kollewe K, Fahlbusch M, et al. Onset and spreading patterns of upper and lower motor neuron symptoms in amyotrophic lateral sclerosis. Muscle Nerve 2011;43:636-42.
21. Fujimura-Kiyono C, Kimura F, Ishida S, et al. Onset and spreading patterns of lower neuron involvements predict survival in sporadic amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 2011;82:1244-9.
22. Haverkamp LJ, Appel V, Appel SH. Natural history of amyotrophic lateral sclerosis in a database population. Validation of a scoring system and a model for survival prediction. Brain 1995;118:707-19.
23. Turner MR, Wicks P, Brownstein CA, et al. Concordance between site of onset and limb dominance in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 2011;82:853-4.
24. Velding JH, Kalmijin S, Groeneveld GJ, et al. Physical activity and the association with sporadic ALS. Neurology 2005;64:241-5.
25. Swash M, Leader M, Brown A, et al. Focal loss of anterior horn cells in the cervical cord in motor neuron disease. Brain 1986;109:939-52. (Pubitemid 17175612)
26. Tsukagoshi H, Yanagisawa N, Oguchi K. Morphometric quantification of the cervical limb motor cells in various neuromuscular disease. J Neurol Sci 1980;47:463-72. (Pubitemid 10059362)
27. de Carvalho MA, Pinto S, Swash M. Paraspinal and limb motor neuron involvement within homologous spinal segments in ALS. Clin Neurophysiol 2008;119:1607-13.
28. Kuwabara S, Sonoo M, Komori T, et al Neuromuscular Electrodiagnosis Study Group. Dissociated small hand muscle atrophy in amyotrophic lateral sclerosis: frequency, extent, and specificity. Muscle Nerve 2008;37:426-30. (Pubitemid 351501967)
29. Bae JS, Sawai S, Misawa S, et al. Differences in excitability properties of FDI and ADM motor axons. Muscle Nerve 2009;39:350-4.
30. Pun S, Santos AF, Saxena S, et al. Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF. Nat Neurosci 2006;9:408-19. (Pubitemid 43290971)
31. Dengler R, Konstanzer A, Kuther G, et al. Amyotrophic lateral sclerosis: macro-EMG and twithch forces of single motor units. Muscle Nerve 1990;13:545-50. (Pubitemid 20173585)
32. Ravits JM, La Spada AR. ALS motor phenotype heterogeneity, focality, and deconstructing motor neuron degeneration. Neurology 2009;73:805-11.
33. Ravits JM, Laurie P, Fan Y, et al. Implication of ALS focality: rostral-caudal distribution of lower motor neuron loss postmortem. Neurology 2007;68:1576-82. (Pubitemid 46717981)
34. Turner MR, Brockington A, Scaber J, et al. Pattern of spread and prognosis in lower limb-onset ALS. Amyotroph Lateral Scler 2010;11:369-73.
35. Gargiulo-Monachelli GM, Janota F, Bettini M, et al. Regional spread pattern predicts survival in patients with sporadic amyotrophic lateral sclerosis. Eur J Neurol 2012;19:834-41.
36. Shinoda Y, Zarzecki P, Asanuma H. Spinal branching of pyramidal tract neurons in the monkey. Exp Brain Res 1979;34:59-72. (Pubitemid 9212838)
37. Kiernan JA, Hudson AJ. Changes in sizes of cortical and lower motor neurons in amyotrophic lateral sclerosis. Brain 1991;114:843-53.
38. Pamphlett R, Kril J, Hng TM. Motor neuron disease: a primary disorder of corticomotoneurons? Muscle Nerve 1995;18:314-18.
39. Attarian S, Vedel JP, Pouget J, et al. Progression of cortical and spinal dysfunctions over time in amyotrophic lateral sclerosis. Muscle Nerve 2008;37:364-75. (Pubitemid 351304797)
40. de Carvalho M, Pinto S, Swash M. Does the motor cortex influence denervation in ALS? EMG studies of muscles with both contralateral and bilateral corticospinal innervation. Clin Neurophysiol 2011;122:629-35.
41. Yokota T, Yoshino A, Inaba A, et al. Double cortical stimulation in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry 1996;61:596-600. (Pubitemid 27023337)
42. Vucic S, Kiernan MC. Novel threshould tracking techniques suggest that cortical hyperexcitability is an early feature of motor neuron disease. Brain 2006;129:2436-46. (Pubitemid 44522128)
43. Vucic S, Nicholson GA, Kiernan MC. Cortical hyperexcitability may precede the onset of familial amyotrophic lateral sclerosis. Brain 2008;131:1540-50.
44. Weber M, Eisen A, Stewart H, et al. The split hand in ALS has cortical basis. J Neurol Sci 2000;180:66-70.
45. Priori A, Oliviero A, Donati E, et al. Human handedness and asymmetry of the motor cortical silent period. Exp Brain Res 1999;128:390-6. (Pubitemid 29450192)