Two superoxide dismutase prion strains transmit amyotrophic lateral sclerosis-like disease

Journal of Clinical Investigation

Volumn 126, Issue 6, 2016, Pages 2249-2253

  Ekhtiari Bidhendi, Elaheh ^a   Bergh, Johan ^a   Zetterström, Per ^a   Andersen, Peter M ^a   Marklund, Stefan L ^a   Brännström, Thomas ^a  

^a UMEÅ UNIVERSITY   (Sweden)

Author keywords

[No Author keywords available]

Indexed keywords

PROTEIN AGGREGATE; SUPEROXIDE DISMUTASE; COPPER ZINC SUPEROXIDE DISMUTASE; MUTANT PROTEIN; PRION; RECOMBINANT PROTEIN; SOD1 PROTEIN, HUMAN;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMAL TISSUE; ARTICLE; BRAIN STEM; CENTRIFUGATION; CONTROLLED STUDY; DISEASE COURSE; EPITOPE MAPPING; HISTOPATHOLOGY; INOCULATION; LUMBAR SPINAL CORD; MOLECULAR PATHOLOGY; MOTONEURON; MOUSE; NERVE FIBER; NONHUMAN; PHENOTYPE; PRION; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN EXPRESSION; SPINAL CORD; VIRUS STRAIN; ANIMAL; C57BL MOUSE; CHEMISTRY; DISEASE MODEL; GENETICS; HUMAN; METABOLISM; PATHOLOGY; PROTEINOSIS; TRANSGENIC MOUSE;

AMYOTROPHIC LATERAL SCLEROSIS; ANIMALS; DISEASE MODELS, ANIMAL; HUMANS; MICE; MICE, INBRED C57BL; MICE, TRANSGENIC; MOTOR NEURONS; MUTANT PROTEINS; PRIONS; PROTEIN AGGREGATES; PROTEIN AGGREGATION, PATHOLOGICAL; RECOMBINANT PROTEINS; SPINAL CORD; SUPEROXIDE DISMUTASE-1;

EID: 84974653241     PISSN: 00219738     EISSN: 15588238     Source Type: Journal    
DOI: 10.1172/JCI84360     Document Type: Article

References (15)

1. Charcot JM. Lecons sur les maladies du système nerveux. 2nd series, collected by Bourneville 1873. In: Charcot JM, Sigerson G, eds. Lectures On The Diseases Of The Nervous System. Vol. 2. London, United Kingdom: New Sydenham Society; 1881:163-204.
2. Rosen DR, et al. Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature. 1993;362(6415):59-62.
3. Wroe R, Wai-Ling Butler A, Andersen PM, Powell JF, Al-Chalabi A. ALSOD: the Amyotrophic Lateral Sclerosis Online Database. Amyotroph Lateral Scler. 2008;9(4):249-250.
4. Kato S, et al. New consensus research on neuropathological aspects of familial amyotrophic lateral sclerosis with superoxide dismutase 1 (SOD1) gene mutations: inclusions containing SOD1 in neurons and astrocytes. Amyotroph Lateral Scler Other Motor Neuron Disord. 2000;1(3):163-184.
5. Grad LI, et al. Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms. Proc Natl Acad Sci U S A. 2014;111(9):3620-3625.
6. Munch 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(9):3548-3553.
7. Ayers JI, et al. Experimental transmissibility of mutant SOD1 motor neuron disease. Acta Neuropathol. 2014;128(6):791-803.
8. Ayers JI, Fromholt SE, O'Neal VM, Diamond JH, Borchelt DR. Prion-like propagation of mutant SOD1 misfolding and motor neuron disease spread along neuroanatomical pathways. Acta Neuropathol. 2016;131(1):103-114.
9. Bergh J, et al. Structural and kinetic analysis of protein-aggregate strains in vivo using binary epitope mapping. Proc Natl Acad Sci U S A. 2015;112(14):4489-4494.
10. Grad LI, et al. Intermolecular transmission of superoxide dismutase 1 misfolding in living cells. Proc Natl Acad Sci U S A. 2011;108(39):16398-16403.
11. Bruijn LI, et al. ALS-linked SOD1 mutant G85R mediates damage to astrocytes and promotes rapidly progressive disease with SOD1-containing inclusions. Neuron. 1997;18(2):327-338.
12. Jonsson PA, et al. Disulphide-reduced superoxide dismutase-1 in CNS of transgenic amyotrophic lateral sclerosis models. Brain. 2006;129(pt 2):451-464.
13. Zetterstrom P, et al. Soluble misfolded subfractions of mutant superoxide dismutase-1s are enriched in spinal cords throughout life in murine ALS models. Proc Natl Acad Sci U S A. 2007;104(35):14157-14162.
14. Tokuda E, Brannstrom T, Andersen PM, Marklund SL. Low autophagy capacity implicated in motor system vulnerability to mutant superoxide dismutase. Acta Neuropathol Commun. 2016;4(1):6.
15. Watts JC, Prusiner SB. Mouse models for studying the formation and propagation of prions. J Biol Chem. 2014;289(29):19841-19849.