ER strikes again: Proteostasis Dysfunction in ALS

EMBO Journal

Volumn 35, Issue 8, 2016, Pages 798-800

  Maharjan, Niran ^a   Saxena, Smita ^a  

^a UNIVERSITY OF BERN   (Switzerland)

Author keywords

[No Author keywords available]

Indexed keywords

SUPEROXIDE DISMUTASE; P4HB PROTEIN, HUMAN; PDIA3 PROTEIN, HUMAN; PROCOLLAGEN PROLINE 2 OXOGLUTARATE 4 DIOXYGENASE; PROTEIN DISULFIDE ISOMERASE;

AMYOTROPHIC LATERAL SCLEROSIS; CELL DISRUPTION; CELL MIGRATION; CELL STRUCTURE; ENDOPLASMIC RETICULUM; ENDOPLASMIC RETICULUM STRESS; HUMAN; MOTONEURON; NEUROMUSCULAR SYNAPSE; NEUROPATHOLOGY; NONHUMAN; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN EXPRESSION; PROTEIN FOLDING; PROTEIN HOMEOSTASIS; PROTEIN QUALITY; PROTEIN SYNTHESIS; REVIEW; RNA PROCESSING; TRANSLATION INITIATION; UNFOLDED PROTEIN RESPONSE; GENETICS; METABOLISM; PATHOLOGY; PHYSIOLOGY; PROTEOSTASIS DEFICIENCY;

AMYOTROPHIC LATERAL SCLEROSIS; ENDOPLASMIC RETICULUM; ENDOPLASMIC RETICULUM STRESS; HUMANS; PROCOLLAGEN-PROLINE DIOXYGENASE; PROTEIN DISULFIDE-ISOMERASES; PROTEOSTASIS DEFICIENCIES;

EID: 84960540425     PISSN: 02614189     EISSN: 14602075     Source Type: Journal    
DOI: 10.15252/embj.201694117     Document Type: Review

References (12)

1. Atkin JD, Farg MA, Walker AK, McLean C, Tomas D, Horne MK, (2008) Endoplasmic reticulum stress and induction of the unfolded protein response in human sporadic amyotrophic lateral sclerosis. Neurobiol Dis 30: 400-407
2. Gonzalez-Perez P, Woehlbier U, Chian RJ, Sapp P, Rouleau GA, Leblond CS, Daoud H, Dion PA, Landers JE, Hetz C, Brown RH, (2015) Identification of rare protein disulfide isomerase gene variants in amyotrophic lateral sclerosis patients. Gene 566: 158-165
3. Hetz C, Chevet E, Oakes SA, (2015) Proteostasis control by the unfolded protein response. Nat Cell Biol 17: 829-838
4. Kiskinis E, Sandoe J, Williams LA, Boulting GL, Moccia R, Wainger BJ, Han S, Peng T, Thams S, Mikkilineni S, Mellin C, Merkle FT, Davis-Dusenbery BN, Ziller M, Oakley D, Ichida J, Di Costanzo S, Atwater N, Maeder ML, Goodwin MJ, et al, (2014) Pathways disrupted in human ALS motor neurons identified through genetic correction of mutant SOD1. Cell Stem Cell 14: 781-795
5. Ma T, Trinh MA, Wexler AJ, Bourbon C, Gatti E, Pierre P, Cavener DR, Klann E, (2013) Suppression of eIF2α kinases alleviates Alzheimer's disease-related plasticity and memory deficits. Nat Neurosci 16: 1299-1305
6. Moreno JA, Radford H, Peretti D, Steinert JR, Verity N, Martin MG, Halliday M, Morgan J, Dinsdale D, Ortori CA, Barrett DA, Tsaytler P, Bertolotti A, Willis AE, Bushell M, Mallucci GR, (2012) Sustained translational repression by eIF2α-P mediates prion neurodegeneration. Nature 485: 507-511
7. Perri ER, Thomas CJ, Parakh S, Spencer DM, Atkin JD, (2016) The unfolded protein response and the role of protein disulfide isomerase in Neurodegeneration. Front Cell Dev Biol 3: 80
8. Peters OM, Ghasemi M, Brown R.H. Jr, (2015) Emerging mechanisms of molecular pathology in ALS. J Clin Invest 125: 1767-1779
9. Pun S, Santos AF, Saxena S, Xu L, Caroni P, (2006) Selective vulnerability and pruning of phasic motoneuron axons in motoneuron disease alleviated by CNTF. Nat Neurosci 9: 408-419
10. Saxena S, Cabuy E, Caroni P, (2009) A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice. Nat Neurosci 12: 627-636
11. Turner MR, Hardiman O, Benatar M, Brooks BR, Chio A, de Carvalho M, Ince PG, Lin C, Miller RG, Mitsumoto H, Nicholson G, Ravits J, Shaw PJ, Swash M, Talbot K, Traynor BJ, Van den Berg LH, Veldink JH, Vucic S, Kiernan MC, (2013) Controversies and priorities in amyotrophic lateral sclerosis. Lancet Neurol 12: 310-322
12. Woehlbier U, Colombo A, Saaranen MJ, Pérez V, Ojeda J, Bustos FJ, Andreu CI, Torres M, Valenzuela V, Medinas DB, Rozas P, Vidal RL, Lopez-Gonzalez R, Salameh J, Fernandez-Collemann S, Muñoz N, Matus S, Armisen R, Sagredo A, Palma K, et al, (2016) ALS-linked protein disulfide isomerase variants cause motor dysfunction. EMBO J 35: 845-865