KEGG   PATHWAY: ko05014
ko05014                     Pathway                                
Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by a progressive degeneration of motor neurons in the brain and spinal cord. In 90% of patients, ALS is sporadic, with no clear genetic linkage. On the other hand, the remaining 10% of cases show familial inheritance, with mutations in SOD1, TDP43(TARDBP), FUS, or C9orf72 genes being the most frequent causes. In spite of such difference, familial ALS and sporadic ALS have similarities in their pathological features. Proposed disease mechanisms contributing to motor neuron degeneration in ALS are: impaired proteostasis, aberrant RNA processing, mitochondrial disfunction and oxidative stress, microglia activation, and axonal dysfunction.
Human Diseases; Neurodegenerative disease
Pathway map
ko05014  Amyotrophic lateral sclerosis

H00058  Amyotrophic lateral sclerosis (ALS)
K04451  tumor protein p53
K01370  caspase 1 [EC:]
K04726  BH3 interacting domain death agonist
K02161  apoptosis regulator Bcl-2
K04570  Bcl-2-like 1 (apoptosis regulator Bcl-X)
K02159  apoptosis regulator BAX
K02158  Bcl-2-antagonist of cell death
K02084  apoptotic protease-activating factor
K08738  cytochrome c
K04399  caspase 9 [EC:]
K02187  caspase 3 [EC:]
K04565  superoxide dismutase, Cu-Zn family [EC:]
K03878  NADH-ubiquinone oxidoreductase chain 1 [EC:]
K03879  NADH-ubiquinone oxidoreductase chain 2 [EC:]
K03880  NADH-ubiquinone oxidoreductase chain 3 [EC:]
K03881  NADH-ubiquinone oxidoreductase chain 4 [EC:]
K03882  NADH-ubiquinone oxidoreductase chain 4L [EC:]
K03883  NADH-ubiquinone oxidoreductase chain 5 [EC:]
K03884  NADH-ubiquinone oxidoreductase chain 6 [EC:]
K03942  NADH dehydrogenase (ubiquinone) flavoprotein 1 [EC:]
K03943  NADH dehydrogenase (ubiquinone) flavoprotein 2 [EC:]
K03944  NADH dehydrogenase (ubiquinone) flavoprotein 3
K03945  NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 1
K03946  NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 2
K03947  NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 3
K03948  NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 4
K03949  NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 5
K03950  NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 6
K03951  NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 7
K03952  NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 8
K03953  NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 9
K03954  NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 10
K03955  NADH dehydrogenase (ubiquinone) 1 alpha/beta subcomplex 1, acyl-carrier protein
K03956  NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 11
K11352  NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 12
K11353  NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 13
K03957  NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 1
K03958  NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 2
K03959  NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 3
K03960  NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 4
K03961  NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 5
K03962  NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 6
K03963  NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 7
K03964  NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 8
K03965  NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 9
K03966  NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 10
K11351  NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 11
K03934  NADH dehydrogenase (ubiquinone) Fe-S protein 1 [EC:]
K03935  NADH dehydrogenase (ubiquinone) Fe-S protein 2 [EC:]
K03936  NADH dehydrogenase (ubiquinone) Fe-S protein 3 [EC:]
K03937  NADH dehydrogenase (ubiquinone) Fe-S protein 4
K03938  NADH dehydrogenase (ubiquinone) Fe-S protein 5
K03939  NADH dehydrogenase (ubiquinone) Fe-S protein 6
K03940  NADH dehydrogenase (ubiquinone) Fe-S protein 7 [EC:]
K03941  NADH dehydrogenase (ubiquinone) Fe-S protein 8 [EC:]
K03967  NADH dehydrogenase (ubiquinone) 1 subunit C1
K03968  NADH dehydrogenase (ubiquinone) 1 subunit C2
K00234  succinate dehydrogenase (ubiquinone) flavoprotein subunit [EC:]
K00235  succinate dehydrogenase (ubiquinone) iron-sulfur subunit [EC:]
K00236  succinate dehydrogenase (ubiquinone) cytochrome b560 subunit
K00237  succinate dehydrogenase (ubiquinone) membrane anchor subunit
K00411  ubiquinol-cytochrome c reductase iron-sulfur subunit [EC:]
K00412  ubiquinol-cytochrome c reductase cytochrome b subunit
K00413  ubiquinol-cytochrome c reductase cytochrome c1 subunit
K00414  ubiquinol-cytochrome c reductase core subunit 1
K00415  ubiquinol-cytochrome c reductase core subunit 2
K00416  ubiquinol-cytochrome c reductase subunit 6
K00417  ubiquinol-cytochrome c reductase subunit 7
K00418  ubiquinol-cytochrome c reductase subunit 8
K00419  ubiquinol-cytochrome c reductase subunit 9
K00420  ubiquinol-cytochrome c reductase subunit 10
K02262  cytochrome c oxidase subunit 3
K02256  cytochrome c oxidase subunit 1 [EC:]
K02261  cytochrome c oxidase subunit 2
K02263  cytochrome c oxidase subunit 4
K02264  cytochrome c oxidase subunit 5a
K02265  cytochrome c oxidase subunit 5b
K02266  cytochrome c oxidase subunit 6a
K02267  cytochrome c oxidase subunit 6b
K02268  cytochrome c oxidase subunit 6c
K02270  cytochrome c oxidase subunit 7a
K02271  cytochrome c oxidase subunit 7b
K02272  cytochrome c oxidase subunit 7c
K02273  cytochrome c oxidase subunit 8
K02132  F-type H+-transporting ATPase subunit alpha
K02133  F-type H+-transporting ATPase subunit beta [EC:]
K02136  F-type H+-transporting ATPase subunit gamma
K02134  F-type H+-transporting ATPase subunit delta
K02135  F-type H+-transporting ATPase subunit epsilon
K02126  F-type H+-transporting ATPase subunit a
K02127  F-type H+-transporting ATPase subunit b
K02128  F-type H+-transporting ATPase subunit c
K02138  F-type H+-transporting ATPase subunit d
K02137  F-type H+-transporting ATPase subunit O
K02131  F-type H+-transporting ATPase subunit 6
K02125  F-type H+-transporting ATPase subunit 8
K05862  voltage-dependent anion channel protein 1
K11518  mitochondrial import receptor subunit TOM40
K20858  calcium uniporter protein, mitochondrial
K05688  PTEN induced putative kinase 1 [EC:]
K04556  parkin [EC:]
K19946  optineurin
K14381  sequestosome 1
K10435  microtubule-associated protein 1 light chain
K05410  TANK-binding kinase 1 [EC:]
K12650  TRAF family member-associated NF-kappa-B activator
K23610  WD repeat-containing protein 41
K23611  guanine nucleotide exchange protein SMCR8
K23609  guanine nucleotide exchange protein C9orf72
K07901  Ras-related protein Rab-8A
K07925  Ras-related protein Rab-39B
K07874  Ras-related protein Rab-1A
K07203  serine/threonine-protein kinase mTOR [EC:]
K21357  serine/threonine-protein kinase ULK1 [EC:]
K08269  serine/threonine-protein kinase ULK2 [EC:]
K19730  autophagy-related protein 101
K08331  autophagy-related protein 13
K17589  RB1-inducible coiled-coil protein 1
K17985  activating molecule in BECN1-regulated autophagy protein 1
K08334  beclin
K08333  phosphoinositide-3-kinase, regulatory subunit 4 [EC:]
K17889  beclin 1-associated autophagy-related key regulator
K21246  nuclear receptor-binding factor 2
K00914  phosphatidylinositol 3-kinase [EC:]
K17906  autophagy-related protein 2
K17908  autophagy-related protein 18
K04575  amyotrophic lateral sclerosis 2 protein
K07887  Ras-related protein Rab-5A
K04392  Ras-related C3 botulinum toxin substrate 1
K12192  charged multivesicular body protein 2B
K22913  phosphatidylinositol 3,5-bisphosphate 5-phosphatase [EC:3.1.3.-]
K19026  spatacsin
K04348  serine/threonine-protein phosphatase 2B catalytic subunit [EC:]
K06268  serine/threonine-protein phosphatase 2B regulatory subunit
K04523  ubiquilin
K13525  transitional endoplasmic reticulum ATPase
K11519  Derlin-1
K02730  20S proteasome subunit alpha 1 [EC:]
K02726  20S proteasome subunit alpha 2 [EC:]
K02728  20S proteasome subunit alpha 3 [EC:]
K02731  20S proteasome subunit alpha 4 [EC:]
K02729  20S proteasome subunit alpha 5 [EC:]
K02725  20S proteasome subunit alpha 6 [EC:]
K02727  20S proteasome subunit alpha 7 [EC:]
K02738  20S proteasome subunit beta 1 [EC:]
K02739  20S proteasome subunit beta 2 [EC:]
K02735  20S proteasome subunit beta 3 [EC:]
K02734  20S proteasome subunit beta 4 [EC:]
K02737  20S proteasome subunit beta 5 [EC:]
K02732  20S proteasome subunit beta 6 [EC:]
K02736  20S proteasome subunit beta 7 [EC:]
K03061  26S proteasome regulatory subunit T1
K03062  26S proteasome regulatory subunit T2
K03063  26S proteasome regulatory subunit T3
K03064  26S proteasome regulatory subunit T4
K03065  26S proteasome regulatory subunit T5
K03066  26S proteasome regulatory subunit T6
K03028  26S proteasome regulatory subunit N1
K03032  26S proteasome regulatory subunit N2
K03033  26S proteasome regulatory subunit N3
K06693  26S proteasome regulatory subunit N4
K03035  26S proteasome regulatory subunit N5
K03036  26S proteasome regulatory subunit N6
K03037  26S proteasome regulatory subunit N7
K03038  26S proteasome regulatory subunit N8
K03039  26S proteasome regulatory subunit N9
K03029  26S proteasome regulatory subunit N10
K03030  26S proteasome regulatory subunit N11
K03031  26S proteasome regulatory subunit N12
K06691  26S proteasome regulatory subunit N13
K10881  26 proteasome complex subunit DSS1
K11407  histone deacetylase 6 [EC:]
K23600  TAR DNA-binding protein 43
K13098  RNA-binding protein FUS
K12741  heterogeneous nuclear ribonucleoprotein A1/A3
K13158  heterogeneous nuclear ribonucleoprotein A2/B1
K23625  ataxin 2/2L
K13213  matrin 3
K16631  angiogenin [EC:3.1.27.-]
K12882  nuclear cap-binding protein subunit 1
K12881  THO complex subunit 4
K12892  serine/arginine-rich splicing factor 3
K12896  serine/arginine-rich splicing factor 7
K14284  nuclear RNA export factor
K14285  NTF2-related export protein 1/2
K18723  nucleoporin GLE1
K09291  nucleoprotein TPR
K14295  nuclear pore complex protein Nup50
K14296  nuclear pore complex protein Nup153
K14297  nuclear pore complex protein Nup98-Nup96
K14298  mRNA export factor
K14004  protein transport protein SEC13
K14299  nucleoporin SEH1
K14300  nuclear pore complex protein Nup133
K14301  nuclear pore complex protein Nup107
K14302  nuclear pore complex protein Nup37
K14303  nuclear pore complex protein Nup160
K14304  nuclear pore complex protein Nup85
K14305  nuclear pore complex protein Nup43
K14306  nuclear pore complex protein Nup62
K14307  nucleoporin p58/p45
K14308  nuclear pore complex protein Nup54
K14309  nuclear pore complex protein Nup93
K14310  nuclear pore complex protein Nup205
K14311  nuclear pore complex protein Nup188
K14312  nuclear pore complex protein Nup155
K14313  nuclear pore complex protein Nup53
K14314  nuclear pore complex protein Nup210
K14315  nucleoporin NDC1
K14316  nuclear pore complex protein Nup121
K14317  nuclear pore complex protein Nup214
K14318  nuclear pore complex protein Nup88
K12172  E3 SUMO-protein ligase RanBP2 [EC:2.3.2.-]
K10706  senataxin [EC:5.6.2.-]
K22759  coiled-coil-helix-coiled-coil-helix domain-containing protein 10
K09490  endoplasmic reticulum chaperone BiP [EC:]
K10707  vesicle-associated membrane protein-associated protein B
K09054  cyclic AMP-dependent transcription factor ATF-6 alpha
K04452  DNA damage-inducible transcript 3
K08852  serine/threonine-protein kinase/endoribonuclease IRE1 [EC: 3.1.26.-]
K09027  X box-binding protein 1
K03173  TNF receptor-associated factor 2 [EC:]
K04426  mitogen-activated protein kinase kinase kinase 5 [EC:]
K04741  caspase 12 [EC:3.4.22.-]
K08860  eukaryotic translation initiation factor 2-alpha kinase 3 [EC:]
K03237  translation initiation factor 2 subunit 1
K04374  cyclic AMP-dependent transcription factor ATF-4
K04960  inositol 1,4,5-triphosphate receptor type 3
K20719  sigma non-opioid intracellular receptor
K03156  tumor necrosis factor superfamily, member 2
K03158  tumor necrosis factor receptor superfamily member 1A
K05141  tumor necrosis factor receptor superfamily member 1B
K02308  death-associated protein 6
K04432  mitogen-activated protein kinase kinase 3 [EC:]
K04433  mitogen-activated protein kinase kinase 6 [EC:]
K04441  p38 MAP kinase [EC:]
K13240  nitric-oxide synthase, brain [EC:]
K04569  copper chaperone for superoxide dismutase
K00432  glutathione peroxidase [EC:]
K03781  catalase [EC:]
K07607  peripherin
K04572  neurofilament light polypeptide
K04573  neurofilament medium polypeptide (neurofilament 3)
K04574  neurofilament heavy polypeptide
K05759  profilin
K05692  actin beta/gamma 1
K17095  annexin A7/11
K10396  kinesin family member 5
K10407  kinesin light chain
K07374  tubulin alpha
K07375  tubulin beta
K04648  dynactin 1
K10424  dynactin 2
K10425  dynactin 3
K10426  dynactin 4
K10427  dynactin 5
K10428  dynactin 6
K16575  centractin
K16576  actin-related protein 10
K04647  huntingtin-associated protein 1
K10408  dynein axonemal heavy chain
K10410  dynein axonemal light intermediate chain 1
K10409  dynein axonemal intermediate chain 1
K11143  dynein axonemal intermediate chain 2
K10411  dynein axonemal light chain 1
K10412  dynein axonemal light chain 4
K05613  solute carrier family 1 (glial high affinity glutamate transporter), member 2
K13241  nitric-oxide synthase, inducible [EC:]
K05197  glutamate receptor 1
K05198  glutamate receptor 2
K05208  glutamate receptor ionotropic, NMDA 1
K05209  glutamate receptor ionotropic, NMDA 2A
K05210  glutamate receptor ionotropic, NMDA 2B
K05211  glutamate receptor ionotropic, NMDA 2C
K05212  glutamate receptor ionotropic, NMDA 2D
K05455  neuregulin 1
K05456  neuregulin 2
K05457  neuregulin 3
K05458  neuregulin 4
K05085  receptor tyrosine-protein kinase erbB-4 [EC:]
C00001  H2O
C00008  ADP
C00025  L-Glutamate
C00027  Hydrogen peroxide
C00039  DNA
C00046  RNA
C00062  L-Arginine
C00070  Copper
C00076  Calcium cation
C00533  Nitric oxide
C00704  Superoxide
C04549  1-Phosphatidyl-1D-myo-inositol 3-phosphate
C16844  Hydroxyl radical
C16845  Peroxynitrite
Brown RH, Al-Chalabi A
Amyotrophic Lateral Sclerosis.
N Engl J Med 377:162-172 (2017)
Webster CP, Smith EF, Shaw PJ, De Vos KJ
Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?
Front Mol Neurosci 10:123 (2017)
Valko K, Ciesla L
Amyotrophic lateral sclerosis.
Prog Med Chem 58:63-117 (2019)
Mathis S, Goizet C, Soulages A, Vallat JM, Masson GL
Genetics of amyotrophic lateral sclerosis: A review.
J Neurol Sci 399:217-226 (2019)
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
Controversies and priorities in amyotrophic lateral sclerosis.
Lancet Neurol 12:310-22 (2013)
Pasinelli P, Brown RH
Molecular biology of amyotrophic lateral sclerosis: insights from genetics.
Nat Rev Neurosci 7:710-23 (2006)
Goodall EF, Morrison KE
Amyotrophic lateral sclerosis (motor neuron disease): proposed mechanisms and pathways to treatment.
Expert Rev Mol Med 8:1-22 (2006)
Smith EF, Shaw PJ, De Vos KJ
The role of mitochondria in amyotrophic lateral sclerosis.
Neurosci Lett 710:132933 (2019)
Kodavati M, Wang H, Hegde ML
Altered Mitochondrial Dynamics in Motor Neuron Disease: An Emerging Perspective.
Cells 9:E1065 (2020)
Hervias I, Beal MF, Manfredi G
Mitochondrial dysfunction and amyotrophic lateral sclerosis.
Muscle Nerve 33:598-608 (2006)
Bacman SR, Bradley WG, Moraes CT
Mitochondrial involvement in amyotrophic lateral sclerosis: trigger or target?
Mol Neurobiol 33:113-31 (2006)
Lin MT, Beal MF
Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases.
Nature 443:787-95 (2006)
Bendotti C, Bao Cutrona M, Cheroni C, Grignaschi G, Lo Coco D, Peviani M, Tortarolo M, Veglianese P, Zennaro E
Inter- and intracellular signaling in amyotrophic lateral sclerosis: role of p38 mitogen-activated protein kinase.
Neurodegener Dis 2:128-34 (2005)
Simpson EP, Yen AA, Appel SH
Oxidative Stress: a common denominator in the pathogenesis of amyotrophic lateral sclerosis.
Curr Opin Rheumatol 15:730-6 (2003)
Bendotti C, Carri MT
Lessons from models of SOD1-linked familial ALS.
Trends Mol Med 10:393-400 (2004)
Robertson J, Kriz J, Nguyen MD, Julien JP
Pathways to motor neuron degeneration in transgenic mouse models.
Biochimie 84:1151-60 (2002)
Eve DJ, Dennis JS, Citron BA
Transcription factor p53 in degenerating spinal cords.
Brain Res 1150:174-81 (2007)
Bahar E, Kim H, Yoon H
ER Stress-Mediated Signaling: Action Potential and Ca(2+) as Key Players.
Int J Mol Sci 17:E1558 (2016)
Bezprozvanny I
Calcium signaling and neurodegenerative diseases.
Trends Mol Med 15:89-100 (2009)
von Lewinski F, Keller BU
Ca2+, mitochondria and selective motoneuron vulnerability: implications for ALS.
Trends Neurosci 28:494-500 (2005)
Julien JP.
Amyotrophic lateral sclerosis. unfolding the toxicity of the misfolded.
Cell 104:581-91 (2001)
Hand CK, Rouleau GA
Familial amyotrophic lateral sclerosis.
Muscle Nerve 25:135-59 (2002)
Barber SC, Mead RJ, Shaw PJ
Oxidative stress in ALS: a mechanism of neurodegeneration and a therapeutic target.
Biochim Biophys Acta 1762:1051-67 (2006)
Prasad A, Bharathi V, Sivalingam V, Girdhar A, Patel BK
Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis.
Front Mol Neurosci 12:25 (2019)
Nguyen DKH, Thombre R, Wang J
Autophagy as a common pathway in amyotrophic lateral sclerosis.
Neurosci Lett 697:34-48 (2019)
Lee JK, Shin JH, Lee JE, Choi EJ
Role of autophagy in the pathogenesis of amyotrophic lateral sclerosis.
Biochim Biophys Acta 1852:2517-24 (2015)
Oakes JA, Davies MC, Collins MO
TBK1: a new player in ALS linking autophagy and neuroinflammation.
Mol Brain 10:5 (2017)
Ramesh N, Pandey UB
Autophagy Dysregulation in ALS: When Protein Aggregates Get Out of Hand.
Front Mol Neurosci 10:263 (2017)
Kunita R, Otomo A, Mizumura H, Suzuki-Utsunomiya K, Hadano S, Ikeda JE
The Rab5 activator ALS2/alsin acts as a novel Rac1 effector through Rac1-activated endocytosis.
J Biol Chem 282:16599-611 (2007)
Hadano S, Kunita R, Otomo A, Suzuki-Utsunomiya K, Ikeda JE
Molecular and cellular function of ALS2/alsin: implication of membrane dynamics in neuronal development and degeneration.
Neurochem Int 51:74-84 (2007)
Ruffoli R, Bartalucci A, Frati A, Fornai F
Ultrastructural studies of ALS mitochondria connect altered function and permeability with defects of mitophagy and mitochondriogenesis.
Front Cell Neurosci 9:341 (2015)
Menzies FM, Fleming A, Rubinsztein DC
Compromised autophagy and neurodegenerative diseases.
Nat Rev Neurosci 16:345-57 (2015)
Casterton RL, Hunt RJ, Fanto M
Pathomechanism Heterogeneity in the Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Disease Spectrum: Providing Focus Through the Lens of Autophagy.
J Mol Biol 432:2692-2713 (2020)
Lin G, Mao D, Bellen HJ
Amyotrophic Lateral Sclerosis Pathogenesis Converges on Defects in Protein Homeostasis Associated with TDP-43 Mislocalization and Proteasome-Mediated Degradation Overload.
Curr Top Dev Biol 121:111-171 (2017)
Hetz C, Mollereau B
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
Nat Rev Neurosci 15:233-49 (2014)
Xiang C, Wang Y, Zhang H, Han F
The role of endoplasmic reticulum stress in neurodegenerative disease.
Apoptosis 22:1-26 (2017)
Hughes D, Mallucci GR
The unfolded protein response in neurodegenerative disorders - therapeutic modulation of the PERK pathway.
FEBS J 286:342-355 (2019)
Doyle KM, Kennedy D, Gorman AM, Gupta S, Healy SJ, Samali A
Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders.
J Cell Mol Med 15:2025-39 (2011)
Turner BJ, Atkin JD
ER stress and UPR in familial amyotrophic lateral sclerosis.
Curr Mol Med 6:79-86 (2006)
Nishitoh H, Kadowaki H, Nagai A, Maruyama T, Yokota T, Fukutomi H, Noguchi T, Matsuzawa A, Takeda K, Ichijo H
ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1.
Genes Dev 22:1451-64 (2008)
Walker AK, Atkin JD
Stress signaling from the endoplasmic reticulum: A central player in the pathogenesis of amyotrophic lateral sclerosis.
IUBMB Life 63:754-63 (2011)
Renaud L, Picher-Martel V, Codron P, Julien JP
Key role of UBQLN2 in pathogenesis of amyotrophic lateral sclerosis and frontotemporal dementia.
Acta Neuropathol Commun 7:103 (2019)
Nguyen HP, Van Broeckhoven C, van der Zee J
ALS Genes in the Genomic Era and their Implications for FTD.
Trends Genet 34:404-423 (2018)
Atkin G, Paulson H
Ubiquitin pathways in neurodegenerative disease.
Front Mol Neurosci 7:63 (2014)
Kim JM, Billington E, Reyes A, Notarianni T, Sage J, Agbas E, Taylor M, Monast I, Stanford JA, Agbas A
Impaired Cu-Zn Superoxide Dismutase (SOD1) and Calcineurin (Cn) Interaction in ALS: A Presumed Consequence for TDP-43 and Zinc Aggregation in Tg SOD1(G93A) Rodent Spinal Cord Tissue.
Neurochem Res 44:228-233 (2019)
Benarroch EE
Sigma-1 receptor and amyotrophic lateral sclerosis.
Neurology 91:743-747 (2018)
Millecamps S, Julien JP
Axonal transport deficits and neurodegenerative diseases.
Nat Rev Neurosci 14:161-76 (2013)
ko00190  Oxidative phosphorylation
ko03013  Nucleocytoplasmic transport
ko03050  Proteasome
ko04010  MAPK signaling pathway
ko04012  ErbB signaling pathway
ko04020  Calcium signaling pathway
ko04115  p53 signaling pathway
ko04137  Mitophagy - animal
ko04140  Autophagy - animal
ko04141  Protein processing in endoplasmic reticulum
ko04210  Apoptosis
ko04724  Glutamatergic synapse
ko04810  Regulation of actin cytoskeleton

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