PATHWAY: mmu05014 Help
Entry
Name
Amyotrophic lateral sclerosis - Mus musculus (house mouse)
Description
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.
Class
Human Diseases; Neurodegenerative disease
BRITE hierarchy
Pathway map
Ortholog table
Organism
Mus musculus (house mouse) [GN:
mmu ]
Gene
22059 Trp53; transformation related protein 53 [KO:K04451 ]
12122 Bid; BH3 interacting domain death agonist [KO:K04726 ]
12015 Bad; BCL2-associated agonist of cell death [KO:K02158 ]
11783 Apaf1; apoptotic peptidase activating factor 1 [KO:K02084 ]
78330 Ndufv3; NADH:ubiquinone oxidoreductase core subunit V3 [KO:K03944 ]
54405 Ndufa1; NADH:ubiquinone oxidoreductase subunit A1 [KO:K03945 ]
17991 Ndufa2; NADH:ubiquinone oxidoreductase subunit A2 [KO:K03946 ]
66091 Ndufa3; NADH:ubiquinone oxidoreductase subunit A3 [KO:K03947 ]
17992 Ndufa4; Ndufa4, mitochondrial complex associated [KO:K03948 ]
407790 Ndufa4l2; Ndufa4, mitochondrial complex associated like 2 [KO:K03948 ]
68202 Ndufa5; NADH:ubiquinone oxidoreductase subunit A5 [KO:K03949 ]
67130 Ndufa6; NADH:ubiquinone oxidoreductase subunit A6 [KO:K03950 ]
66416 Ndufa7; NADH:ubiquinone oxidoreductase subunit A7 [KO:K03951 ]
68375 Ndufa8; NADH:ubiquinone oxidoreductase subunit A8 [KO:K03952 ]
66108 Ndufa9; NADH:ubiquinone oxidoreductase subunit A9 [KO:K03953 ]
67273 Ndufa10; NADH:ubiquinone oxidoreductase subunit A10 [KO:K03954 ]
70316 Ndufab1; NADH:ubiquinone oxidoreductase subunit AB1 [KO:K03955 ]
102634451 Ndufab1-ps; NADH:ubiquinone oxidoreductase subunit AB1b [KO:K03955 ]
69875 Ndufa11; NADH:ubiquinone oxidoreductase subunit A11 [KO:K03956 ]
66414 Ndufa12; NADH:ubiquinone oxidoreductase subunit A12 [KO:K11352 ]
67184 Ndufa13; NADH:ubiquinone oxidoreductase subunit A13 [KO:K11353 ]
68198 Ndufb2; NADH:ubiquinone oxidoreductase subunit B2 [KO:K03958 ]
66495 Ndufb3; NADH:ubiquinone oxidoreductase subunit B3 [KO:K03959 ]
68194 Ndufb4; NADH:ubiquinone oxidoreductase subunit B4 [KO:K03960 ]
66046 Ndufb5; NADH:ubiquinone oxidoreductase subunit B5 [KO:K03961 ]
230075 Ndufb6; NADH:ubiquinone oxidoreductase subunit B6 [KO:K03962 ]
66916 Ndufb7; NADH:ubiquinone oxidoreductase subunit B7 [KO:K03963 ]
67264 Ndufb8; NADH:ubiquinone oxidoreductase subunit B8 [KO:K03964 ]
66218 Ndufb9; NADH:ubiquinone oxidoreductase subunit B9 [KO:K03965 ]
68342 Ndufb10; NADH:ubiquinone oxidoreductase subunit B10 [KO:K03966 ]
104130 Ndufb11; NADH:ubiquinone oxidoreductase subunit B11 [KO:K11351 ]
17993 Ndufs4; NADH:ubiquinone oxidoreductase core subunit S4 [KO:K03937 ]
595136 Ndufs5; NADH:ubiquinone oxidoreductase core subunit S5 [KO:K03938 ]
407785 Ndufs6; NADH:ubiquinone oxidoreductase core subunit S6 [KO:K03939 ]
623286 Ndufs6b; NADH:ubiquinone oxidoreductase subunit S6B [KO:K03939 ]
66377 Ndufc1; NADH:ubiquinone oxidoreductase subunit C1 [KO:K03967 ]
68197 Ndufc2; NADH:ubiquinone oxidoreductase subunit C2 [KO:K03968 ]
66945 Sdha; succinate dehydrogenase complex, subunit A, flavoprotein (Fp) [KO:K00234 ] [EC:1.3.5.1 ]
67680 Sdhb; succinate dehydrogenase complex, subunit B, iron sulfur (Ip) [KO:K00235 ] [EC:1.3.5.1 ]
66052 Sdhc; succinate dehydrogenase complex, subunit C, integral membrane protein [KO:K00236 ]
66925 Sdhd; succinate dehydrogenase complex, subunit D, integral membrane protein [KO:K00237 ]
66694 Uqcrfs1; ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1 [KO:K00411 ] [EC:7.1.1.8 ]
22273 Uqcrc1; ubiquinol-cytochrome c reductase core protein 1 [KO:K00414 ]
67003 Uqcrc2; ubiquinol cytochrome c reductase core protein 2 [KO:K00415 ]
66576 Uqcrh; ubiquinol-cytochrome c reductase hinge protein [KO:K00416 ]
67530 Uqcrb; ubiquinol-cytochrome c reductase binding protein [KO:K00417 ]
22272 Uqcrq; ubiquinol-cytochrome c reductase, complex III subunit VII [KO:K00418 ]
66152 Uqcr10; ubiquinol-cytochrome c reductase, complex III subunit X [KO:K00419 ]
66594 Uqcr11; ubiquinol-cytochrome c reductase, complex III subunit XI [KO:K00420 ]
17710 COX3; cytochrome c oxidase subunit III [KO:K02262 ]
17709 COX2; cytochrome c oxidase subunit II [KO:K02261 ]
12857 Cox4i1; cytochrome c oxidase subunit 4I1 [KO:K02263 ]
84682 Cox4i2; cytochrome c oxidase subunit 4I2 [KO:K02263 ]
12858 Cox5a; cytochrome c oxidase subunit 5A [KO:K02264 ]
12859 Cox5b; cytochrome c oxidase subunit 5B [KO:K02265 ]
12861 Cox6a1; cytochrome c oxidase subunit 6A1 [KO:K02266 ]
12862 Cox6a2; cytochrome c oxidase subunit 6A2 [KO:K02266 ]
110323 Cox6b1; cytochrome c oxidase, subunit 6B1 [KO:K02267 ]
12864 Cox6c; cytochrome c oxidase subunit 6C [KO:K02268 ]
12866 Cox7a2; cytochrome c oxidase subunit 7A2 [KO:K02270 ]
12865 Cox7a1; cytochrome c oxidase subunit 7A1 [KO:K02270 ]
20463 Cox7a2l; cytochrome c oxidase subunit 7A2 like [KO:K02270 ]
66142 Cox7b; cytochrome c oxidase subunit 7B [KO:K02271 ]
78174 Cox7b2; cytochrome c oxidase subunit 7B2 [KO:K02271 ]
12867 Cox7c; cytochrome c oxidase subunit 7C [KO:K02272 ]
12868 Cox8a; cytochrome c oxidase subunit 8A [KO:K02273 ]
12869 Cox8b; cytochrome c oxidase subunit 8B [KO:K02273 ]
75483 Cox8c; cytochrome c oxidase subunit 8C [KO:K02273 ]
11946 Atp5f1a; ATP synthase F1 subunit alpha [KO:K02132 ]
11949 Atp5f1c; ATP synthase F1 subunit gamma [KO:K02136 ]
66043 Atp5f1d; ATP synthase F1 subunit delta [KO:K02134 ]
67126 Atp5f1e; ATP synthase F1 subunit epsilon [KO:K02135 ]
11950 Atp5pb; ATP synthase peripheral stalk-membrane subunit b [KO:K02127 ]
11951 Atp5mc1; ATP synthase membrane subunit c locus 1 [KO:K02128 ]
67942 Atp5mc2; ATP synthase membrane subunit c locus 2 [KO:K02128 ]
228033 Atp5mc3; ATP synthase membrane subunit c locus 3 [KO:K02128 ]
71679 Atp5pd; ATP synthase peripheral stalk subunit d [KO:K02138 ]
28080 Atp5po; ATP synthase peripheral stalk subunit OSCP [KO:K02137 ]
11957 Atp5pf; ATP synthase peripheral stalk subunit F6 [KO:K02131 ]
22333 Vdac1; voltage-dependent anion channel 1 [KO:K05862 ]
53333 Tomm40; translocase of outer mitochondrial membrane 40 [KO:K11518 ]
641376 Tomm40l; translocase of outer mitochondrial membrane 40-like [KO:K11518 ]
66734 Map1lc3a; microtubule-associated protein 1 light chain 3 alpha [KO:K10435 ]
67443 Map1lc3b; microtubule-associated protein 1 light chain 3 beta [KO:K10435 ]
21353 Tank; TRAF family member-associated Nf-kappa B activator [KO:K12650 ]
237782 Smcr8; Smith-Magenis syndrome chromosome region, candidate 8 homolog (human) [KO:K23611 ]
73205 C9orf72; C9orf72, member of C9orf72-SMCR8 complex [KO:K23609 ]
17274 Rab8a; RAB8A, member RAS oncogene family [KO:K07901 ]
67790 Rab39b; RAB39B, member RAS oncogene family [KO:K07925 ]
19324 Rab1a; RAB1A, member RAS oncogene family [KO:K07874 ]
52639 Wipi1; WD repeat domain, phosphoinositide interacting 1 [KO:K17908 ]
74781 Wipi2; WD repeat domain, phosphoinositide interacting 2 [KO:K17908 ]
74018 Als2; alsin Rho guanine nucleotide exchange factor [KO:K04575 ]
68942 Chmp2b; charged multivesicular body protein 2B [KO:K12192 ]
103199 Fig4; FIG4 phosphoinositide 5-phosphatase [KO:K22913 ] [EC:3.1.3.-]
214585 Spg11; SPG11, spatacsin vesicle trafficking associated [KO:K19026 ]
19057 Ppp3cc; protein phosphatase 3, catalytic subunit, gamma isoform [KO:K04348 ] [EC:3.1.3.16 ]
19055 Ppp3ca; protein phosphatase 3, catalytic subunit, alpha isoform [KO:K04348 ] [EC:3.1.3.16 ]
19058 Ppp3r1; protein phosphatase 3, regulatory subunit B, alpha isoform (calcineurin B, type I) [KO:K06268 ]
19059 Ppp3r2; protein phosphatase 3, regulatory subunit B, alpha isoform (calcineurin B, type II) [KO:K06268 ]
67819 Derl1; Der1-like domain family, member 1 [KO:K11519 ]
19181 Psmc2; proteasome (prosome, macropain) 26S subunit, ATPase 2 [KO:K03061 ]
19179 Psmc1; protease (prosome, macropain) 26S subunit, ATPase 1 [KO:K03062 ]
23996 Psmc4; proteasome (prosome, macropain) 26S subunit, ATPase, 4 [KO:K03063 ]
67089 Psmc6; proteasome (prosome, macropain) 26S subunit, ATPase, 6 [KO:K03064 ]
19182 Psmc3; proteasome (prosome, macropain) 26S subunit, ATPase 3 [KO:K03065 ]
19184 Psmc5; protease (prosome, macropain) 26S subunit, ATPase 5 [KO:K03066 ]
21762 Psmd2; proteasome (prosome, macropain) 26S subunit, non-ATPase, 2 [KO:K03028 ]
70247 Psmd1; proteasome (prosome, macropain) 26S subunit, non-ATPase, 1 [KO:K03032 ]
22123 Psmd3; proteasome (prosome, macropain) 26S subunit, non-ATPase, 3 [KO:K03033 ]
67151 Psmd9; proteasome (prosome, macropain) 26S subunit, non-ATPase, 9 [KO:K06693 ]
66997 Psmd12; proteasome (prosome, macropain) 26S subunit, non-ATPase, 12 [KO:K03035 ]
69077 Psmd11; proteasome (prosome, macropain) 26S subunit, non-ATPase, 11 [KO:K03036 ]
66413 Psmd6; proteasome (prosome, macropain) 26S subunit, non-ATPase, 6 [KO:K03037 ]
17463 Psmd7; proteasome (prosome, macropain) 26S subunit, non-ATPase, 7 [KO:K03038 ]
23997 Psmd13; proteasome (prosome, macropain) 26S subunit, non-ATPase, 13 [KO:K03039 ]
19185 Psmd4; proteasome (prosome, macropain) 26S subunit, non-ATPase, 4 [KO:K03029 ]
59029 Psmd14; proteasome (prosome, macropain) 26S subunit, non-ATPase, 14 [KO:K03030 ]
57296 Psmd8; proteasome (prosome, macropain) 26S subunit, non-ATPase, 8 [KO:K03031 ]
56436 Adrm1; adhesion regulating molecule 1 26S proteasome ubiquitin receptor [KO:K06691 ]
20422 Sem1; SEM1, 26S proteasome complex subunit [KO:K10881 ]
15382 Hnrnpa1; heterogeneous nuclear ribonucleoprotein A1 [KO:K12741 ]
229279 Hnrnpa3; heterogeneous nuclear ribonucleoprotein A3 [KO:K12741 ]
545091 Hnrnpa1l2-ps2; heterogeneous nuclear ribonucleoprotein A1-like 2, pseudogene 2 [KO:K12741 ]
53379 Hnrnpa2b1; heterogeneous nuclear ribonucleoprotein A2/B1 [KO:K13158 ]
11727 Ang; angiogenin, ribonuclease, RNase A family, 5 [KO:K16631 ] [EC:3.1.27.-]
11731 Ang2; angiogenin, ribonuclease A family, member 2 [KO:K16631 ] [EC:3.1.27.-]
219033 Ang4; angiogenin, ribonuclease A family, member 4 [KO:K16631 ] [EC:3.1.27.-]
503844 Ang5; angiogenin, ribonuclease A family, member 5 [KO:K16631 ] [EC:3.1.27.-]
11730 Ang3; angiogenin, ribonuclease A family, member 3 [KO:K16631 ] [EC:3.1.27.-]
630952 Ang6; angiogenin, ribonuclease A family, member 6 [KO:K16631 ] [EC:3.1.27.-]
433702 Ncbp1; nuclear cap binding protein subunit 1 [KO:K12882 ]
20383 Srsf3; serine and arginine-rich splicing factor 3 [KO:K12892 ]
225027 Srsf7; serine and arginine-rich splicing factor 7 [KO:K12896 ]
237082 Nxt2; nuclear transport factor 2-like export factor 2 [KO:K14285 ]
108989 Tpr; translocated promoter region, nuclear basket protein [KO:K09291 ]
110379 Sec13; SEC13 homolog, nuclear pore and COPII coat complex component [KO:K14004 ]
107939 Pom121; nuclear pore membrane protein 121 [KO:K14316 ]
195236 Pom121l2; POM121 transmembrane nucleoporin like 2 [KO:K14316 ]
19386 Ranbp2; RAN binding protein 2 [KO:K12172 ] [EC:2.3.2.-]
103172 Chchd10; coiled-coil-helix-coiled-coil-helix domain containing 10 [KO:K22759 ]
56491 Vapb; vesicle-associated membrane protein, associated protein B and C [KO:K10707 ]
13198 Ddit3; DNA-damage inducible transcript 3 [KO:K04452 ]
13666 Eif2ak3; eukaryotic translation initiation factor 2 alpha kinase 3 [KO:K08860 ] [EC:2.7.11.1 ]
13665 Eif2s1; eukaryotic translation initiation factor 2, subunit 1 alpha [KO:K03237 ]
11911 Atf4; activating transcription factor 4 [KO:K04374 ]
16440 Itpr3; inositol 1,4,5-triphosphate receptor 3 [KO:K04960 ]
18391 Sigmar1; sigma non-opioid intracellular receptor 1 [KO:K20719 ]
21937 Tnfrsf1a; tumor necrosis factor receptor superfamily, member 1a [KO:K03158 ]
21938 Tnfrsf1b; tumor necrosis factor receptor superfamily, member 1b [KO:K05141 ]
13163 Daxx; Fas death domain-associated protein [KO:K02308 ]
12460 Ccs; copper chaperone for superoxide dismutase [KO:K04569 ]
18039 Nefl; neurofilament, light polypeptide [KO:K04572 ]
18040 Nefm; neurofilament, medium polypeptide [KO:K04573 ]
54130 Actr1a; ARP1 actin-related protein 1A, centractin alpha [KO:K16575 ]
226977 Actr1b; ARP1 actin-related protein 1B, centractin beta [KO:K16575 ]
56444 Actr10; ARP10 actin-related protein 10 [KO:K16576 ]
15114 Hap1; huntingtin-associated protein 1 [KO:K04647 ]
13411 Dnah11; dynein, axonemal, heavy chain 11 [KO:K10408 ]
13417 Dnah8; dynein, axonemal, heavy chain 8 [KO:K10408 ]
56087 Dnah10; dynein, axonemal, heavy chain 10 [KO:K10408 ]
69926 Dnah17; dynein, axonemal, heavy chain 17 [KO:K10408 ]
75563 Dnali1; dynein, axonemal, light intermediate polypeptide 1 [KO:K10410 ]
68922 Dnai1; dynein axonemal intermediate chain 1 [KO:K10409 ]
432611 Dnai2; dynein axonemal intermediate chain 2 [KO:K11143 ]
54152 Dnal4; dynein, axonemal, light chain 4 [KO:K10412 ]
20511 Slc1a2; solute carrier family 1 (glial high affinity glutamate transporter), member 2 [KO:K05613 ]
14799 Gria1; glutamate receptor, ionotropic, AMPA1 (alpha 1) [KO:K05197 ]
14800 Gria2; glutamate receptor, ionotropic, AMPA2 (alpha 2) [KO:K05198 ]
14810 Grin1; glutamate receptor, ionotropic, NMDA1 (zeta 1) [KO:K05208 ]
14811 Grin2a; glutamate receptor, ionotropic, NMDA2A (epsilon 1) [KO:K05209 ]
14812 Grin2b; glutamate receptor, ionotropic, NMDA2B (epsilon 2) [KO:K05210 ]
14813 Grin2c; glutamate receptor, ionotropic, NMDA2C (epsilon 3) [KO:K05211 ]
14814 Grin2d; glutamate receptor, ionotropic, NMDA2D (epsilon 4) [KO:K05212 ]
Compound
C04549 1-Phosphatidyl-1D-myo-inositol 3-phosphate
Reference
Authors
Brown RH, Al-Chalabi A
Title
Amyotrophic Lateral Sclerosis.
Journal
Reference
Authors
Webster CP, Smith EF, Shaw PJ, De Vos KJ
Title
Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?
Journal
Reference
Authors
Valko K, Ciesla L
Title
Amyotrophic lateral sclerosis.
Journal
Reference
Authors
Mathis S, Goizet C, Soulages A, Vallat JM, Masson GL
Title
Genetics of amyotrophic lateral sclerosis: A review.
Journal
Reference
Authors
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
Title
Controversies and priorities in amyotrophic lateral sclerosis.
Journal
Reference
Authors
Pasinelli P, Brown RH
Title
Molecular biology of amyotrophic lateral sclerosis: insights from genetics.
Journal
Reference
Authors
Goodall EF, Morrison KE
Title
Amyotrophic lateral sclerosis (motor neuron disease): proposed mechanisms and pathways to treatment.
Journal
Reference
Authors
Smith EF, Shaw PJ, De Vos KJ
Title
The role of mitochondria in amyotrophic lateral sclerosis.
Journal
Reference
Authors
Kodavati M, Wang H, Hegde ML
Title
Altered Mitochondrial Dynamics in Motor Neuron Disease: An Emerging Perspective.
Journal
Reference
Authors
Hervias I, Beal MF, Manfredi G
Title
Mitochondrial dysfunction and amyotrophic lateral sclerosis.
Journal
Reference
Authors
Bacman SR, Bradley WG, Moraes CT
Title
Mitochondrial involvement in amyotrophic lateral sclerosis: trigger or target?
Journal
Reference
Authors
Lin MT, Beal MF
Title
Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases.
Journal
Reference
Authors
Bendotti C, Bao Cutrona M, Cheroni C, Grignaschi G, Lo Coco D, Peviani M, Tortarolo M, Veglianese P, Zennaro E
Title
Inter- and intracellular signaling in amyotrophic lateral sclerosis: role of p38 mitogen-activated protein kinase.
Journal
Reference
Authors
Simpson EP, Yen AA, Appel SH
Title
Oxidative Stress: a common denominator in the pathogenesis of amyotrophic lateral sclerosis.
Journal
Reference
Authors
Bendotti C, Carri MT
Title
Lessons from models of SOD1-linked familial ALS.
Journal
Reference
Authors
Robertson J, Kriz J, Nguyen MD, Julien JP
Title
Pathways to motor neuron degeneration in transgenic mouse models.
Journal
Reference
Authors
Eve DJ, Dennis JS, Citron BA
Title
Transcription factor p53 in degenerating spinal cords.
Journal
Reference
Authors
Bahar E, Kim H, Yoon H
Title
ER Stress-Mediated Signaling: Action Potential and Ca(2+) as Key Players.
Journal
Reference
Authors
Bezprozvanny I
Title
Calcium signaling and neurodegenerative diseases.
Journal
Reference
Authors
von Lewinski F, Keller BU
Title
Ca2+, mitochondria and selective motoneuron vulnerability: implications for ALS.
Journal
Reference
Authors
Julien JP.
Title
Amyotrophic lateral sclerosis. unfolding the toxicity of the misfolded.
Journal
Reference
Authors
Hand CK, Rouleau GA
Title
Familial amyotrophic lateral sclerosis.
Journal
Reference
Authors
Barber SC, Mead RJ, Shaw PJ
Title
Oxidative stress in ALS: a mechanism of neurodegeneration and a therapeutic target.
Journal
Reference
Authors
Prasad A, Bharathi V, Sivalingam V, Girdhar A, Patel BK
Title
Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis.
Journal
Reference
Authors
Nguyen DKH, Thombre R, Wang J
Title
Autophagy as a common pathway in amyotrophic lateral sclerosis.
Journal
Reference
Authors
Lee JK, Shin JH, Lee JE, Choi EJ
Title
Role of autophagy in the pathogenesis of amyotrophic lateral sclerosis.
Journal
Reference
Authors
Oakes JA, Davies MC, Collins MO
Title
TBK1: a new player in ALS linking autophagy and neuroinflammation.
Journal
Reference
Authors
Ramesh N, Pandey UB
Title
Autophagy Dysregulation in ALS: When Protein Aggregates Get Out of Hand.
Journal
Reference
Authors
Kunita R, Otomo A, Mizumura H, Suzuki-Utsunomiya K, Hadano S, Ikeda JE
Title
The Rab5 activator ALS2/alsin acts as a novel Rac1 effector through Rac1-activated endocytosis.
Journal
Reference
Authors
Hadano S, Kunita R, Otomo A, Suzuki-Utsunomiya K, Ikeda JE
Title
Molecular and cellular function of ALS2/alsin: implication of membrane dynamics in neuronal development and degeneration.
Journal
Reference
Authors
Ruffoli R, Bartalucci A, Frati A, Fornai F
Title
Ultrastructural studies of ALS mitochondria connect altered function and permeability with defects of mitophagy and mitochondriogenesis.
Journal
Reference
Authors
Menzies FM, Fleming A, Rubinsztein DC
Title
Compromised autophagy and neurodegenerative diseases.
Journal
Reference
Authors
Casterton RL, Hunt RJ, Fanto M
Title
Pathomechanism Heterogeneity in the Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Disease Spectrum: Providing Focus Through the Lens of Autophagy.
Journal
Reference
Authors
Lin G, Mao D, Bellen HJ
Title
Amyotrophic Lateral Sclerosis Pathogenesis Converges on Defects in Protein Homeostasis Associated with TDP-43 Mislocalization and Proteasome-Mediated Degradation Overload.
Journal
Reference
Authors
Hetz C, Mollereau B
Title
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
Journal
Reference
Authors
Xiang C, Wang Y, Zhang H, Han F
Title
The role of endoplasmic reticulum stress in neurodegenerative disease.
Journal
Reference
Authors
Hughes D, Mallucci GR
Title
The unfolded protein response in neurodegenerative disorders - therapeutic modulation of the PERK pathway.
Journal
Reference
Authors
Doyle KM, Kennedy D, Gorman AM, Gupta S, Healy SJ, Samali A
Title
Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders.
Journal
Reference
Authors
Turner BJ, Atkin JD
Title
ER stress and UPR in familial amyotrophic lateral sclerosis.
Journal
Reference
Authors
Nishitoh H, Kadowaki H, Nagai A, Maruyama T, Yokota T, Fukutomi H, Noguchi T, Matsuzawa A, Takeda K, Ichijo H
Title
ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1.
Journal
Reference
Authors
Walker AK, Atkin JD
Title
Stress signaling from the endoplasmic reticulum: A central player in the pathogenesis of amyotrophic lateral sclerosis.
Journal
Reference
Authors
Renaud L, Picher-Martel V, Codron P, Julien JP
Title
Key role of UBQLN2 in pathogenesis of amyotrophic lateral sclerosis and frontotemporal dementia.
Journal
Reference
Authors
Nguyen HP, Van Broeckhoven C, van der Zee J
Title
ALS Genes in the Genomic Era and their Implications for FTD.
Journal
Reference
Authors
Atkin G, Paulson H
Title
Ubiquitin pathways in neurodegenerative disease.
Journal
Reference
Authors
Kim JM, Billington E, Reyes A, Notarianni T, Sage J, Agbas E, Taylor M, Monast I, Stanford JA, Agbas A
Title
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.
Journal
Reference
Authors
Benarroch EE
Title
Sigma-1 receptor and amyotrophic lateral sclerosis.
Journal
Reference
Authors
Millecamps S, Julien JP
Title
Axonal transport deficits and neurodegenerative diseases.
Journal
Related pathway
mmu04141 Protein processing in endoplasmic reticulum
mmu04810 Regulation of actin cytoskeleton
KO pathway
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