Entry
Name
Amyotrophic lateral sclerosis - Mandrillus leucophaeus (drill)
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
Mandrillus leucophaeus (drill) [GN:
mleu ]
Gene
105548296 APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084 ]
105544481 NDUFV1; NADH dehydrogenase [ubiquinone] flavoprotein 1, mitochondrial isoform X1 [KO:K03942 ] [EC:7.1.1.2 ]
105542372 NDUFV2; NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial isoform X1 [KO:K03943 ] [EC:7.1.1.2 ]
105555594 NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial [KO:K03944 ]
105537526 NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945 ]
105528352 NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 isoform X1 [KO:K03946 ]
105539213 NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947 ]
105552516 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947 ]
105545563 NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948 ]
105539723 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like isoform X1 [KO:K03949 ]
105550883 NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949 ]
105538803 NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950 ]
105529172 NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951 ]
105546320 NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 isoform X1 [KO:K03952 ]
105551123 NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953 ]
105531588 NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954 ]
105530057 NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956 ]
105546990 NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 isoform X1 [KO:K11352 ]
105547363 NDUFA13; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353 ]
105547955 NDUFB1; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957 ]
105550513 NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial isoform X1 [KO:K03958 ]
105535428 NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959 ]
105533216 NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960 ]
105539929 NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961 ]
105539631 NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962 ]
105540981 NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963 ]
105533683 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial-like [KO:K03964 ]
105537752 NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X1 [KO:K03964 ]
105554617 NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965 ]
105529740 NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966 ]
105541684 NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial isoform X1 [KO:K11351 ]
105549398 NDUFS1; LOW QUALITY PROTEIN: NADH-ubiquinone oxidoreductase 75 kDa subunit, mitochondrial [KO:K03934 ] [EC:7.1.1.2 ]
105536733 NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial isoform X1 [KO:K03935 ] [EC:7.1.1.2 ]
105554828 NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936 ] [EC:7.1.1.2 ]
105553460 NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937 ]
105537917 LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] iron-sulfur protein 5-like [KO:K03938 ]
105531487 NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939 ]
105551946 NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940 ] [EC:7.1.1.2 ]
105544487 NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941 ] [EC:7.1.1.2 ]
105543644 NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967 ]
105546505 NADH dehydrogenase [ubiquinone] 1 subunit C2 isoform X1 [KO:K03968 ]
105528135 SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234 ] [EC:1.3.5.1 ]
105537465 succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234 ] [EC:1.3.5.1 ]
105552727 SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235 ] [EC:1.3.5.1 ]
105540160 succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial-like isoform X1 [KO:K00235 ] [EC:1.3.5.1 ]
105548772 LOW QUALITY PROTEIN: succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like [KO:K00236 ]
105536578 SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial isoform X1 [KO:K00236 ]
105529588 succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like isoform X1 [KO:K00236 ]
105550790 succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like isoform X1 [KO:K00236 ]
105551506 LOW QUALITY PROTEIN: succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial-like [KO:K00237 ]
105537410 SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237 ]
105536314 cytochrome c1, heme protein, mitochondrial isoform X1 [KO:K00413 ]
105539498 cytochrome c oxidase subunit 4 isoform 2, mitochondrial [KO:K02263 ]
105528615 cytochrome c oxidase subunit 4 isoform 1, mitochondrial-like [KO:K02263 ]
105553641 LOW QUALITY PROTEIN: cytochrome c oxidase subunit 5A, mitochondrial-like [KO:K02264 ]
105541474 cytochrome c oxidase subunit 6A2, mitochondrial isoform X1 [KO:K02266 ]
105538345 cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270 ]
105530782 cytochrome c oxidase subunit 7B, mitochondrial isoform X1 [KO:K02271 ]
105532785 LOW QUALITY PROTEIN: ATP synthase subunit alpha, mitochondrial-like [KO:K02132 ]
105535712 ATP5C1; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136 ]
105554307 ATP5F1; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127 ]
105553294 ATP5G1; ATP synthase F(0) complex subunit C1, mitochondrial isoform X1 [KO:K02128 ]
105548040 ATP5G3; ATP synthase F(0) complex subunit C3, mitochondrial isoform X1 [KO:K02128 ]
105548527 ATP5G2; ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128 ]
105536822 ATP5H; ATP synthase subunit d, mitochondrial isoform X1 [KO:K02138 ]
105553154 ATP5O; ATP synthase subunit O, mitochondrial isoform X1 [KO:K02137 ]
105545476 ATP5J; ATP synthase-coupling factor 6, mitochondrial isoform X1 [KO:K02131 ]
105531327 VDAC1; voltage-dependent anion-selective channel protein 1 isoform X1 [KO:K05862 ]
105535839 TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518 ]
105536633 TOMM40L; mitochondrial import receptor subunit TOM40B isoform X1 [KO:K11518 ]
105533812 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435 ]
105546751 MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C [KO:K10435 ]
105533867 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435 ]
105547719 TANK; TRAF family member-associated NF-kappa-B activator isoform X1 [KO:K12650 ]
105536018 SMCR8; smith-Magenis syndrome chromosomal region candidate gene 8 protein [KO:K23611 ]
105554801 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 [KO:K17985 ]
105553640 ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889 ]
105551090 WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908 ]
105533512 WIPI1; WD repeat domain phosphoinositide-interacting protein 1 isoform X1 [KO:K17908 ]
105537161 CHMP2B; charged multivesicular body protein 2b isoform X1 [KO:K12192 ]
105547498 PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348 ] [EC:3.1.3.16 ]
105529928 PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348 ] [EC:3.1.3.16 ]
105531014 PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348 ] [EC:3.1.3.16 ]
105541024 PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028 ]
105552414 PSMD1; 26S proteasome non-ATPase regulatory subunit 1 isoform X1 [KO:K03032 ]
105553052 PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033 ]
105527964 PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693 ]
105553193 PSMD12; 26S proteasome non-ATPase regulatory subunit 12 isoform X1 [KO:K03035 ]
105548703 PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036 ]
105539886 PSMD6; LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037 ]
105537186 PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038 ]
105530811 PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039 ]
105534732 PSMD4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029 ]
105547714 PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030 ]
105534582 PSMD8; 26S proteasome non-ATPase regulatory subunit 8 isoform X1 [KO:K03031 ]
105548358 ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691 ]
105535576 SHFM1; 26S proteasome complex subunit DSS1 isoform X1 [KO:K10881 ]
105545735 HNRNPA1L2; LOW QUALITY PROTEIN: heterogeneous nuclear ribonucleoprotein A1-like 2 [KO:K12741 ]
105533523 heterogeneous nuclear ribonucleoprotein A3 isoform X1 [KO:K12741 ]
105554897 LOW QUALITY PROTEIN: heterogeneous nuclear ribonucleoprotein A1-like [KO:K12741 ]
105548249 heterogeneous nuclear ribonucleoprotein A1-like isoform X1 [KO:K12741 ]
105533746 HNRNPA2B1; heterogeneous nuclear ribonucleoproteins A2/B1 isoform X1 [KO:K13158 ]
105544230 SRSF3; serine/arginine-rich splicing factor 3 isoform X1 [KO:K12892 ]
105551902 SRSF7; serine/arginine-rich splicing factor 7 isoform X1 [KO:K12896 ]
105530281 LOW QUALITY PROTEIN: nuclear RNA export factor 2-like [KO:K14284 ]
105530179 NUP153; nuclear pore complex protein Nup153 isoform X1 [KO:K14296 ]
105549250 NUP98; nuclear pore complex protein Nup98-Nup96 isoform X1 [KO:K14297 ]
105553347 NUP155; nuclear pore complex protein Nup155 isoform X1 [KO:K14312 ]
105542183 NUP210; LOW QUALITY PROTEIN: nuclear pore membrane glycoprotein 210 [KO:K14314 ]
105536848 POM121C; nuclear envelope pore membrane protein POM 121C [KO:K14316 ]
105532837 NUP214; nuclear pore complex protein Nup214 isoform X1 [KO:K14317 ]
105540048 RANBP2; LOW QUALITY PROTEIN: E3 SUMO-protein ligase RanBP2 [KO:K12172 ] [EC:2.3.2.-]
105530419 CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10, mitochondrial isoform X1 [KO:K22759 ]
105548395 vesicle-associated membrane protein-associated protein B/C-like [KO:K10707 ]
105551004 ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054 ]
105545576 DDIT3; DNA damage-inducible transcript 3 protein isoform X1 [KO:K04452 ]
105549624 EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
105538852 ATF4; cyclic AMP-dependent transcription factor ATF-4 isoform X1 [KO:K04374 ]
105544182 ITPR3; inositol 1,4,5-trisphosphate receptor type 3 isoform X1 [KO:K04960 ]
105541778 TNFRSF1A; tumor necrosis factor receptor superfamily member 1A [KO:K03158 ]
105551364 TNFRSF1B; tumor necrosis factor receptor superfamily member 1B [KO:K05141 ]
105553084 HAP1; LOW QUALITY PROTEIN: huntingtin-associated protein 1 [KO:K04647 ]
105536668 DNAH3; LOW QUALITY PROTEIN: dynein heavy chain 3, axonemal [KO:K10408 ]
105549581 DNAH5; LOW QUALITY PROTEIN: dynein heavy chain 5, axonemal [KO:K10408 ]
105531557 DNALI1; axonemal dynein light intermediate polypeptide 1 isoform X1 [KO:K10410 ]
105539669 DNAI1; dynein intermediate chain 1, axonemal isoform X1 [KO:K10409 ]
105528691 SLC1A2; excitatory amino acid transporter 2 isoform X1 [KO:K05613 ]
105537989 NRG1; pro-neuregulin-1, membrane-bound isoform isoform X1 [KO:K05455 ]
105550326 NRG3; pro-neuregulin-3, membrane-bound isoform isoform X1 [KO:K05457 ]
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
mleu04141 Protein processing in endoplasmic reticulum
KO pathway
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