Entry
Name
Amyotrophic lateral sclerosis
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
ko05014 Amyotrophic lateral sclerosis
Disease
H00058 Amyotrophic lateral sclerosis (ALS)
Orthology
K00234 SDHA, SDH1; succinate dehydrogenase (ubiquinone) flavoprotein subunit [EC:1.3.5.1 ]
K00235 SDHB, SDH2; succinate dehydrogenase (ubiquinone) iron-sulfur subunit [EC:1.3.5.1 ]
K00236 SDHC, SDH3; succinate dehydrogenase (ubiquinone) cytochrome b560 subunit
K00237 SDHD, SDH4; succinate dehydrogenase (ubiquinone) membrane anchor subunit
K00411 UQCRFS1, RIP1, petA; ubiquinol-cytochrome c reductase iron-sulfur subunit [EC:7.1.1.8 ]
K00412 CYTB, petB; ubiquinol-cytochrome c reductase cytochrome b subunit
K00413 CYC1, CYT1, petC; ubiquinol-cytochrome c reductase cytochrome c1 subunit
K00414 QCR1, UQCRC1; ubiquinol-cytochrome c reductase core subunit 1
K00415 QCR2, UQCRC2; ubiquinol-cytochrome c reductase core subunit 2
K00416 QCR6, UQCRH; ubiquinol-cytochrome c reductase subunit 6
K00417 QCR7, UQCRB; ubiquinol-cytochrome c reductase subunit 7
K00418 QCR8, UQCRQ; ubiquinol-cytochrome c reductase subunit 8
K00419 QCR9, UCRC; ubiquinol-cytochrome c reductase subunit 9
K00420 QCR10, UQCR; ubiquinol-cytochrome c reductase subunit 10
K02084 APAF1; apoptotic protease-activating factor
K02125 ATPeF08, MTATP8, ATP8; F-type H+-transporting ATPase subunit 8
K02126 ATPeF0A, MTATP6, ATP6; F-type H+-transporting ATPase subunit a
K02127 ATPeF0B, ATP5F1, ATP4; F-type H+-transporting ATPase subunit b
K02128 ATPeF0C, ATP5G, ATP9; F-type H+-transporting ATPase subunit c
K02131 ATPeF0F6, ATP5J; F-type H+-transporting ATPase subunit 6
K02132 ATPeF1A, ATP5A1, ATP1; F-type H+-transporting ATPase subunit alpha
K02133 ATPeF1B, ATP5B, ATP2; F-type H+-transporting ATPase subunit beta [EC:7.1.2.2 ]
K02134 ATPeF1D, ATP5D, ATP16; F-type H+-transporting ATPase subunit delta
K02135 ATPeF1E, ATP5E, ATP15; F-type H+-transporting ATPase subunit epsilon
K02136 ATPeF1G, ATP5C1, ATP3; F-type H+-transporting ATPase subunit gamma
K02137 ATPeF0O, ATP5O, ATP5; F-type H+-transporting ATPase subunit O
K02138 ATPeF0D, ATP5H, ATP7; F-type H+-transporting ATPase subunit d
K02158 BAD; Bcl-2-antagonist of cell death
K02159 BAX; apoptosis regulator BAX
K02161 BCL2; apoptosis regulator Bcl-2
K02261 COX2; cytochrome c oxidase subunit 2
K02262 COX3; cytochrome c oxidase subunit 3
K02263 COX4; cytochrome c oxidase subunit 4
K02264 COX5A; cytochrome c oxidase subunit 5a
K02265 COX5B; cytochrome c oxidase subunit 5b
K02266 COX6A; cytochrome c oxidase subunit 6a
K02267 COX6B; cytochrome c oxidase subunit 6b
K02268 COX6C; cytochrome c oxidase subunit 6c
K02270 COX7A; cytochrome c oxidase subunit 7a
K02271 COX7B; cytochrome c oxidase subunit 7b
K02272 COX7C; cytochrome c oxidase subunit 7c
K02273 COX8; cytochrome c oxidase subunit 8
K02308 DAXX; death-associated protein 6
K03028 PSMD2, RPN1; 26S proteasome regulatory subunit N1
K03029 PSMD4, RPN10; 26S proteasome regulatory subunit N10
K03030 PSMD14, RPN11, POH1; 26S proteasome regulatory subunit N11
K03031 PSMD8, RPN12; 26S proteasome regulatory subunit N12
K03032 PSMD1, RPN2; 26S proteasome regulatory subunit N2
K03033 PSMD3, RPN3; 26S proteasome regulatory subunit N3
K03035 PSMD12, RPN5; 26S proteasome regulatory subunit N5
K03036 PSMD11, RPN6; 26S proteasome regulatory subunit N6
K03037 PSMD6, RPN7; 26S proteasome regulatory subunit N7
K03038 PSMD7, RPN8; 26S proteasome regulatory subunit N8
K03039 PSMD13, RPN9; 26S proteasome regulatory subunit N9
K03061 PSMC2, RPT1; 26S proteasome regulatory subunit T1
K03062 PSMC1, RPT2; 26S proteasome regulatory subunit T2
K03063 PSMC4, RPT3; 26S proteasome regulatory subunit T3
K03064 PSMC6, RPT4; 26S proteasome regulatory subunit T4
K03065 PSMC3, RPT5; 26S proteasome regulatory subunit T5
K03066 PSMC5, RPT6; 26S proteasome regulatory subunit T6
K03156 TNF, TNFA; tumor necrosis factor superfamily, member 2
K03158 TNFRSF1A, TNFR1, CD120a; tumor necrosis factor receptor superfamily member 1A
K03237 EIF2S1; translation initiation factor 2 subunit 1
K03882 ND4L; NADH-ubiquinone oxidoreductase chain 4L [EC:7.1.1.2 ]
K03934 NDUFS1; NADH dehydrogenase (ubiquinone) Fe-S protein 1 [EC:7.1.1.2 ]
K03935 NDUFS2; NADH dehydrogenase (ubiquinone) Fe-S protein 2 [EC:7.1.1.2 ]
K03936 NDUFS3; NADH dehydrogenase (ubiquinone) Fe-S protein 3 [EC:7.1.1.2 ]
K03937 NDUFS4; NADH dehydrogenase (ubiquinone) Fe-S protein 4
K03938 NDUFS5; NADH dehydrogenase (ubiquinone) Fe-S protein 5
K03939 NDUFS6; NADH dehydrogenase (ubiquinone) Fe-S protein 6
K03940 NDUFS7; NADH dehydrogenase (ubiquinone) Fe-S protein 7 [EC:7.1.1.2 ]
K03941 NDUFS8; NADH dehydrogenase (ubiquinone) Fe-S protein 8 [EC:7.1.1.2 ]
K03942 NDUFV1; NADH dehydrogenase (ubiquinone) flavoprotein 1 [EC:7.1.1.2 ]
K03943 NDUFV2; NADH dehydrogenase (ubiquinone) flavoprotein 2 [EC:7.1.1.2 ]
K03944 NDUFV3; NADH dehydrogenase (ubiquinone) flavoprotein 3
K03945 NDUFA1; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 1
K03946 NDUFA2; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 2
K03947 NDUFA3; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 3
K03948 NDUFA4; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 4
K03949 NDUFA5; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 5
K03950 NDUFA6; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 6
K03951 NDUFA7; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 7
K03952 NDUFA8; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 8
K03953 NDUFA9; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 9
K03954 NDUFA10; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 10
K03955 NDUFAB1; NADH dehydrogenase (ubiquinone) 1 alpha/beta subcomplex 1, acyl-carrier protein
K03956 NDUFA11; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 11
K03957 NDUFB1; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 1
K03958 NDUFB2; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 2
K03959 NDUFB3; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 3
K03960 NDUFB4; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 4
K03961 NDUFB5; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 5
K03962 NDUFB6; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 6
K03963 NDUFB7; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 7
K03964 NDUFB8; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 8
K03965 NDUFB9; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 9
K03966 NDUFB10; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 10
K03967 NDUFC1; NADH dehydrogenase (ubiquinone) 1 subunit C1
K03968 NDUFC2; NADH dehydrogenase (ubiquinone) 1 subunit C2
K04348 PPP3C, CNA; serine/threonine-protein phosphatase 2B catalytic subunit [EC:3.1.3.16 ]
K04374 ATF4, CREB2; cyclic AMP-dependent transcription factor ATF-4
K04392 RAC1; Ras-related C3 botulinum toxin substrate 1
K04426 MAP3K5, ASK1; mitogen-activated protein kinase kinase kinase 5 [EC:2.7.11.25 ]
K04432 MAP2K3, MKK3; mitogen-activated protein kinase kinase 3 [EC:2.7.12.2 ]
K04433 MAP2K6, MKK6; mitogen-activated protein kinase kinase 6 [EC:2.7.12.2 ]
K04451 TP53, P53; tumor protein p53
K04452 DDIT3, GADD153; DNA damage-inducible transcript 3
K04569 CCS; copper chaperone for superoxide dismutase
K04570 BCL2L1, bcl-xL; Bcl-2-like 1 (apoptosis regulator Bcl-X)
K04572 NEFL, NF-L; neurofilament light polypeptide
K04573 NEF3, NF-M; neurofilament medium polypeptide (neurofilament 3)
K04574 NEFH, NF-H; neurofilament heavy polypeptide
K04575 ALS2; amyotrophic lateral sclerosis 2 protein
K04647 HAP1; huntingtin-associated protein 1
K04726 BID; BH3 interacting domain death agonist
K04741 CASP12; caspase 12 [EC:3.4.22.-]
K04960 ITPR3; inositol 1,4,5-triphosphate receptor type 3
K05085 ERBB4, HER4; receptor tyrosine-protein kinase erbB-4 [EC:2.7.10.1 ]
K05141 TNFRSF1B, TNFR2, CD120b; tumor necrosis factor receptor superfamily member 1B
K05197 GRIA1; glutamate receptor 1
K05198 GRIA2; glutamate receptor 2
K05208 GRIN1; glutamate receptor ionotropic, NMDA 1
K05209 GRIN2A; glutamate receptor ionotropic, NMDA 2A
K05210 GRIN2B; glutamate receptor ionotropic, NMDA 2B
K05211 GRIN2C; glutamate receptor ionotropic, NMDA 2C
K05212 GRIN2D; glutamate receptor ionotropic, NMDA 2D
K05613 SLC1A2, EAAT2; solute carrier family 1 (glial high affinity glutamate transporter), member 2
K05692 ACTB_G1; actin beta/gamma 1
K05862 VDAC1; voltage-dependent anion channel protein 1
K06268 PPP3R, CNB; serine/threonine-protein phosphatase 2B regulatory subunit
K06691 RPN13; 26S proteasome regulatory subunit N13
K06693 PSMD9, RPN4; 26S proteasome regulatory subunit N4
K07203 MTOR, FRAP, TOR; serine/threonine-protein kinase mTOR [EC:2.7.11.1 ]
K07874 RAB1A; Ras-related protein Rab-1A
K07887 RAB5A; Ras-related protein Rab-5A
K07901 RAB8A, MEL; Ras-related protein Rab-8A
K07925 RAB39B; Ras-related protein Rab-39B
K08331 ATG13; autophagy-related protein 13
K08333 PIK3R4, VPS15; phosphoinositide-3-kinase, regulatory subunit 4 [EC:2.7.11.1 ]
K08334 BECN, VPS30, ATG6; beclin
K08852 ERN1; serine/threonine-protein kinase/endoribonuclease IRE1 [EC:2.7.11.1 3.1.26.-]
K08860 EIF2AK3, PERK; eukaryotic translation initiation factor 2-alpha kinase 3 [EC:2.7.11.1 ]
K09027 XBP1; X box-binding protein 1
K09054 ATF6A; cyclic AMP-dependent transcription factor ATF-6 alpha
K09291 TPR, MLP1, MLP2; nucleoprotein TPR
K10396 KIF5; kinesin family member 5 (plus-end-directed kinesin ATPase) [EC:5.6.1.3 ]
K10407 KLC; kinesin light chain
K10408 DNAH; dynein axonemal heavy chain
K10409 DNAI1; dynein axonemal intermediate chain 1
K10410 DNALI; dynein axonemal light intermediate chain 1
K10411 DNAL1; dynein axonemal light chain 1
K10412 DNAL4; dynein axonemal light chain 4
K10435 MAP1LC; microtubule-associated protein 1 light chain
K10707 VAPB, ALS8; vesicle-associated membrane protein-associated protein B
K10881 SHFM1, DSS1, RPN15; 26 proteasome complex subunit DSS1
K11143 DNAI2; dynein axonemal intermediate chain 2
K11351 NDUFB11; NADH dehydrogenase (ubiquinone) 1 beta subcomplex subunit 11
K11352 NDUFA12; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 12
K11353 NDUFA13; NADH dehydrogenase (ubiquinone) 1 alpha subcomplex subunit 13
K11518 TOM40; mitochondrial import receptor subunit TOM40
K12172 RANBP2, NUP358; E3 SUMO-protein ligase RanBP2 [EC:2.3.2.-]
K12192 CHMP2B; charged multivesicular body protein 2B
K12650 TANK; TRAF family member-associated NF-kappa-B activator
K12741 HNRNPA1_3; heterogeneous nuclear ribonucleoprotein A1/A3
K12881 THOC4, ALY; THO complex subunit 4
K12882 NCBP1, CBP80; nuclear cap-binding protein subunit 1
K12892 SRSF3, SFRS3; serine/arginine-rich splicing factor 3
K12896 SRSF7, SFRS7; serine/arginine-rich splicing factor 7
K13098 TLS, FUS; RNA-binding protein FUS
K13158 HNRNPA2B1; heterogeneous nuclear ribonucleoprotein A2/B1
K13525 VCP, CDC48; transitional endoplasmic reticulum ATPase
K14004 SEC13; protein transport protein SEC13
K14284 NXF, TAP, MEX67; nuclear RNA export factor
K14285 NXT1_2, P15; NTF2-related export protein 1/2
K14295 NUP50, NPAP60; nuclear pore complex protein Nup50
K14296 NUP153; nuclear pore complex protein Nup153
K14297 NUP98, ADAR2, NUP116; nuclear pore complex protein Nup98-Nup96
K14298 RAE1, GLE2; mRNA export factor
K14300 NUP133; nuclear pore complex protein Nup133
K14301 NUP107, NUP84; nuclear pore complex protein Nup107
K14302 NUP37; nuclear pore complex protein Nup37
K14303 NUP160, NUP120; nuclear pore complex protein Nup160
K14304 NUP85; nuclear pore complex protein Nup85
K14305 NUP43; nuclear pore complex protein Nup43
K14306 NUP62, NSP1; nuclear pore complex protein Nup62
K14307 NUPL1, NUP49; nucleoporin p58/p45
K14308 NUP54, NUP57; nuclear pore complex protein Nup54
K14309 NUP93, NIC96; nuclear pore complex protein Nup93
K14310 NUP205, NUP192; nuclear pore complex protein Nup205
K14311 NUP188; nuclear pore complex protein Nup188
K14312 NUP155, NUP170, NUP157; nuclear pore complex protein Nup155
K14313 NUP35, NUP53; nuclear pore complex protein Nup53
K14314 NUP210, GP210; nuclear pore complex protein Nup210
K14315 NDC1, TMEM48; nucleoporin NDC1
K14316 POM121, NUP121; nuclear pore complex protein Nup121
K14317 NUP214, CAN; nuclear pore complex protein Nup214
K14318 NUP88; nuclear pore complex protein Nup88
K16575 ACTR1, ARP1; centractin
K16576 ACTR10, ARP11; actin-related protein 10
K16631 ANG, RNASE5; angiogenin [EC:3.1.27.-]
K17095 ANXA7_11; annexin A7/11
K17589 RB1CC1; RB1-inducible coiled-coil protein 1
K17889 ATG14L, ATG14; beclin 1-associated autophagy-related key regulator
K17906 ATG2; autophagy-related protein 2
K17908 WIPI1_2, ATG18; autophagy-related protein 18
K17985 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1
K19730 ATG101; autophagy-related protein 101
K20719 SIGMAR1; sigma non-opioid intracellular receptor
K20858 MCU; calcium uniporter protein, mitochondrial
K21246 NRBF2; nuclear receptor-binding factor 2
K22759 CHCHD10; coiled-coil-helix-coiled-coil-helix domain-containing protein 10
K22913 FIG4; phosphatidylinositol 3,5-bisphosphate 5-phosphatase [EC:3.1.3.-]
K23600 TARDBP, TDP43; TAR DNA-binding protein 43
K23609 C9ORF72; guanine nucleotide exchange protein C9orf72
K23610 WDR41; WD repeat-containing protein 41
K23611 SMCR8; guanine nucleotide exchange protein SMCR8
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
ko03013 Nucleocytoplasmic transport
ko04141 Protein processing in endoplasmic reticulum
ko04810 Regulation of actin cytoskeleton