PATHWAY: llv05022 Help
Entry
Name
Pathways of neurodegeneration - multiple diseases - Lutra lutra (Eurasian river otter)
Description
Neurodegeneration is generally defined as progressive, irreversible loss of neurons, which may affect either the peripheral or central nervous system (CNS). Neurodegenerative diseases (NDs) include highly debilitating illnesses, such as Alzheimer (AD), Parkinson disease (PD), amyotrophic lateral sclerosis, Huntington disease, spinocerebellar ataxias, and prion diseases (PrD). The hallmark event, which is thought to be at the root of these diseases, is the progressive accumulation of misfolded protein aggregates. Major basic processes include abnormal protein dynamics due to deficiency of the ubiquitin-proteosome-autophagy system, oxidative stress and free radical formation, endoplasmic reticulum stress, mitochondrial dysfunction and (secondary) disruptions of axonal transport.
Class
Human Diseases; Neurodegenerative disease
BRITE hierarchy
Pathway map
llv05022 Pathways of neurodegeneration - multiple diseases
Ortholog table
Organism
Lutra lutra (Eurasian river otter) [GN:
llv ]
Gene
125095266 LOW QUALITY PROTEIN: amyloid-beta precursor protein-like [KO:K04520 ]
125083151 guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634 ]
125108695 LOW QUALITY PROTEIN: 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1-like [KO:K05858 ] [EC:3.1.4.11 ]
125093345 short transient receptor potential channel 3 isoform X1 [KO:K04966 ]
125109360 inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958 ]
125107116 inositol 1,4,5-trisphosphate receptor type 2 isoform X1 [KO:K04959 ]
125103123 inositol 1,4,5-trisphosphate receptor type 3 isoform X1 [KO:K04960 ]
125083483 voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849 ]
125107204 voltage-dependent L-type calcium channel subunit alpha-1C isoform X1 [KO:K04850 ]
125078710 LOW QUALITY PROTEIN: voltage-dependent L-type calcium channel subunit alpha-1D-like [KO:K04851 ]
125091668 voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853 ]
125086002 voltage-dependent L-type calcium channel subunit alpha-1S isoform X1 [KO:K04857 ]
125102899 LOW QUALITY PROTEIN: ubiquitin-60S ribosomal protein L40-like [KO:K02927 ]
125079296 26S proteasome non-ATPase regulatory subunit 13 isoform X1 [KO:K03039 ]
125108458 vesicle-associated membrane protein-associated protein B isoform X1 [KO:K10707 ]
125085601 cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054 ]
125081642 LOW QUALITY PROTEIN: uncharacterized protein LOC125081642 [KO:K09027 ]
125107912 LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] flavoprotein 2, mitochondrial-like [KO:K03943 ] [EC:7.1.1.2 ]
125093717 nucleolar and coiled-body phosphoprotein 1-like isoform X1 [KO:K03944 ]
125091362 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945 ]
125108496 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1-like [KO:K03945 ]
125100354 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946 ]
125094229 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2-like [KO:K03946 ]
125088848 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like isoform X1 [KO:K03947 ]
125107352 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948 ]
125091808 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949 ]
125094078 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949 ]
125080931 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949 ]
125106375 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950 ]
125085932 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951 ]
125083711 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8-like [KO:K03952 ]
125088846 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952 ]
125106590 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953 ]
125095304 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial isoform X1 [KO:K03954 ]
125088449 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11-like [KO:K03956 ]
125106370 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352 ]
125081657 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353 ]
125091537 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1-like [KO:K03957 ]
125085582 LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1-like [KO:K03957 ]
125080587 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial-like [KO:K03958 ]
125094996 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959 ]
125097907 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960 ]
125100711 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961 ]
125083630 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962 ]
125083554 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963 ]
125085175 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964 ]
125096953 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965 ]
125104059 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10-like [KO:K03966 ]
125090506 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966 ]
125091408 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351 ]
125096286 LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial-like [KO:K03936 ] [EC:7.1.1.2 ]
125100031 NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937 ]
125097856 NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938 ]
125100336 NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939 ]
125089153 NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940 ] [EC:7.1.1.2 ]
125078801 NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941 ] [EC:7.1.1.2 ]
125093405 NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial-like [KO:K03967 ]
125107051 LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial-like [KO:K03967 ]
125100859 succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234 ] [EC:1.3.5.1 ]
125085786 succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236 ]
125110335 succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial-like [KO:K00237 ]
125095337 cytochrome b-c1 complex subunit 2, mitochondrial-like [KO:K00415 ]
125090285 cytochrome b-c1 complex subunit 2, mitochondrial isoform X1 [KO:K00415 ]
125097897 cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416 ]
125085010 cytochrome c oxidase subunit 4 isoform 1, mitochondrial-like [KO:K02263 ]
125109679 cytochrome c oxidase subunit 4 isoform 2, mitochondrial-like isoform X1 [KO:K02263 ]
125089146 cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263 ]
125084063 LOW QUALITY PROTEIN: cytochrome c oxidase subunit 5A, mitochondrial-like [KO:K02264 ]
125102537 LOW QUALITY PROTEIN: cytochrome c oxidase subunit 6B1-like [KO:K02267 ]
125108724 cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270 ]
125091285 LOW QUALITY PROTEIN: cytochrome c oxidase subunit 8A, mitochondrial-like [KO:K02273 ]
125084568 ATP synthase F(0) complex subunit C3, mitochondrial-like [KO:K02128 ]
125093193 ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128 ]
125102334 ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128 ]
125095139 ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128 ]
125095210 ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128 ]
125096423 ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128 ]
125080864 ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128 ]
125081272 ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128 ]
125106031 ATP synthase F(0) complex subunit C2, mitochondrial-like isoform X1 [KO:K02128 ]
125082594 ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128 ]
125084993 LOW QUALITY PROTEIN: ATP synthase subunit O, mitochondrial-like [KO:K02137 ]
125086141 mitochondrial import receptor subunit TOM40B isoform X1 [KO:K11518 ]
125088674 LOW QUALITY PROTEIN: mitochondrial import receptor subunit TOM40 homolog [KO:K11518 ]
125084662 LOW QUALITY PROTEIN: serine/threonine-protein phosphatase 2B catalytic subunit beta isoform-like [KO:K04348 ] [EC:3.1.3.16 ]
125092859 serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348 ] [EC:3.1.3.16 ]
125093220 serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348 ] [EC:3.1.3.16 ]
125079150 bcl2-associated agonist of cell death-like isoform X1 [KO:K02158 ]
125084890 tumor necrosis factor receptor superfamily member 6-like [KO:K04390 ]
125106618 LOW QUALITY PROTEIN: leucine-rich repeat serine/threonine-protein kinase 2-like [KO:K08844 ] [EC:2.7.11.1 ]
125092799 voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041 ]
125096807 intraflagellar transport protein 57 homolog isoform X1 [KO:K04638 ]
125086434 microtubule-associated proteins 1A/1B light chain 3C-like [KO:K10435 ]
125079924 microtubule-associated proteins 1A/1B light chain 3B-like [KO:K10435 ]
125095469 TRAF family member-associated NF-kappa-B activator isoform X1 [KO:K12650 ]
125084054 guanine nucleotide exchange factor C9orf72 isoform X1 [KO:K23609 ]
125079465 activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985 ]
125087891 WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908 ]
125090587 WD repeat domain phosphoinositide-interacting protein 2 [KO:K17908 ]
125103962 zinc finger FYVE domain-containing protein 1 isoform X1 [KO:K17603 ]
125102707 polyphosphoinositide phosphatase isoform X1 [KO:K22913 ] [EC:3.1.3.-]
125107758 tumor necrosis factor receptor superfamily member 1A-like isoform X1 [KO:K03158 ]
125098107 tumor necrosis factor receptor superfamily member 1B-like isoform X1 [KO:K05141 ]
125087546 LOW QUALITY PROTEIN: huntingtin-associated protein 1-like [KO:K04647 ]
125087139 LOW QUALITY PROTEIN: dynein axonemal heavy chain 9-like [KO:K10408 ]
125083962 LOW QUALITY PROTEIN: dynein axonemal intermediate chain 1-like [KO:K10409 ]
125101157 segment polarity protein dishevelled homolog DVL-3 isoform X1 [KO:K02353 ]
125087806 segment polarity protein dishevelled homolog DVL-2 isoform X1 [KO:K02353 ]
125097045 segment polarity protein dishevelled homolog DVL-1 isoform X1 [KO:K02353 ]
125102493 advanced glycosylation end product-specific receptor-like isoform X1 [KO:K19722 ]
125091990 LOW QUALITY PROTEIN: cytochrome b-245 heavy chain-like [KO:K21421 ] [EC:1.-.-.-]
125098134 macrophage colony-stimulating factor 1-like isoform X1 [KO:K05453 ]
Compound
C01245 D-myo-Inositol 1,4,5-trisphosphate
C04549 1-Phosphatidyl-1D-myo-inositol 3-phosphate
C04599 1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine
C11310 1-Methyl-4-phenylpyridinium
C16514 Amyloid beta protein 40
C16515 Amyloid beta protein 42
C21011 [Protein]-N(epsilon)-(carboxymethyl)lysine
C21012 [Protein]-N(epsilon)-(carboxyethyl)lysine
Reference
Authors
Gan L, Cookson MR, Petrucelli L, La Spada AR
Title
Converging pathways in neurodegeneration, from genetics to mechanisms.
Journal
Reference
Authors
Dugger BN, Dickson DW
Title
Pathology of Neurodegenerative Diseases.
Journal
Reference
Authors
Jellinger KA
Title
Basic mechanisms of neurodegeneration: a critical update.
Journal
Reference
Authors
Jellinger KA
Title
Recent advances in our understanding of neurodegeneration.
Journal
Reference
Authors
Van Bulck M, Sierra-Magro A, Alarcon-Gil J, Perez-Castillo A, Morales-Garcia JA
Title
Novel Approaches for the Treatment of Alzheimer's and Parkinson's Disease.
Journal
Reference
Authors
Ross CA, Poirier MA
Title
Protein aggregation and neurodegenerative disease.
Journal
Reference
Authors
Soto C, Pritzkow S
Title
Protein misfolding, aggregation, and conformational strains in neurodegenerative diseases.
Journal
Reference
Authors
Dantuma NP, Bott LC
Title
The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution.
Journal
Reference
Authors
Zheng C, Geetha T, Babu JR
Title
Failure of ubiquitin proteasome system: risk for neurodegenerative diseases.
Journal
Reference
Authors
Park H, Kang JH, Lee S
Title
Autophagy in Neurodegenerative Diseases: A Hunter for Aggregates.
Journal
Reference
Authors
Malik BR, Maddison DC, Smith GA, Peters OM
Title
Autophagic and endo-lysosomal dysfunction in neurodegenerative disease.
Journal
Reference
Authors
Metaxakis A, Ploumi C, Tavernarakis N
Title
Autophagy in Age-Associated Neurodegeneration.
Journal
Reference
Authors
Vidal RL, Matus S, Bargsted L, Hetz C
Title
Targeting autophagy in neurodegenerative diseases.
Journal
Reference
Authors
Menzies FM, Fleming A, Caricasole A, Bento CF, Andrews SP, Ashkenazi A, Fullgrabe J, Jackson A, Jimenez Sanchez M, Karabiyik C, Licitra F, Lopez Ramirez A, Pavel M, Puri C, Renna M, Ricketts T, Schlotawa L, Vicinanza M, Won H, Zhu Y, Skidmore J, Rubinsztein DC
Title
Autophagy and Neurodegeneration: Pathogenic Mechanisms and Therapeutic Opportunities.
Journal
Reference
Authors
Chu CT
Title
Mechanisms of selective autophagy and mitophagy: Implications for neurodegenerative diseases.
Journal
Reference
Authors
Kim I, Xu W, Reed JC
Title
Cell death and endoplasmic reticulum stress: disease relevance and therapeutic opportunities.
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
Halliday M, Hughes D, Mallucci GR
Title
Fine-tuning PERK signaling for neuroprotection.
Journal
Reference
Authors
Rozpedek-Kaminska W, Siwecka N, Wawrzynkiewicz A, Wojtczak R, Pytel D, Diehl JA, Majsterek I
Title
The PERK-Dependent Molecular Mechanisms as a Novel Therapeutic Target for Neurodegenerative Diseases.
Journal
Reference
Authors
Hetz C, Mollereau B
Title
Disturbance of endoplasmic reticulum proteostasis in neurodegenerative diseases.
Journal
Reference
Authors
Bezprozvanny I
Title
Calcium signaling and neurodegenerative diseases.
Journal
Reference
Authors
Ureshino RP, Erustes AG, Bassani TB, Wachilewski P, Guarache GC, Nascimento AC, Costa AJ, Smaili SS, Pereira GJDS
Title
The Interplay between Ca(2+) Signaling Pathways and Neurodegeneration.
Journal
Reference
Authors
Golpich M, Amini E, Mohamed Z, Azman Ali R, Mohamed Ibrahim N, Ahmadiani A
Title
Mitochondrial Dysfunction and Biogenesis in Neurodegenerative diseases: Pathogenesis and Treatment.
Journal
Reference
Authors
Morfini GA, Burns M, Binder LI, Kanaan NM, LaPointe N, Bosco DA, Brown RH Jr, Brown H, Tiwari A, Hayward L, Edgar J, Nave KA, Garberrn J, Atagi Y, Song Y, Pigino G, Brady ST
Title
Axonal transport defects in neurodegenerative diseases.
Journal
Reference
Authors
Gibbs KL, Greensmith L, Schiavo G
Title
Regulation of Axonal Transport by Protein Kinases.
Journal
Reference
Authors
Millecamps S, Julien JP
Title
Axonal transport deficits and neurodegenerative diseases.
Journal
Reference
Authors
Sleigh JN, Rossor AM, Fellows AD, Tosolini AP, Schiavo G
Title
Axonal transport and neurological disease.
Journal
Reference
Authors
Perlson E, Maday S, Fu MM, Moughamian AJ, Holzbaur EL
Title
Retrograde axonal transport: pathways to cell death?
Journal
Related pathway
llv04141 Protein processing in endoplasmic reticulum
llv04933 AGE-RAGE signaling pathway in diabetic complications
KO pathway
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