PATHWAY: puc05022 Help
Entry
Name
Pathways of neurodegeneration - multiple diseases - Panthera uncia (snow leopard)
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
puc05022 Pathways of neurodegeneration - multiple diseases
Ortholog table
Organism
Panthera uncia (snow leopard) [GN:
puc ]
Gene
125925154 guanine nucleotide-binding protein G(q) subunit alpha isoform X1 [KO:K04634 ]
125929585 inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958 ]
125920161 inositol 1,4,5-trisphosphate receptor type 2 isoform X1 [KO:K04959 ]
125920056 voltage-dependent L-type calcium channel subunit alpha-1C isoform X1 [KO:K04850 ]
125931680 voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853 ]
125925380 voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857 ]
125911014 LOW QUALITY PROTEIN: ubiquitin-60S ribosomal protein L40-like [KO:K02927 ]
125931749 LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 1-like [KO:K03032 ]
125926490 26S proteasome non-ATPase regulatory subunit 3 isoform X1 [KO:K03033 ]
125937741 26S proteasome non-ATPase regulatory subunit 13 isoform X1 [KO:K03039 ]
125912102 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029 ]
125911593 26S proteasome non-ATPase regulatory subunit 14 isoform X1 [KO:K03030 ]
125936455 vesicle-associated membrane protein-associated protein B/C [KO:K10707 ]
125925955 cyclic AMP-dependent transcription factor ATF-6 alpha isoform X1 [KO:K09054 ]
125920691 NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944 ]
125932271 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945 ]
125935595 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946 ]
125915573 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 isoform X1 [KO:K03947 ]
125909361 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947 ]
125919235 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948 ]
125930105 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 isoform X1 [KO:K03949 ]
125935199 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5-like [KO:K03949 ]
125920384 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950 ]
125928955 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 isoform X1 [KO:K03951 ]
125920952 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952 ]
125918751 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953 ]
125911004 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954 ]
125928903 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956 ]
125933363 LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12-like [KO:K11352 ]
125920326 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352 ]
125930638 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353 ]
125909241 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 isoform X1 [KO:K03957 ]
125917067 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1-like [KO:K03957 ]
125917871 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1-like [KO:K03957 ]
125930928 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958 ]
125911778 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 isoform X1 [KO:K03959 ]
125921781 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960 ]
125923979 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4-like [KO:K03960 ]
125921863 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial isoform X1 [KO:K03961 ]
125918669 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 isoform X1 [KO:K03962 ]
125929104 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963 ]
125933435 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X1 [KO:K03964 ]
125924744 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965 ]
125927170 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966 ]
125931635 NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351 ]
125912613 NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial isoform X1 [KO:K03936 ] [EC:7.1.1.2 ]
125934593 NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial isoform X1 [KO:K03937 ]
125912737 NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938 ]
125934737 NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939 ]
125928801 NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940 ] [EC:7.1.1.2 ]
125930376 NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941 ] [EC:7.1.1.2 ]
125923709 NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967 ]
125932349 LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial-like [KO:K03967 ]
125930990 LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 subunit C2-like [KO:K03968 ]
125934758 succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234 ] [EC:1.3.5.1 ]
125925571 succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236 ]
125909889 succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237 ]
125929310 cytochrome b-c1 complex subunit 1, mitochondrial isoform X1 [KO:K00414 ]
125912658 cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416 ]
125916388 cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263 ]
125936705 cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263 ]
125937501 cytochrome c oxidase subunit 7A-related protein, mitochondrial isoform X1 [KO:K02270 ]
125924277 ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128 ]
125911414 ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128 ]
125919131 ATP synthase F(0) complex subunit C2, mitochondrial isoform X1 [KO:K02128 ]
125919974 ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128 ]
125912786 ATP synthase F(0) complex subunit C1, mitochondrial-like [KO:K02128 ]
125928983 ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128 ]
125933210 LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 1-like [KO:K05862 ]
125925576 mitochondrial import receptor subunit TOM40B isoform X1 [KO:K11518 ]
125923333 serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348 ] [EC:3.1.3.16 ]
125932723 serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348 ] [EC:3.1.3.16 ]
125933220 tumor necrosis factor receptor superfamily member 6 isoform X1 [KO:K04390 ]
125938035 LOW QUALITY PROTEIN: voltage-dependent anion-selective channel protein 3-like [KO:K15041 ]
125925097 microtubule-associated proteins 1A/1B light chain 3C-like [KO:K10435 ]
125917385 microtubule-associated proteins 1A/1B light chain 3B-like [KO:K10435 ]
125917395 microtubule-associated proteins 1A/1B light chain 3B-like [KO:K10435 ]
125911592 TRAF family member-associated NF-kappa-B activator isoform X1 [KO:K12650 ]
125912665 activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985 ]
125917068 WD repeat domain phosphoinositide-interacting protein 2 [KO:K17908 ]
125917872 WD repeat domain phosphoinositide-interacting protein 2 [KO:K17908 ]
125926696 WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908 ]
125909368 zinc finger FYVE domain-containing protein 1 isoform X1 [KO:K17603 ]
125928294 ras-related C3 botulinum toxin substrate 1 isoform X1 [KO:K04392 ]
125938790 polyphosphoinositide phosphatase isoform X1 [KO:K22913 ] [EC:3.1.3.-]
125927067 LOW QUALITY PROTEIN: segment polarity protein dishevelled homolog DVL-2 [KO:K02353 ]
125913233 LOW QUALITY PROTEIN: segment polarity protein dishevelled homolog DVL-1 [KO:K02353 ]
125938409 advanced glycosylation end product-specific receptor isoform X1 [KO:K19722 ]
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
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KO pathway
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