Entry
Name
Pathways of neurodegeneration - multiple diseases - Lemur catta (Ring-tailed lemur)
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
lcat05022 Pathways of neurodegeneration - multiple diseases
Organism
Lemur catta (Ring-tailed lemur) [GN:
lcat ]
Gene
123620321 PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036 ]
123620323 CDK5R1; LOW QUALITY PROTEIN: cyclin-dependent kinase 5 activator 1 [KO:K11716 ]
123620597 PSMC5; 26S proteasome regulatory subunit 8 isoform X1 [KO:K03066 ]
123620754 ATP5MC1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128 ]
123620852 PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033 ]
123622057 PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858 ] [EC:3.1.4.11 ]
123622065 PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X1 [KO:K05858 ] [EC:3.1.4.11 ]
123622419 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435 ]
123622513 ADRM1; proteasomal ubiquitin receptor ADRM1 isoform X1 [KO:K06691 ]
123622735 PLCG1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase gamma-1 isoform X1 [KO:K01116 ] [EC:3.1.4.11 ]
123622897 VAPB; vesicle-associated membrane protein-associated protein B/C [KO:K10707 ]
123623040 TUBAL3; LOW QUALITY PROTEIN: tubulin alpha chain-like 3 [KO:K07374 ]
123623167 CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D [KO:K04851 ]
123623513 PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037 ]
123623574 ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958 ]
123623979 TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518 ]
123624366 PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858 ] [EC:3.1.4.11 ]
123624378 PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031 ]
123624681 NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947 ]
123625148 PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038 ]
123625343 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435 ]
123626088 PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693 ]
123626515 PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348 ] [EC:3.1.3.16 ]
123626606 VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041 ]
123626819 CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S [KO:K04857 ]
123626873 ATP5F1C; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136 ]
123627206 NFKB1; nuclear factor NF-kappa-B p105 subunit isoform X1 [KO:K02580 ]
123627290 PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348 ] [EC:3.1.3.16 ]
123627462 MAP1LC3C; microtubule-associated proteins 1A/1B light chain 3C [KO:K10435 ]
123627700 CAMK2D; calcium/calmodulin-dependent protein kinase type II subunit delta isoform X1 [KO:K04515 ] [EC:2.7.11.17 ]
123628649 NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351 ]
123628789 NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945 ]
123628833 CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853 ]
123630288 NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961 ]
123630321 DVL3; segment polarity protein dishevelled homolog DVL-3 isoform X1 [KO:K02353 ]
123630330 PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028 ]
123630502 NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960 ]
123630658 ATP5PF; ATP synthase-coupling factor 6, mitochondrial [KO:K02131 ]
123630777 NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944 ]
123631474 NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966 ]
123631520 WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908 ]
123631780 AGER; advanced glycosylation end product-specific receptor isoform X1 [KO:K19722 ]
123631967 DNAH8; LOW QUALITY PROTEIN: dynein axonemal heavy chain 8 [KO:K10408 ]
123632363 APC2; adenomatous polyposis coli protein 2 isoform X1 [KO:K02085 ]
123632367 GRIN2A; glutamate receptor ionotropic, NMDA 2A isoform X1 [KO:K05209 ]
123632797 NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial isoform X1 [KO:K03940 ] [EC:7.1.1.2 ]
123632820 ITPR3; inositol 1,4,5-trisphosphate receptor type 3 isoform X1 [KO:K04960 ]
123633156 FIG4; polyphosphoinositide phosphatase isoform X1 [KO:K22913 ] [EC:3.1.3.-]
123633966 PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029 ]
123634459 TNFRSF1B; tumor necrosis factor receptor superfamily member 1B isoform X1 [KO:K05141 ]
123634668 FASLG; tumor necrosis factor ligand superfamily member 6 [KO:K04389 ]
123634820 ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054 ]
123634905 CALML6; LOW QUALITY PROTEIN: calmodulin-like protein 6 [KO:K02183 ]
123635010 CSF1; macrophage colony-stimulating factor 1 isoform X1 [KO:K05453 ]
123635033 ATP5PB; ATP synthase F(0) complex subunit B1, mitochondrial isoform X1 [KO:K02127 ]
123635220 NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935 ] [EC:7.1.1.2 ]
123635223 TOMM40L; mitochondrial import receptor subunit TOM40B isoform X1 [KO:K11518 ]
123635226 SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236 ]
123635558 NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 isoform X1 [KO:K03951 ]
123635724 NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938 ]
123635747 DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410 ]
123635928 SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235 ] [EC:1.3.5.1 ]
123636020 DVL1; segment polarity protein dishevelled homolog DVL-1 isoform X1 [KO:K02353 ]
123636458 NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956 ]
123637772 VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862 ]
123637866 NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946 ]
123638412 NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353 ]
123638560 NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967 ]
123639404 APAF1; apoptotic protease-activating factor 1 isoform X1 [KO:K02084 ]
123639435 NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352 ]
123639613 NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948 ]
123639755 ATP5MC2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128 ]
123639915 ITPR2; inositol 1,4,5-trisphosphate receptor type 2 isoform X1 [KO:K04959 ]
123639999 LRP6; low-density lipoprotein receptor-related protein 6 [KO:K03068 ]
123640135 TNFRSF1A; tumor necrosis factor receptor superfamily member 1A [KO:K03158 ]
123640141 NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953 ]
123640189 CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C isoform X1 [KO:K04850 ]
123640284 NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950 ]
123640339 ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374 ]
123641403 ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906 ]
123641429 BAD; bcl2-associated agonist of cell death isoform X1 [KO:K02158 ]
123641430 PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 isoform X1 [KO:K05858 ] [EC:3.1.4.11 ]
123641691 SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial isoform X1 [KO:K00237 ]
123642542 BDNF; LOW QUALITY PROTEIN: brain-derived neurotrophic factor [KO:K04355 ]
123642652 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985 ]
123642678 PSMC3; LOW QUALITY PROTEIN: 26S proteasome regulatory subunit 6A [KO:K03065 ]
123642684 NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936 ] [EC:7.1.1.2 ]
123642816 LRP5; low-density lipoprotein receptor-related protein 5 [KO:K03068 ]
123642823 NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial [KO:K03941 ] [EC:7.1.1.2 ]
123642877 SPTBN2; spectrin beta chain, non-erythrocytic 2 isoform X1 [KO:K23932 ]
123642988 PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039 ]
123643524 NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954 ]
123643857 PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032 ]
123643900 NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959 ]
123643931 TANK; LOW QUALITY PROTEIN: TRAF family member-associated NF-kappa-B activator [KO:K12650 ]
123643962 PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030 ]
123644027 ATP5MC3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128 ]
123644439 NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965 ]
123644873 ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906 ]
123645165 RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589 ]
123645215 CHRNA7; neuronal acetylcholine receptor subunit alpha-7 [KO:K04809 ]
123645245 NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 isoform X1 [KO:K03957 ]
123645738 NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952 ]
123646197 DNAI1; dynein axonemal intermediate chain 1 isoform X1 [KO:K10409 ]
123646230 NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962 ]
123646308 GNAQ; guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634 ]
123646347 CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X1 [KO:K04849 ]
123647452 NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949 ]
123647608 CAMK2B; calcium/calmodulin-dependent protein kinase type II subunit beta isoform X1 [KO:K04515 ] [EC:2.7.11.17 ]
123647646 NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958 ]
123647696 EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
123648206 NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial isoform X1 [KO:K03939 ]
123648345 SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234 ] [EC:1.3.5.1 ]
123648644 NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937 ]
123648813 ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889 ]
123649928 FAS; tumor necrosis factor receptor superfamily member 6 isoform X1 [KO:K04390 ]
123650126 NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial isoform X1 [KO:K03964 ]
123650270 VDAC2; voltage-dependent anion-selective channel protein 2 [KO:K15040 ]
123650285 CAMK2G; calcium/calmodulin-dependent protein kinase type II subunit gamma isoform X1 [KO:K04515 ] [EC:2.7.11.17 ]
123650296 PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348 ] [EC:3.1.3.16 ]
123650594 PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035 ]
123650613 WIPI1; WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908 ]
123650852 DVL2; segment polarity protein dishevelled homolog DVL-2 isoform X1 [KO:K02353 ]
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.
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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.
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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.
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Reference
Authors
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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.
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Reference
Authors
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Title
Targeting autophagy in neurodegenerative diseases.
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Reference
Authors
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Title
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Journal
Reference
Authors
Chu CT
Title
Mechanisms of selective autophagy and mitophagy: Implications for neurodegenerative diseases.
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Reference
Authors
Kim I, Xu W, Reed JC
Title
Cell death and endoplasmic reticulum stress: disease relevance and therapeutic opportunities.
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Reference
Authors
Doyle KM, Kennedy D, Gorman AM, Gupta S, Healy SJ, Samali A
Title
Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders.
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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
lcat04141 Protein processing in endoplasmic reticulum
lcat04933 AGE-RAGE signaling pathway in diabetic complications
KO pathway