Entry
Name
Pathways of neurodegeneration - multiple diseases - Propithecus coquereli (Coquerel's sifaka)
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
pcoq05022 Pathways of neurodegeneration - multiple diseases
Organism
Propithecus coquereli (Coquerel's sifaka) [GN:
pcoq ]
Gene
105804954 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435 ]
105804976 PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037 ]
105805081 TNFRSF1A; tumor necrosis factor receptor superfamily member 1A [KO:K03158 ]
105805232 ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374 ]
105805267 NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950 ]
105805290 CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X1 [KO:K04851 ]
105805988 RAC1; ras-related C3 botulinum toxin substrate 1 isoform X1 [KO:K04392 ]
105806035 WIPI2; WD repeat domain phosphoinositide-interacting protein 2 isoform X1 [KO:K17908 ]
105806167 PLCB1; LOW QUALITY PROTEIN: 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 [KO:K05858 ] [EC:3.1.4.11 ]
105806182 PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858 ] [EC:3.1.4.11 ]
105806237 NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961 ]
105806404 ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054 ]
105806434 NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935 ] [EC:7.1.1.2 ]
105806450 TOMM40L; mitochondrial import receptor subunit TOM40B [KO:K11518 ]
105806914 ATP5J; ATP synthase-coupling factor 6, mitochondrial isoform X1 [KO:K02131 ]
105807364 DVL3; segment polarity protein dishevelled homolog DVL-3 isoform X1 [KO:K02353 ]
105807521 DNAH12; LOW QUALITY PROTEIN: dynein heavy chain 12, axonemal [KO:K10408 ]
105807577 PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028 ]
105807952 NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951 ]
105808667 NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960 ]
105808941 CHRNA7; neuronal acetylcholine receptor subunit alpha-7 isoform X1 [KO:K04809 ]
105808983 NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963 ]
105809556 NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947 ]
105810116 DNAH14; LOW QUALITY PROTEIN: dynein heavy chain 14, axonemal [KO:K10408 ]
105810175 NEFH; LOW QUALITY PROTEIN: neurofilament heavy polypeptide [KO:K04574 ]
105811072 NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936 ] [EC:7.1.1.2 ]
105811088 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985 ]
105811289 NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940 ] [EC:7.1.1.2 ]
105811699 IFT57; LOW QUALITY PROTEIN: intraflagellar transport protein 57 homolog [KO:K04638 ]
105811892 NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956 ]
105812318 NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938 ]
105812321 DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410 ]
105812493 CAMK2B; calcium/calmodulin-dependent protein kinase type II subunit beta isoform X1 [KO:K04515 ] [EC:2.7.11.17 ]
105812706 ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958 ]
105813016 GRIN2A; glutamate receptor ionotropic, NMDA 2A isoform X1 [KO:K05209 ]
105813050 NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966 ]
105813564 PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036 ]
105814705 NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial [KO:K03958 ]
105815261 TRPC3; short transient receptor potential channel 3 isoform X1 [KO:K04966 ]
105815301 NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957 ]
105815495 GNAQ; guanine nucleotide-binding protein G(q) subunit alpha [KO:K04634 ]
105815572 ATP5G3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128 ]
105815791 CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C isoform X1 [KO:K04850 ]
105816029 VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862 ]
105816542 NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948 ]
105816545 PSMD13; 26S proteasome non-ATPase regulatory subunit 13 [KO:K03039 ]
105816581 PLCG1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase gamma-1 isoform X1 [KO:K01116 ] [EC:3.1.4.11 ]
105816719 PSMD1; LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032 ]
105816783 CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853 ]
105816836 NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351 ]
105817103 NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949 ]
105817645 TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518 ]
105817852 AGER; advanced glycosylation end product-specific receptor isoform X1 [KO:K19722 ]
105817968 ATP5G2; ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128 ]
105818168 NDUFA13; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353 ]
105818713 GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208 ]
105818751 NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967 ]
105819128 NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937 ]
105819191 NFKB1; nuclear factor NF-kappa-B p105 subunit isoform X1 [KO:K02580 ]
105819421 NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959 ]
105819434 DVL2; segment polarity protein dishevelled homolog DVL-2 isoform X1 [KO:K02353 ]
105819510 LRP6; low-density lipoprotein receptor-related protein 6 [KO:K03068 ]
105819542 PSMD14; LOW QUALITY PROTEIN: 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030 ]
105819572 TANK; LOW QUALITY PROTEIN: TRAF family member-associated NF-kappa-B activator [KO:K12650 ]
105819681 VDAC3; voltage-dependent anion-selective channel protein 3 [KO:K15041 ]
105819705 DNAI1; dynein intermediate chain 1, axonemal isoform X1 [KO:K10409 ]
105819858 EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
105820089 VAPB; vesicle-associated membrane protein-associated protein B/C isoform X1 [KO:K10707 ]
105820430 PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035 ]
105820583 PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031 ]
105820631 NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962 ]
105820913 CALML6; LOW QUALITY PROTEIN: calmodulin-like protein 6 [KO:K02183 ]
105820951 DVL1; segment polarity protein dishevelled homolog DVL-1 isoform X1 [KO:K02353 ]
105821039 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435 ]
105821114 FASLG; tumor necrosis factor ligand superfamily member 6 isoform X1 [KO:K04389 ]
105821432 DNAH8; LOW QUALITY PROTEIN: dynein heavy chain 8, axonemal [KO:K10408 ]
105821611 PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform [KO:K04348 ] [EC:3.1.3.16 ]
105821915 PSMD4; 26S proteasome non-ATPase regulatory subunit 4 isoform X1 [KO:K03029 ]
105822085 ATP5F1; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127 ]
105822197 SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237 ]
105822691 FAS; tumor necrosis factor receptor superfamily member 6 [KO:K04390 ]
105822913 NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial isoform X1 [KO:K03944 ]
105823056 SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 [KO:K00234 ] [EC:1.3.5.1 ]
105823339 PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038 ]
105823587 NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 [KO:K03965 ]
105824035 NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946 ]
105824092 RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589 ]
105824114 NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939 ]
105824126 PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033 ]
105824308 NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964 ]
105824734 ATP5G1; ATP synthase F(0) complex subunit C1, mitochondrial [KO:K02128 ]
105824798 WIPI1; WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908 ]
105824864 SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235 ] [EC:1.3.5.1 ]
105824987 TNFRSF1B; tumor necrosis factor receptor superfamily member 1B isoform X1 [KO:K05141 ]
105825283 PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform [KO:K04348 ] [EC:3.1.3.16 ]
105825488 VDAC2; voltage-dependent anion-selective channel protein 2 isoform X1 [KO:K15040 ]
105825498 PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348 ] [EC:3.1.3.16 ]
105825513 MCU; calcium uniporter protein, mitochondrial isoform X1 [KO:K20858 ]
105825541 CSF1; macrophage colony-stimulating factor 1 isoform X1 [KO:K05453 ]
105825547 NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954 ]
105825671 PSMD9; 26S proteasome non-ATPase regulatory subunit 9 isoform X1 [KO:K06693 ]
105825918 ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889 ]
105826090 NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352 ]
105826272 NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945 ]
105826451 NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952 ]
105826626 WDR41; LOW QUALITY PROTEIN: WD repeat-containing protein 41 [KO:K23610 ]
105826816 LRP5; low-density lipoprotein receptor-related protein 5 [KO:K03068 ]
105826962 SMCR8; smith-Magenis syndrome chromosomal region candidate gene 8 protein [KO:K23611 ]
105827297 PLCB3; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-3 isoform X1 [KO:K05858 ] [EC:3.1.4.11 ]
105827410 ATG2A; autophagy-related protein 2 homolog A isoform X1 [KO:K17906 ]
105827413 NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941 ] [EC:7.1.1.2 ]
105827450 SPTBN2; spectrin beta chain, non-erythrocytic 2 isoform X1 [KO:K23932 ]
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
pcoq04141 Protein processing in endoplasmic reticulum
pcoq04933 AGE-RAGE signaling pathway in diabetic complications
KO pathway