PATHWAY: mpuf05022 Help
Entry
Name
Pathways of neurodegeneration - multiple diseases - Mustela putorius furo (domestic ferret)
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
mpuf05022 Pathways of neurodegeneration - multiple diseases
Ortholog table
Organism
Mustela putorius furo (domestic ferret) [GN:
mpuf ]
Gene
101687155 SPTBN2; spectrin beta chain, non-erythrocytic 2 isoform X1 [KO:K23932 ]
101674115 GNAQ; guanine nucleotide-binding protein G(q) subunit alpha isoform X1 [KO:K04634 ]
101679345 PLCB4; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-4 isoform X1 [KO:K05858 ] [EC:3.1.4.11 ]
101673753 PLCB2; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-2 isoform X1 [KO:K05858 ] [EC:3.1.4.11 ]
101678347 PLCB1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase beta-1 isoform X2 [KO:K05858 ] [EC:3.1.4.11 ]
101685356 PLCG1; 1-phosphatidylinositol 4,5-bisphosphate phosphodiesterase gamma-1 isoform X1 [KO:K01116 ] [EC:3.1.4.11 ]
101675176 TRPC3; short transient receptor potential channel 3 isoform X1 [KO:K04966 ]
101677799 ITPR1; inositol 1,4,5-trisphosphate receptor type 1 isoform X1 [KO:K04958 ]
101683487 GRIN1; glutamate receptor ionotropic, NMDA 1 isoform X1 [KO:K05208 ]
101672107 GRIN2A; glutamate receptor ionotropic, NMDA 2A isoform X1 [KO:K05209 ]
101673094 CACNA1B; voltage-dependent N-type calcium channel subunit alpha-1B isoform X2 [KO:K04849 ]
101679746 CACNA1C; voltage-dependent L-type calcium channel subunit alpha-1C isoform X10 [KO:K04850 ]
101669870 CACNA1D; voltage-dependent L-type calcium channel subunit alpha-1D isoform X1 [KO:K04851 ]
101689134 CACNA1F; voltage-dependent L-type calcium channel subunit alpha-1F isoform X1 [KO:K04853 ]
101688476 CACNA1S; voltage-dependent L-type calcium channel subunit alpha-1S isoform X1 [KO:K04857 ]
101681443 CHRNA7; neuronal acetylcholine receptor subunit alpha-7 [KO:K04809 ]
101692211 VCP; transitional endoplasmic reticulum ATPase isoform X1 [KO:K13525 ]
101689832 PSMC6; 26S protease regulatory subunit 10B isoform X1 [KO:K03064 ]
101679423 PSMD2; 26S proteasome non-ATPase regulatory subunit 2 [KO:K03028 ]
101677647 PSMD1; 26S proteasome non-ATPase regulatory subunit 1 [KO:K03032 ]
101689542 PSMD3; 26S proteasome non-ATPase regulatory subunit 3 [KO:K03033 ]
101681801 PSMD9; 26S proteasome non-ATPase regulatory subunit 9 [KO:K06693 ]
101682559 PSMD12; 26S proteasome non-ATPase regulatory subunit 12 [KO:K03035 ]
101682352 PSMD11; 26S proteasome non-ATPase regulatory subunit 11 [KO:K03036 ]
101670551 PSMD6; 26S proteasome non-ATPase regulatory subunit 6 [KO:K03037 ]
101690051 PSMD7; 26S proteasome non-ATPase regulatory subunit 7 [KO:K03038 ]
101674526 PSMD13; 26S proteasome non-ATPase regulatory subunit 13 isoform X2 [KO:K03039 ]
101672325 PSMD4; 26S proteasome non-ATPase regulatory subunit 4 [KO:K03029 ]
101670572 PSMD14; 26S proteasome non-ATPase regulatory subunit 14 [KO:K03030 ]
101687128 PSMD8; 26S proteasome non-ATPase regulatory subunit 8 [KO:K03031 ]
101691521 VAPB; vesicle-associated membrane protein-associated protein B/C isoform X1 [KO:K10707 ]
101689116 ATF6; cyclic AMP-dependent transcription factor ATF-6 alpha [KO:K09054 ]
101689747 EIF2AK3; LOW QUALITY PROTEIN: eukaryotic translation initiation factor 2-alpha kinase 3 [KO:K08860 ] [EC:2.7.11.1 ]
101692556 EIF2S1; eukaryotic translation initiation factor 2 subunit 1 [KO:K03237 ]
101680524 ATF4; cyclic AMP-dependent transcription factor ATF-4 [KO:K04374 ]
101679801 NDUFV3; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial [KO:K03944 ]
101683103 NDUFA1; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 1 [KO:K03945 ]
101681305 NDUFA2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 2 [KO:K03946 ]
101671885 NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3-like [KO:K03947 ]
101678258 NDUFA3; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 3 [KO:K03947 ]
101678946 NDUFA4L2; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 4-like 2 [KO:K03948 ]
101688416 NDUFA5; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 5 [KO:K03949 ]
101673253 NDUFA6; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 6 [KO:K03950 ]
101686920 NDUFA7; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 7 [KO:K03951 ]
101675000 NDUFA8; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 8 [KO:K03952 ]
101680168 NDUFA9; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 9, mitochondrial [KO:K03953 ]
101693615 NDUFA10; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 10, mitochondrial [KO:K03954 ]
101684673 NDUFA11; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 11 [KO:K03956 ]
101674482 NDUFA12; NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 12 [KO:K11352 ]
101679389 NDUFA13; LOW QUALITY PROTEIN: NADH dehydrogenase [ubiquinone] 1 alpha subcomplex subunit 13 [KO:K11353 ]
101694062 NDUFB1; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 1 [KO:K03957 ]
101674641 NDUFB2; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 2, mitochondrial isoform X2 [KO:K03958 ]
101683892 NDUFB3; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 3 [KO:K03959 ]
101691663 NDUFB4; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 4 [KO:K03960 ]
101687434 NDUFB5; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 5, mitochondrial [KO:K03961 ]
101688606 NDUFB6; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 6 [KO:K03962 ]
101675882 NDUFB7; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 7 [KO:K03963 ]
101675804 NDUFB8; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 8, mitochondrial [KO:K03964 ]
101682240 NDUFB9; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 9 isoform X1 [KO:K03965 ]
101681324 NDUFB10; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 10 [KO:K03966 ]
101682983 NDUFB11; NADH dehydrogenase [ubiquinone] 1 beta subcomplex subunit 11, mitochondrial [KO:K11351 ]
101688652 NDUFS2; NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial [KO:K03935 ] [EC:7.1.1.2 ]
101686313 NDUFS3; NADH dehydrogenase [ubiquinone] iron-sulfur protein 3, mitochondrial [KO:K03936 ] [EC:7.1.1.2 ]
101674933 NDUFS4; NADH dehydrogenase [ubiquinone] iron-sulfur protein 4, mitochondrial [KO:K03937 ]
101684469 NDUFS5; NADH dehydrogenase [ubiquinone] iron-sulfur protein 5 [KO:K03938 ]
101692362 NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial-like [KO:K03939 ]
101688718 NDUFS6; NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial [KO:K03939 ]
101674706 NDUFS7; NADH dehydrogenase [ubiquinone] iron-sulfur protein 7, mitochondrial [KO:K03940 ] [EC:7.1.1.2 ]
101679983 NDUFS8; NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial isoform X1 [KO:K03941 ] [EC:7.1.1.2 ]
101691647 NDUFC1; NADH dehydrogenase [ubiquinone] 1 subunit C1, mitochondrial [KO:K03967 ]
101678731 SDHA; succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial [KO:K00234 ] [EC:1.3.5.1 ]
101692858 SDHB; succinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrial [KO:K00235 ] [EC:1.3.5.1 ]
101685355 LOW QUALITY PROTEIN: succinate dehydrogenase cytochrome b560 subunit, mitochondrial-like [KO:K00236 ]
101680264 SDHC; succinate dehydrogenase cytochrome b560 subunit, mitochondrial [KO:K00236 ]
101694292 SDHD; succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial [KO:K00237 ]
101685926 LOW QUALITY PROTEIN: cytochrome b-c1 complex subunit 2, mitochondrial-like [KO:K00415 ]
101681748 cytochrome b-c1 complex subunit 6, mitochondrial isoform X1 [KO:K00416 ]
101680163 cytochrome c oxidase subunit 4 isoform 1, mitochondrial [KO:K02263 ]
101676075 cytochrome c oxidase subunit 4 isoform 2, mitochondrial isoform X1 [KO:K02263 ]
101672219 cytochrome c oxidase subunit 7A-related protein, mitochondrial [KO:K02270 ]
101689814 ATP5C1; ATP synthase subunit gamma, mitochondrial isoform X1 [KO:K02136 ]
101683533 ATP5F1; ATP synthase F(0) complex subunit B1, mitochondrial [KO:K02127 ]
101672934 ATP5G1; ATP synthase F(0) complex subunit C1, mitochondrial isoform X1 [KO:K02128 ]
101673943 ATP5G2; LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C2, mitochondrial [KO:K02128 ]
106005210 LOW QUALITY PROTEIN: uncharacterized protein LOC106005210 [KO:K02128 ]
106006495 LOW QUALITY PROTEIN: ATP synthase F(0) complex subunit C2, mitochondrial-like [KO:K02128 ]
101690076 ATP5G3; ATP synthase F(0) complex subunit C3, mitochondrial [KO:K02128 ]
101679422 VDAC1; voltage-dependent anion-selective channel protein 1 [KO:K05862 ]
101678959 TOMM40L; mitochondrial import receptor subunit TOM40B [KO:K11518 ]
101680015 TOMM40; mitochondrial import receptor subunit TOM40 homolog [KO:K11518 ]
101679131 MCU; calcium uniporter protein, mitochondrial isoform X1 [KO:K20858 ]
101674924 CALML4; LOW QUALITY PROTEIN: calmodulin-like protein 4 [KO:K02183 ]
101675854 PPP3CC; serine/threonine-protein phosphatase 2B catalytic subunit gamma isoform isoform X1 [KO:K04348 ] [EC:3.1.3.16 ]
101675984 PPP3CB; serine/threonine-protein phosphatase 2B catalytic subunit beta isoform isoform X1 [KO:K04348 ] [EC:3.1.3.16 ]
101682554 PPP3CA; serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform isoform X1 [KO:K04348 ] [EC:3.1.3.16 ]
101687517 FASLG; tumor necrosis factor ligand superfamily member 6 [KO:K04389 ]
101677976 FAS; tumor necrosis factor receptor superfamily member 6 isoform X1 [KO:K04390 ]
101674296 VDAC2; voltage-dependent anion-selective channel protein 2 isoform X1 [KO:K15040 ]
101687790 VDAC3; voltage-dependent anion-selective channel protein 3 isoform X1 [KO:K15041 ]
101681973 IFT57; intraflagellar transport protein 57 homolog isoform X1 [KO:K04638 ]
101675711 MAP1LC3B; microtubule-associated proteins 1A/1B light chain 3B [KO:K10435 ]
101675872 MAP1LC3A; microtubule-associated proteins 1A/1B light chain 3A [KO:K10435 ]
101694085 TANK; TRAF family member-associated NF-kappa-B activator isoform X1 [KO:K12650 ]
101670526 SMCR8; Smith-Magenis syndrome chromosomal region candidate gene 8 protein [KO:K23611 ]
101692151 RB1CC1; RB1-inducible coiled-coil protein 1 isoform X1 [KO:K17589 ]
101680997 AMBRA1; activating molecule in BECN1-regulated autophagy protein 1 isoform X1 [KO:K17985 ]
101684646 ATG14; beclin 1-associated autophagy-related key regulator [KO:K17889 ]
101691773 ATG2B; autophagy-related protein 2 homolog B isoform X1 [KO:K17906 ]
101674575 ATG2A; autophagy-related protein 2 homolog A isoform X2 [KO:K17906 ]
101688271 WIPI2; WD repeat domain phosphoinositide-interacting protein 2 [KO:K17908 ]
101676578 WIPI1; WD repeat domain phosphoinositide-interacting protein 1 [KO:K17908 ]
101691222 ZFYVE1; zinc finger FYVE domain-containing protein 1 isoform X1 [KO:K17603 ]
101693834 RAC1; ras-related C3 botulinum toxin substrate 1 isoform X1 [KO:K04392 ]
101692520 FIG4; polyphosphoinositide phosphatase isoform X1 [KO:K22913 ] [EC:3.1.3.-]
101676562 TNFRSF1A; tumor necrosis factor receptor superfamily member 1A [KO:K03158 ]
101689344 TNFRSF1B; tumor necrosis factor receptor superfamily member 1B [KO:K05141 ]
101679788 CCS; copper chaperone for superoxide dismutase isoform X1 [KO:K04569 ]
101687038 BDNF; LOW QUALITY PROTEIN: brain-derived neurotrophic factor [KO:K04355 ]
101692651 DNALI1; axonemal dynein light intermediate polypeptide 1 [KO:K10410 ]
101679241 DNAI1; dynein intermediate chain 1, axonemal isoform X2 [KO:K10409 ]
101681123 CAMK2G; calcium/calmodulin-dependent protein kinase type II subunit gamma isoform X1 [KO:K04515 ] [EC:2.7.11.17 ]
101682911 CAMK2A; calcium/calmodulin-dependent protein kinase type II subunit alpha isoform X1 [KO:K04515 ] [EC:2.7.11.17 ]
101670702 CAMK2D; calcium/calmodulin-dependent protein kinase type II subunit delta isoform X1 [KO:K04515 ] [EC:2.7.11.17 ]
101684734 CAMK2B; calcium/calmodulin-dependent protein kinase type II subunit beta isoform X2 [KO:K04515 ] [EC:2.7.11.17 ]
101692817 LRP6; low-density lipoprotein receptor-related protein 6 [KO:K03068 ]
101674621 LRP5; low-density lipoprotein receptor-related protein 5 [KO:K03068 ]
101672500 DVL3; segment polarity protein dishevelled homolog DVL-3 [KO:K02353 ]
101676333 DVL2; segment polarity protein dishevelled homolog DVL-2 isoform X1 [KO:K02353 ]
101677876 DVL1; segment polarity protein dishevelled homolog DVL-1 isoform X1 [KO:K02353 ]
101673283 APC2; adenomatous polyposis coli protein 2 isoform X1 [KO:K02085 ]
101682399 AGER; advanced glycosylation end product-specific receptor isoform X1 [KO:K19722 ]
101680542 NFKB1; nuclear factor NF-kappa-B p105 subunit isoform X1 [KO:K02580 ]
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
mpuf04141 Protein processing in endoplasmic reticulum
mpuf04933 AGE-RAGE signaling pathway in diabetic complications
KO pathway
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