KEGG   NETWORK: N01144
Entry
N01144                      Network                                

Name
Mutation-caused aberrant SOD1 to 26S proteasome-mediated protein degradation
Definition
SOD1* -| DERL1 -> (Protein+UB) -- 26S -> Peptide
  Expanded
6647v1 -| 79139 -> (C00017+(6233,7311,7314,7316)) -- (5687+5683+5685+5688+143471+5686+5682+5684+5694+5695+5691+5690+5693+5689+5692+5701+5700+5704+5706+5702+5705+5708+5707+5709+5715+5718+5717+9861+5713+5719+5710+10213+5714+11047+7979) -> C00012
Class
nt06420 Ubiquitin-proteasome system
nt06464 Amyotrophic lateral sclerosis
Type
Variant
Pathway
hsa05014  Amyotrophic lateral sclerosis
Disease
H00058  Amyotrophic lateral sclerosis (ALS)
Gene
6647  SOD1; superoxide dismutase 1
79139  DERL1; derlin 1
6233  RPS27A; ribosomal protein S27a
7311  UBA52; ubiquitin A-52 residue ribosomal protein fusion product 1
7314  UBB; ubiquitin B
7316  UBC; ubiquitin C
5687  PSMA6; proteasome 20S subunit alpha 6
5683  PSMA2; proteasome 20S subunit alpha 2
5685  PSMA4; proteasome 20S subunit alpha 4
5688  PSMA7; proteasome 20S subunit alpha 7
143471  PSMA8; proteasome 20S subunit alpha 8
5686  PSMA5; proteasome 20S subunit alpha 5
5682  PSMA1; proteasome 20S subunit alpha 1
5684  PSMA3; proteasome 20S subunit alpha 3
5694  PSMB6; proteasome 20S subunit beta 6
5695  PSMB7; proteasome 20S subunit beta 7
5691  PSMB3; proteasome 20S subunit beta 3
5690  PSMB2; proteasome 20S subunit beta 2
5693  PSMB5; proteasome 20S subunit beta 5
5689  PSMB1; proteasome 20S subunit beta 1
5692  PSMB4; proteasome 20S subunit beta 4
5701  PSMC2; proteasome 26S subunit, ATPase 2
5700  PSMC1; proteasome 26S subunit, ATPase 1
5704  PSMC4; proteasome 26S subunit, ATPase 4
5706  PSMC6; proteasome 26S subunit, ATPase 6
5702  PSMC3; proteasome 26S subunit, ATPase 3
5705  PSMC5; proteasome 26S subunit, ATPase 5
5708  PSMD2; proteasome 26S subunit, non-ATPase 2
5707  PSMD1; proteasome 26S subunit, non-ATPase 1
5709  PSMD3; proteasome 26S subunit, non-ATPase 3
5715  PSMD9; proteasome 26S subunit, non-ATPase 9
5718  PSMD12; proteasome 26S subunit, non-ATPase 12
5717  PSMD11; proteasome 26S subunit, non-ATPase 11
9861  PSMD6; proteasome 26S subunit, non-ATPase 6
5713  PSMD7; proteasome 26S subunit, non-ATPase 7
5719  PSMD13; proteasome 26S subunit, non-ATPase 13
5710  PSMD4; proteasome 26S subunit, non-ATPase 4
10213  PSMD14; proteasome 26S subunit, non-ATPase 14
5714  PSMD8; proteasome 26S subunit, non-ATPase 8
11047  ADRM1; adhesion regulating molecule 1
7979  SEM1; SEM1 26S proteasome complex subunit
Variant
6647v1 (SOD1*)  SOD1 mutation
Metabolite
C00017  Protein
C00012  Peptide
Reference
  Authors
Nishitoh H, Kadowaki H, Nagai A, Maruyama T, Yokota T, Fukutomi H, Noguchi T, Matsuzawa A, Takeda K, Ichijo H
  Title
ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1.
  Journal
Genes Dev 22:1451-64 (2008)
DOI:10.1101/gad.1640108
Reference
  Authors
Webster CP, Smith EF, Shaw PJ, De Vos KJ
  Title
Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?
  Journal
Front Mol Neurosci 10:123 (2017)
DOI:10.3389/fnmol.2017.00123

  All links  
Pathway (1)   
   KEGG PATHWAY (1)   
Disease (1)   
   KEGG DISEASE (1)   
Chemical substance (2)   
   KEGG COMPOUND (2)   
Gene (41)   
   KEGG GENES (41)   
Literature (2)   
   PubMed (2)   
All databases (47)   

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