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
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