KEGG   NETWORK: N01145
Entry
N01145                      Network                                
Name
Mutation-inactivated VCP to 26S proteasome-mediated protein degradation
Definition
VCP* -| (Protein+UB) -- 26S -> Peptide
  Expanded
7415v1 -| (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
nt06464 Amyotrophic lateral sclerosis
nt06466 Pathways of neurodegeneration
Type
Variant
Pathway
hsa05014  Amyotrophic lateral sclerosis
Disease
H00058  Amyotrophic lateral sclerosis (ALS)
Gene
7415  VCP; valosin containing protein
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
7415v1 (VCP*)  VCP mutation
Metabolite
C00017  Protein
C00012  Peptide
Reference
PMID:28057298
  Authors
Lin G, Mao D, Bellen HJ
  Title
Amyotrophic Lateral Sclerosis Pathogenesis Converges on Defects in Protein Homeostasis Associated with TDP-43 Mislocalization and Proteasome-Mediated Degradation Overload.
  Journal
Curr Top Dev Biol 121:111-171 (2017)
DOI:10.1016/bs.ctdb.2016.07.004
Reference
PMID:29951484
  Authors
Esaki M, Johjima-Murata A, Islam MT, Ogura T
  Title
Biological and Pathological Implications of an Alternative ATP-Powered Proteasomal Assembly With Cdc48 and the 20S Peptidase.
  Journal
Front Mol Biosci 5:56 (2018)
DOI:10.3389/fmolb.2018.00056
Reference
PMID:26134898
  Authors
Barthelme D, Jauregui R, Sauer RT
  Title
An ALS disease mutation in Cdc48/p97 impairs 20S proteasome binding and proteolytic communication.
  Journal
Protein Sci 24:1521-7 (2015)
DOI:10.1002/pro.2740
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