KEGG   NETWORK: N01147
Entry
N01147                      Network                                

Name
Mutation-caused aberrant SOD1 to ATF6-mediated transcription
Definition
SOD1* -| BIP -| ATF6 => DDIT3
  Expanded
6647v1 -| 3309 -| 22926 => 1649
Class
nt06412 Unfolded protein response (UPR) signaling
nt06464 Amyotrophic lateral sclerosis
Type
Variant
Pathway
hsa05014  Amyotrophic lateral sclerosis
Disease
H00058  Amyotrophic lateral sclerosis (ALS)
Gene
6647  SOD1; superoxide dismutase 1
3309  HSPA5; heat shock protein family A (Hsp70) member 5
22926  ATF6; activating transcription factor 6
1649  DDIT3; DNA damage inducible transcript 3
Variant
6647v1 (SOD1*)  SOD1 mutation
Reference
  Authors
Kikuchi H, Almer G, Yamashita S, Guegan C, Nagai M, Xu Z, Sosunov AA, McKhann GM 2nd, Przedborski S
  Title
Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model.
  Journal
Proc Natl Acad Sci U S A 103:6025-30 (2006)
DOI:10.1073/pnas.0509227103
Reference
  Authors
Walker AK, Atkin JD
  Title
Stress signaling from the endoplasmic reticulum: A central player in the pathogenesis of amyotrophic lateral sclerosis.
  Journal
IUBMB Life 63:754-63 (2011)
DOI:10.1002/iub.520
Reference
  Authors
Turner BJ, Atkin JD
  Title
ER stress and UPR in familial amyotrophic lateral sclerosis.
  Journal
Curr Mol Med 6:79-86 (2006)
DOI:10.2174/156652406775574550
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
J Cell Mol Med 15:2025-39 (2011)
DOI:10.1111/j.1582-4934.2011.01374.x

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