General Information:

Id: 172 (click here to show other Interactions for entry)
Diseases: Amyotrophic lateral sclerosis
Mus musculus
SOD1(G93A) fast-mice
BTO:0000312 motoneuron (vulnerable)
article
Reference: Saxena S et al.(2009) A role for motoneuron subtype-selective ER stress in disease manifestations of FALS mice Nat. Neurosci. 12: 627-636 [PMID: 19330001]

Interaction Information:

Comment From stage P12 on, VUL but not RES motoneurons (VUL=vulnerable to motoneuron disease, RES=resistant to) of G93A-fast mice specifically showed an upregulation of genes involved in stress-related pathways, including protein ubiquitination, hypoxia and NRF2-mediated stress pathways. This stress-related response increased up to P26 but was abruptly lost at P32 and beyond.
Formal Description
Interaction-ID: 691

gene/protein mutant

SOD1-p.G93A

increases_activity of

in motoneurons; from stage P12 up to P26
Comment Early salubrinal treatment led to low levels of BiP, no evidence of a UPR and no microglial activation at stage P38 and to early signs of a UPR, mild microglial activation and no loss of synaptic vesicles at neuromuscular junctions at stage P53 in mutant SOD1-G93A-fast mice (VUL) motoneurons.
Formal Description
Interaction-ID: 746

drug/chemical compound

Salubrinal

decreases_activity of

in mutant SOD1-G93A-fast mice
Comment The stress-protecting factor CNTF effectively attenuated the axonal pathology in VUL motoneurons in mutant G93A-fast-mice.
Formal Description
Interaction-ID: 747

gene/protein

CNTF

decreases_activity of

in mutant SOD1-G93A-fast mice