General Information:

Id: 310 (click here to show other Interactions for entry)
Diseases: Amyotrophic lateral sclerosis
Mus musculus
SOD1 wt or SOD1G93A or SOD1G85R transgenic mouse, transfected with human wt SOD1 or human SOD1G93A or human SOD1G85R
BTO:0000312 motoneuron
article
Reference: Raoul C et al.(2006) Chronic activation in presymptomatic amyotrophic lateral sclerosis (ALS) mice of a feedback loop involving Fas, Daxx, and FasL Proc. Natl. Acad. Sci. U.S.A. 103: 6007-6012 [PMID: 16581901]

Interaction Information:

Comment In both control and mutant SOD1 motoneurons, exogenous NO (addition of Detanonoate) leads to up-regulation of FasL. As a reporter of Fas activation, phophorylation of p38 kinase was quantified. However, only in the presence of G93A or G85R mutant forms of SOD1 does this mechanism lead to a Fas-dependent increase in phosphorylation of p38 kinase.
Formal Description
Interaction-ID: 1224

drug/chemical compound

NO

increases_quantity of

gene/protein

FASLG

in wt and mutant mice
Comment Levels of Fas were similar between C57BL/6 and mutant SOD1 motoneurons and unchanged by the presence of NO donors such as Detanonoate.
Formal Description
Interaction-ID: 1228

drug/chemical compound

NO

NOT affects_quantity of

gene/protein

FAS

in wt and mutant mice
Drugbank entries Show/Hide entries for FAS
Comment NO leads to death of mutant, but not control, motoneurons.
Formal Description
Interaction-ID: 1232

drug/chemical compound

NO

increases_activity of

process

neuron death

in SOD1(G93A) or SOD1(G85R) mutant mice motoneurons
Comment In mutant SOD1G93A and SOD1G85R, but not in control motoneurons, the upregulation of FasL results in activation of Fas, leading through Daxx to phosphorylation of p38 and further NO synthesis by upregulation of nNOS. This Fas/NO feedback amplification loop is required for motoneuron death in vitro.
Formal Description
Interaction-ID: 13483

gene/protein

NOS1

increases_quantity of

drug/chemical compound

NO

in SOD1(G93A) or SOD1(G85R) mutant mice motoneurons
Drugbank entries Show/Hide entries for NOS1