General Information:

Id: 222 (click here to show other Interactions for entry)
Diseases: Amyotrophic lateral sclerosis
Homo sapiens
SOD1G85R or SOD1G93A transgenic cells
BTO:0000007 HEK-293 cell
article
Reference: Ying Z et al.(2009) Gp78, an ER associated E3, promotes SOD1 and ataxin-3 degradation. Hum. Mol. Genet. 18: 4268-4281 [PMID: 19661182]

Interaction Information:

Comment Gp78 interacts with and ubiquitinates SOD1, but more ubiquitinated mutant SOD1 (SOD1G85R, SOD1G93A) than wild-type SOD1 was observed.
Formal Description
Interaction-ID: 13337

gene/protein

AMFR

increases_ubiquitination/sumoylation of

gene/protein mutant

SOD1-p.G93A

Comment Gp78 interacts with and ubiquitinates SOD1. The amounts of gp78 co-immunoprecipitated were higher in cells co-transfected with mutant SOD1s than in that co-transfected with wild-type SOD1, suggesting stronger interactions between endogenous gp78 and mutant SOD1.
Formal Description
Interaction-ID: 13338

gene/protein

AMFR

interacts (colocalizes) with

gene/protein mutant

SOD1-p.G93A

Comment Gp78 specifically stimulates the degradation of SOD1, (SOD1WT, SOD1G85R, SOD1G93A). As degradation of SOD1 could be inhibited by MG132 (a proteasome inhibitor) the data suggested that gp78-mediated degradation of SOD1 is proteasome-dependent.
Formal Description
Interaction-ID: 13339

gene/protein

AMFR

decreases_quantity of

gene/protein mutant

SOD1-p.G93A