General Information:

Id: 101 (click here to show other Interactions for entry)
Diseases: Amyotrophic lateral sclerosis
Mus musculus
BTO:0004488 NSC-34 cell; BTO:0000312 motoneuron
article
Reference: Nishitoh H et al.(2008) ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1 Genes Dev 22: 1451-1464 [PMID: 18519638]

Interaction Information:

Comment SOD1mut triggers ER stress. SOD1mut (SOD1(A4V), SOD1(G85R), and SOD1(G93A)) but not wild-type SOD1 (SOD1wt) activated IRE1 and PERK.
Formal Description
Interaction-ID: 341

gene/protein mutant

SOD1-p.G93A

increases_activity of

gene/protein

ERN1

in motor neurons
Drugbank entries Show/Hide entries for ERN1
Comment SOD1mut triggers ER stress. SOD1mut (SOD1(A4V), SOD1(G85R), and SOD1(G93A)) but not wild-type SOD1 (SOD1wt) activated IRE1 and PERK.
Formal Description
Interaction-ID: 343

gene/protein mutant

SOD1-p.G93A

affects_activity of

process

response to endoplasmic reticulum stress

in motor neurons
Comment SOD1mut triggers ER stress. SOD1mut (SOD1(A4V), SOD1(G85R), and SOD1(G93A)) but not wild-type SOD1 (SOD1wt) activated IRE1 and PERK.
Formal Description
Interaction-ID: 346

gene/protein mutant

SOD1-p.G93A

increases_activity of

gene/protein

EIF2AK3

in motor neurons
Comment Expression of SOD1mut SOD1(A4V), SOD1(G85R), and SOD1(G93A), but not SOD1wt, activated endogenous ASK1.
Formal Description
Interaction-ID: 347

gene/protein mutant

SOD1-p.G93A

increases_activity of

gene/protein

MAP3K5

in motor neurons
Comment SOD1mut (SOD1(A4V), SOD1(G85R), and SOD1(G93A) induced the expression of the ER stress marker protein CHOP.
Formal Description
Interaction-ID: 353

gene/protein mutant

SOD1-p.G93A

increases_quantity of

gene/protein

DDIT3

in motor neurons
Comment SOD1mut (SOD1(A4V), SOD1(G85R), and SOD1(G93A) induced the expression of the ER stress marker protein BIP.
Formal Description
Interaction-ID: 356

gene/protein mutant

SOD1-p.G93A

increases_quantity of

gene/protein

HSPA5

in motor neurons
Drugbank entries Show/Hide entries for HSPA5
Comment SOD1mut (SOD1(A4V), SOD1(G85R), and SOD1(G93A) specifically impaired ERAD ((ER)-associated degradation) function but not cytosolic protein degradation in motor neurons.
Formal Description
Interaction-ID: 363

gene/protein mutant

SOD1-p.G93A

decreases_activity of

process

ER-associated ubiquitin-dependent protein catabolic process

in motor neurons
Comment SOD1mut (SOD1(A4V), SOD1(G85R), and SOD1(G93A) proteins specifically interacted with the ER membrane protein Derlin-1.
Formal Description
Interaction-ID: 366

gene/protein mutant

SOD1-p.G93A

interacts (colocalizes) with

gene/protein

DERL1

in motor neurons
Comment SOD1mut SOD1(A4V), SOD1(G85R), and SOD1(G93A) induces motor neuron death.
Formal Description
Interaction-ID: 398

gene/protein mutant

SOD1-p.G93A

increases_activity of

phenotype

motor neuron degeneration

in motor neurons