General Information:

Id: 101
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: 337

gene/protein mutant

SOD1-p.A4V

affects_activity of

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: 339

gene/protein mutant

SOD1-p.A4V

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: 340

gene/protein mutant

SOD1-p.G85R

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: 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: 342

gene/protein mutant

SOD1-p.G85R

affects_activity of

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: 343

gene/protein mutant

SOD1-p.G93A

affects_activity of

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: 344

gene/protein mutant

SOD1-p.A4V

increases_activity of

gene/protein

EIF2AK3

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: 345

gene/protein mutant

SOD1-p.G85R

increases_activity of

gene/protein

EIF2AK3

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: 351

gene/protein mutant

SOD1-p.A4V

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 CHOP.
Formal Description
Interaction-ID: 352

gene/protein mutant

SOD1-p.G85R

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 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: 354

gene/protein mutant

SOD1-p.A4V

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) induced the expression of the ER stress marker protein BIP.
Formal Description
Interaction-ID: 355

gene/protein mutant

SOD1-p.G85R

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) 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: 361

gene/protein mutant

SOD1-p.A4V

decreases_activity of

in motor neurons
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: 362

gene/protein mutant

SOD1-p.G85R

decreases_activity of

in motor neurons
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

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: 364

gene/protein mutant

SOD1-p.A4V

interacts (colocalizes) with

gene/protein

DERL1

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: 365

gene/protein mutant

SOD1-p.G85R

interacts (colocalizes) with

gene/protein

DERL1

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 Expression of SOD1mut SOD1(A4V), SOD1(G85R), and SOD1(G93A), but not SOD1wt, activated endogenous ASK1.
Formal Description
Interaction-ID: 383

gene/protein mutant

SOD1-p.A4V

increases_activity of

gene/protein

MAP3K5

in motor neurons
Comment Expression of SOD1mut SOD1(A4V), SOD1(G85R), and SOD1(G93A), but not SOD1wt, activated endogenous ASK1.
Formal Description
Interaction-ID: 384

gene/protein mutant

SOD1-p.G85R

increases_activity of

gene/protein

MAP3K5

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

in motor neurons
Comment SOD1mut SOD1(A4V), SOD1(G85R), and SOD1(G93A) induces motor neuron death.
Formal Description
Interaction-ID: 4218

gene/protein mutant

SOD1-p.A4V

increases_activity of

in motor neurons
Comment SOD1mut SOD1(A4V), SOD1(G85R), and SOD1(G93A) induces motor neuron death.
Formal Description
Interaction-ID: 4219

gene/protein mutant

SOD1-p.G85R

increases_activity of

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: 13234

gene/protein

DERL1

is localized in

cellular component

endoplasmic reticulum membrane

in motor neurons