General Information:

Id: 143
Diseases: Amyotrophic lateral sclerosis
Mus musculus
BTO:0004488 NSC-34 cell; BTO:0000312 motoneuron
article
Reference: Suzuki H et al.(2009) ALS-linked P56S-VAPB, an aggregated loss-of-function mutant of VAPB, predisposes motor neurons to ER stress-related death by inducing aggregation of co-expressed wild-type VAPB J. Neurochem. 108: 973-985 [PMID: 19183264]

Interaction Information:

Comment Wt-VAPB, but not P56S-VAPB, induces the activation of the unfolded protein response (UPR) IRE1/XBP1 pathway.
Formal Description
Interaction-ID: 513

gene/protein mutant

VAPB-p.P56S

NOT affects_activity of

Comment The expression of P56S-VAPB increases sensitivity of NSC34 motor neuronal cells to ER stress-induced death.
Formal Description
Interaction-ID: 519

gene/protein mutant

VAPB-p.P56S

increases_activity of

process

neuron death

Comment The expression of P56S-VAPB caused aggregation of co-expressed wt-VAPB.
Formal Description
Interaction-ID: 521

gene/protein mutant

VAPB-p.P56S

increases_quantity of

cellular component

inclusion body

Comment The interaction between wt-VAPB and P56S-VAPB took place more tightly than that between wt-VAPB and wt-VAPB.
Formal Description
Interaction-ID: 522

gene/protein mutant

VAPB-p.P56S

interacts (colocalizes) with

gene/protein

VAPB

Comment Wt-VAPB, but not P56S-VAPB, induces the activation of the unfolded protein response (UPR) IRE1/XBP1 pathway.
Formal Description
Interaction-ID: 732

gene/protein

VAPB

increases_activity of