General Information:

Id: 196
Diseases: Amyotrophic lateral sclerosis
Mus musculus
SOD1 wt or SOD1G93A transgenic mouse, transfected with human wt SOD1 or human SOD1G93A
BTO:0001279 spinal cord; BTO:0000312 motoneuron
article
Reference: Kieran D et al.(2007) Deletion of the BH3-only protein puma protects motoneurons from ER stress-induced apoptosis and delays motoneuron loss in ALS mice. Proc. Natl. Acad. Sci. U.S.A. 104 [PMID: 18077368]

Interaction Information:

Comment SOD1(G93A) mice show motoneuron degeneration in the lumbar spinal cord, histologically detectable from 60 days on.
Formal Description
Interaction-ID: 780

gene/protein mutant

SOD1-p.G93A

increases_activity of

phenotype

motor neuron degeneration

Comment SOD1(G93A) mice showed evidence for increased ER stress and defects in protein degradation in motoneurons during disease progression.
Formal Description
Interaction-ID: 784

gene/protein mutant

SOD1-p.G93A

increases_activity of

process

response to endoplasmic reticulum stress

Comment The expression of the ER-resident chaperones Grp78 and Grp94 in motoneurons of SOD1G93A mice increased as early as 50 days compared with WT littermates.
Formal Description
Interaction-ID: 786

gene/protein mutant

SOD1-p.G93A

increases_quantity of

gene/protein

HSPA5

Drugbank entries Show/Hide entries for HSPA5
Comment The expression of the ER-resident chaperones Grp78 and Grp94 in motoneurons of SOD1G93A mice increased as early as 50 days compared with WT littermates.
Formal Description
Interaction-ID: 788

gene/protein mutant

SOD1-p.G93A

increases_quantity of

gene/protein

HSP90B1

Drugbank entries Show/Hide entries for HSP90B1
Comment SOD1(G93A) mice showed an increase in ubiquitin in motoneurons during the early stage of disease progression.
Formal Description
Interaction-ID: 789

gene/protein mutant

SOD1-p.G93A

increases_quantity of

gene/protein

Ubiquitin

Comment SOD1(G93A) mice showed increased mRNA and protein expression of CHOP, Bim and Puma.
Formal Description
Interaction-ID: 793

gene/protein mutant

SOD1-p.G93A

increases_expression of

gene/protein

DDIT3

Comment SOD1(G93A) mice showed increased mRNA and protein expression of CHOP, Bim and Puma.
Formal Description
Interaction-ID: 794

gene/protein mutant

SOD1-p.G93A

increases_expression of

gene/protein

BCL2L11

Comment SOD1(G93A) mice showed increased mRNA and protein expression of CHOP, Bim and Puma.
Formal Description
Interaction-ID: 795

gene/protein mutant

SOD1-p.G93A

increases_expression of

gene/protein

BBC3

Comment SOD1(G93A) mice showed increased mRNA and protein expression of CHOP, Bim and Puma.
Formal Description
Interaction-ID: 13274

gene/protein mutant

SOD1-p.G93A

increases_quantity of

gene/protein

DDIT3

Comment SOD1(G93A) mice showed increased mRNA and protein expression of CHOP, Bim and Puma.
Formal Description
Interaction-ID: 13275

gene/protein mutant

SOD1-p.G93A

increases_quantity of

gene/protein

BCL2L11

Comment SOD1(G93A) mice showed increased mRNA and protein expression of CHOP, Bim and Puma.
Formal Description
Interaction-ID: 13276

gene/protein mutant

SOD1-p.G93A

increases_quantity of

gene/protein

BBC3