Islet-brain1/C-Jun N-terminal kinase interacting protein-1 (IB1/JIP-1) promoter variant is associated with Alzheimer's disease

N Helbecque, A Abderrhamani, L Meylan… - Molecular …, 2003 - nature.com
Abstract Islet-brain1 (IB1) or c-Jun NH2 terminal kinase interacting protein-1 (JIP-1), the
product of the MAPK8IP1 gene, functions as a neuronal scaffold protein to allow signalling …

Effect of the disulfide bridge and the C-terminal extension on the oligomerization of the amyloid peptide ABri implicated in familial British dementia

OMA El-Agnaf, JM Sheridan, C Sidera, G Siligardi… - Biochemistry, 2001 - ACS Publications
Familial British dementia (FBD) is a rare neurodegenerative disorder and shares features
with Alzheimer's disease, including amyloid plaque deposits, neurofibrillary tangles …

Inhibition of the ISR abrogates mGluR5-dependent long-term depression and spatial memory deficits in a rat model of Alzheimer's disease

Z Hu, P Yu, Y Zhang, Y Yang, M Zhu, S Qin… - Translational …, 2022 - nature.com
Soluble amyloid-β-protein (Aβ) oligomers, a major hallmark of AD, trigger the integrated
stress response (ISR) via multiple pathologies including neuronal hyperactivation …

[HTML][HTML] A PIR-fect storm

L Welberg - Nature Reviews Neuroscience, 2013 - nature.com
A PIR-fect storm | Nature Reviews Neuroscience Skip to main content Thank you for visiting
nature.com. You are using a browser version with limited support for CSS. To obtain the best …

TDP-43 pathology may occur in the BRI2 gene-related dementias

T Lashley, JL Holton, T Revesz - Acta neuropathologica, 2011 - Springer
The ubiquitously expressed TAR DNA-binding protein-43 (TDP-43), involved in transcription
and alternative splicing, is a major disease protein in frontotemporal lobar degeneration and …

A decamer duplication in the 3′ region of the BRI gene originates an amyloid peptide that is associated with dementia in a Danish kindred

R Vidal, T Révész, A Rostagno, E Kim… - Proceedings of the …, 2000 - National Acad Sciences
Familial Danish dementia (FDD), also known as heredopathia ophthalmo-oto-encephalica,
is an autosomal dominant disorder characterized by cataracts, deafness, progressive ataxia …

[HTML][HTML] Recombinant BRICHOS chaperone domains delivered to mouse brain parenchyma by focused ultrasound and microbubbles are internalized by hippocampal …

L Galan-Acosta, C Sierra, A Leppert… - Molecular and Cellular …, 2020 - Elsevier
The BRICHOS domain is found in human precursor proteins associated with cancer,
dementia (Bri2) and amyloid lung disease (proSP-C). Recombinant human (rh) proSP-C …

Properties of neurotoxic peptides related to the Bri gene

O El-Agnaf, G Gibson, M Lee, A Wright… - Protein and Peptide …, 2004 - ingentaconnect.com
Familial British dementia, a rare autosomal dominant neurodegenerative disorder, shares
features with Alzheimer's disease, including amyloid plaque deposits, neurofibrillary tangles …

The chaperone domain BRICHOS prevents CNS toxicity of amyloid-β peptide in Drosophila melanogaster

E Hermansson, S Schultz, D Crowther… - Disease models & …, 2014 - journals.biologists.com
Aggregation of the amyloid-β peptide (Aβ) into toxic oligomers and amyloid fibrils is linked to
the development of Alzheimer's disease (AD). Mutations of the BRICHOS chaperone domain …

[引用][C] Familial British and Danish dementias

J Ghiso, A Rostagno, Y Tomidokoro… - … Proteins: The Beta …, 2005 - Wiley Online Library
This chapter contains sections titled: Introduction FBD and FDD A Novel Gene BRI2 BRI2
Mutations Generate Two New Amyloid Subunits, ABri and ADan Biochemical Properties of …