NRBP1-containing CRL2/CRL4A regulates amyloid β production by targeting BRI2 and BRI3 for degradation

T Yasukawa, A Tsutsui, C Tomomori-Sato, S Sato… - Cell reports, 2020 - cell.com
Alzheimer's disease (AD) is a progressive neurodegenerative disease caused by
accumulations of Aβ peptides. Production and fibrillation of Aβ are downregulated by BRI2 …

[HTML][HTML] Pyroglutamate formation at the N-termini of ABri molecules in familial British dementia is not restricted to the central nervous system

Y Tomidokoro, A Tamaoka, JL Holton… - Hirosaki igaku …, 2010 - ncbi.nlm.nih.gov
Amyloid molecules harboring pyroglutamate (pGlu) residue at the N-termini are considered
to be important for the development of cerebral amyloidosis such as Alzheimer's disease …

An Insertion Within SIRPβ1 Shows a Dual Effect Over Alzheimer's Disease Cognitive Decline Altering the Microglial Response

JM García-Alberca, I de Rojas… - Journal of …, 2024 - content.iospress.com
Background: Microglial dysfunction plays a causative role in Alzheimer's disease (AD)
pathogenesis. Here we focus on a germline insertion/deletion variant mapping SIRPß1, a …

Amyloid precursor protein-induced axonopathies are independent of amyloid-β peptides

GB Stokin, A Almenar-Queralt… - Human molecular …, 2008 - academic.oup.com
Overexpression of amyloid precursor protein (APP), as well as mutations in the APP and
presenilin genes, causes rare forms of Alzheimer's disease (AD). These genetic changes …

Structure and neurotoxicity of novel amyloids derived from the BRI gene

G Gibson, OMA El-Agnaf, Z Anwar… - Biochemical Society …, 2005 - portlandpress.com
A number of human neurodegenerative diseases involve aggregated amyloid proteins in the
brain, eg Alzheimer's disease (β-amyloid) and Parkinson's disease (α-synuclein). Other …

Amyloidogenesis in familial British dementia is associated with a genetic defect on chromosome 13

J Ghiso, R Vidal, A Rostagno… - Annals of the New …, 2000 - Wiley Online Library
Familial British dementia (FBD) is a disorder characterized by the presence of amyloid
deposits in cerebral blood vessels and brain parenchyma coexisting with neurofibrillary …

Bri2 BRICHOS client specificity and chaperone activity are governed by assembly state

G Chen, A Abelein, HE Nilsson, A Leppert… - Nature …, 2017 - nature.com
Protein misfolding and aggregation is increasingly being recognized as a cause of disease.
In Alzheimer's disease the amyloid-β peptide (Aβ) misfolds into neurotoxic oligomers and …

BRI2 interacts with BACE1 and regulates its cellular levels by promoting its degradation and reducing its mRNA levels

M Tsachaki, A Fotinopoulou, N Slavi… - Current Alzheimer …, 2013 - ingentaconnect.com
BRI2, a protein mutated in Familial British and Familial Danish Dementias, interacts with
Amyloid Precursor Protein (APP) and reduces the levels of secreted APPβ (sAPPβ), which …

Furin mediates enhanced production of fibrillogenic ABri peptides in familial British dementia

SH Kim, R Wang, DJ Gordon, J Bass, DF Steiner… - Nature …, 1999 - nature.com
The genetic lesion underlying familial British dementia (FBD), an autosomal dominant
neurodegenerative disorder, is a T–A transversion at the termination codon of the BRI gene …

Non-fibrillar oligomeric species of the amyloid ABri peptide, implicated in familial British dementia, are more potent at inducing apoptotic cell death than protofibrils or …

OMA El-Agnaf, S Nagala, BP Patel… - Journal of molecular …, 2001 - Elsevier
Familial British dementia (FBD) is an autosomal dominant neurodegenerative disorder, with
biochemical and pathological similarities to Alzheimer's disease. FBD is associated with a …