PQBP1: The key to intellectual disability, neurodegenerative diseases, and innate immunity

H Tanaka, H Okazawa - International Journal of Molecular Sciences, 2022 - mdpi.com
The idea that a common pathology underlies various neurodegenerative diseases and
dementias has attracted considerable attention in the basic and medical sciences …

PQBP1, an intrinsically disordered/denatured protein at the crossroad of intellectual disability and neurodegenerative diseases

H Okazawa - Neurochemistry international, 2018 - Elsevier
Abstract PQBP1 (polyglutamine binding protein-1) is the earliest identified molecule among
the group of disease-related intrinsically disordered/denatured proteins. PQBP1 interacts …

PQBP‐1 is expressed predominantly in the central nervous system during development

Y Qi, M Hoshino, Y Wada, S Marubuchi… - European Journal of …, 2005 - Wiley Online Library
Mutations of PQBP‐1 (polyglutamine binding protein‐1) have been shown recently to cause
human mental retardation accompanied by microcephaly at a high frequency. As a first step …

Phenotypic and molecular insights into PQBP1‐related intellectual disability

GMH Abdel‐Salam, N Miyake… - American Journal of …, 2018 - Wiley Online Library
We report two discordant clinical and imaging features in four male patients from two
unrelated families of Egyptian descent with hemizygous pathogenic variants in PQBP1. The …

PQBP-1 transgenic mice show a late-onset motor neuron disease-like phenotype

T Okuda, H Hattori, S Takeuchi, J Shimizu… - Human molecular …, 2003 - academic.oup.com
A body of experimental evidence indicates that transcription and/or mRNA processing
factors interacting with the polyglutamine disease gene products play crucial roles in the …

[HTML][HTML] Expression of human PQBP-1 in Drosophila impairs long-term memory and induces abnormal courtship

N Yoshimura, D Horiuchi, M Shibata, M Saitoe, M Qi… - FEBS letters, 2006 - Elsevier
Frame shift mutations of the polyglutamine binding protein-1 (PQBP1) gene lead to total or
partial truncation of the C-terminal domain (CTD) and cause mental retardation in human …

PQBP-1, a novel polyglutamine tract-binding protein, inhibits transcription activation by Brn-2 and affects cell survival

M Waragai, CH Lammers, S Takeuchi… - Human molecular …, 1999 - academic.oup.com
A novel gene, designated PQBP-1, which encodes a 265 residue protein that binds to the
polyglutamine tract of the brain-specific transcription factor Brn-2, was identified. PQBP-1 …

PQBP-1 (Np/PQ): a polyglutamine tract-binding and nuclear inclusion-forming protein

H Okazawa, M Sudol, T Rich - Brain research bulletin, 2001 - Elsevier
Polyglutamine (Q) tract binding protein-1 (PQBP-1) was isolated on the basis of its
interaction with polyglutamine tracts and localizes predominantly to the nucleus where it …

PQBP-1/Npw38, a nuclear protein binding to the polyglutamine tract, interacts with U5-15kD/dim1p via the carboxyl-terminal domain

M Waragai, E Junn, M Kajikawa, S Takeuchi… - Biochemical and …, 2000 - Elsevier
PQBP-1 was identified as a binding protein to the polyglutamine tract present in various
transcription-related factors and causative genes for neurodegenerative disorders. This …

[HTML][HTML] Inhibition of protein misfolding/aggregation using polyglutamine binding peptide QBP1 as a therapy for the polyglutamine diseases

HA Popiel, T Takeuchi, JR Burke, WJ Strittmatter… - …, 2013 - Elsevier
Protein misfolding and aggregation in the brain have been recognized to be crucial in the
pathogenesis of various neurodegenerative diseases, including Alzheimer's, Parkinson's …