Physicochemical properties of cells and their effects on intrinsically disordered proteins (IDPs)

FX Theillet, A Binolfi, T Frembgen-Kesner… - Chemical …, 2014 - ACS Publications
It has long been axiomatic that a protein's structure determines its function. Intrinsically
disordered proteins (IDPs) and disordered protein regions (IDRs) defy this structure …

Mechanisms and consequences of macromolecular phase separation

LP Bergeron-Sandoval, N Safaee, SW Michnick - Cell, 2016 - cell.com
Over a century ago, colloidal phase separation of matter into non-membranous bodies was
recognized as a fundamental organizing principal of cell" protoplasm." Recent insights into …

Proteins containing expanded polyglutamine tracts and neurodegenerative disease

A Adegbuyiro, F Sedighi, AW Pilkington IV… - Biochemistry, 2017 - ACS Publications
Several hereditary neurological and neuromuscular diseases are caused by an abnormal
expansion of trinucleotide repeats. To date, there have been 10 of these trinucleotide repeat …

Slow amyloid nucleation via α-helix-rich oligomeric intermediates in short polyglutamine-containing huntingtin fragments

M Jayaraman, R Kodali, B Sahoo, AK Thakur… - Journal of molecular …, 2012 - Elsevier
The 17-amino-acid N-terminal segment (httNT) that leads into the polyglutamine (polyQ)
segment in the Huntington's disease protein huntingtin (htt) dramatically increases …

Physical chemistry of polyglutamine: intriguing tales of a monotonous sequence

R Wetzel - Journal of molecular biology, 2012 - Elsevier
Polyglutamine (polyQ) sequences of unknown normal function are present in a significant
number of proteins, and their repeat expansion is associated with a number of genetic …

Nanoparticle-guided brain drug delivery: Expanding the therapeutic approach to neurodegenerative diseases

C Riccardi, F Napolitano, D Montesarchio, S Sampaolo… - Pharmaceutics, 2021 - mdpi.com
Neurodegenerative diseases (NDs) represent a heterogeneous group of aging-related
disorders featured by progressive impairment of motor and/or cognitive functions, often …

Huntingtin protein interactions altered by polyglutamine expansion as determined by quantitative proteomic analysis

T Ratovitski, E Chighladze, N Arbez, T Boronina… - Cell Cycle, 2012 - Taylor & Francis
Huntington disease (HD) is a neurodegenerative disorder caused by an expansion of a
polyglutamine repeat within the HD gene product, huntingtin. Huntingtin, a large (347 kDa) …

Molecular interaction between the chaperone Hsc70 and the N-terminal flank of huntingtin exon 1 modulates aggregation

E Monsellier, V Redeker, G Ruiz-Arlandis… - Journal of Biological …, 2015 - ASBMB
The aggregation of polyglutamine (polyQ)-containing proteins is at the origin of nine
neurodegenerative diseases. Molecular chaperones prevent the aggregation of polyQ …

Unraveling the Puzzle of Therapeutic Peptides: A Promising Frontier in Huntington's Disease Treatment

S Ahamad, N Bano, S Khan, MK Hussain… - Journal of Medicinal …, 2024 - ACS Publications
Huntington's disease (HD) is a neurodegenerative genetic disorder characterized by a
mutation in the huntingtin (HTT) gene, resulting in the production of a mutant huntingtin …

Polyglutamine amyloid core boundaries and flanking domain dynamics in huntingtin fragment fibrils determined by solid-state nuclear magnetic resonance

CL Hoop, HK Lin, K Kar, Z Hou, MA Poirier… - Biochemistry, 2014 - ACS Publications
In Huntington's disease, expansion of a polyglutamine (polyQ) domain in the huntingtin (htt)
protein leads to misfolding and aggregation. There is much interest in the molecular features …