Results 51 to 60 of about 21,037 (206)

Polyalanine Expansion in PABPN1 Alters the Structure and Dynamics of Its Nuclear Aggregates in Differentiated Muscle Cells

The FASEB Journal, Volume 39, Issue 12, 30 June 2025.
The accumulation of aggregation‐prone proteins is a hallmark of aging cells. Pathogenic forms of these aggregates are associated with neurodegenerative, neuromuscular, and muscular diseases, yet the differences between pathogenic and non‐pathogenic aggregates remain unclear.
Sander D. Mallon, Erik Bos, Vahid Sheikhhassani, Milad Shademan, Lenard M. Voortman, Alireza Mashaghi, Thomas H. Sharp, Vered Raz   +7 more
wiley   +1 more source

Protein Misfolding and Aggregation as a Therapeutic Target for Polyglutamine Diseases

Brain Science, 2017
The polyglutamine (polyQ) diseases, such as Huntington’s disease and several types of spinocerebellar ataxias, are a group of inherited neurodegenerative diseases that are caused by an abnormal expansion of the polyQ tract in disease-causative proteins ...
T. Takeuchi, Y. Nagai
semanticscholar   +1 more source

SEC‐SAXS/MC Ensemble Structural Studies of the Microtubule Binding Protein Cdt1 Show Monomeric, Folded‐Over Conformations

Cytoskeleton, Volume 82, Issue 6, Page 372-387, June 2025.
ABSTRACT Cdt1 is a mixed folded protein critical for DNA replication licensing and it also has a “moonlighting” role at the kinetochore via direct binding to microtubules and the Ndc80 complex. However, it is unknown how the structure and conformations of Cdt1 could allow it to participate in these multiple, unique sets of protein complexes.
Kyle P. Smith, Srinivas Chakravarthy, Amit Rahi, Manas Chakraborty, Kristen M. Vosberg, Marco Tonelli, Maximilian G. Plach, Arabela A. Grigorescu, Joseph E. Curtis, Dileep Varma   +9 more
wiley   +1 more source

Emerging Links Between Ferroptosis and Neurodegeneration: Implications for Disease Mechanisms and Nutraceutical Interventions

Food Science &Nutrition, Volume 13, Issue 6, June 2025.
Ferroptosis, a controlled cell death, is driven by iron and characterized by reactive oxygen species and lipid peroxidation. The impact of lipid peroxides, GSH/GPX4, and iron metabolism–targeting small molecule inhibitors is discussed. Nucleic acids, proteins, and phytochemicals are implicated as inhibitors of ferroptosis.
Anwar Ali, Quratulain Babar, Ayesha Saeed, Domenico Sergi, Nenad Naumovski, Isam A. Mohamed Ahmed, Ghalia Shamlan, Halah Abdulrahman Hafiz, Muhammad Faisal Manzoor, Joanna Trafialek, Felix Kwashie Madilo   +10 more
wiley   +1 more source

A small-molecule Nrf1 and Nrf2 activator mitigates polyglutamine toxicity in spinal and bulbar muscular atrophy.

Human Molecular Genetics, 2016
Spinal and bulbar muscular atrophy (SBMA, also known as Kennedy's disease) is one of nine neurodegenerative disorders that are caused by expansion of polyglutamine-encoding CAG repeats. Intracellular accumulation of abnormal proteins in these diseases, a
Laura C. Bott, Nisha M. Badders, Ke‐lian Chen, G. Harmison, E. Bautista, C. Shih, M. Katsuno, G. Sobue, J. Taylor, N. Dantuma, K. Fischbeck, C. Rinaldi   +11 more
semanticscholar   +1 more source

Inhibition of tau aggregation by the CCT3 and CCT7 apical domains

Protein Science, Volume 34, Issue 6, June 2025.
Abstract The eukaryotic chaperonin containing t‐complex polypeptide 1 (CCT/TRiC) is a molecular chaperone that assists protein folding in an ATP‐driven manner. It consists of two stacked identical rings that are each made up of eight distinct subunits. Here, we show that the apical domains of subunits CCT3 and CCT7 from humans are strong inhibitors of ...
Miki Ben‐Maimon, Nadav Elad, Segev Naveh‐Tassa, Yaakov Levy, Amnon Horovitz   +4 more
wiley   +1 more source

Huntingtin’s spherical solenoid structure enables polyglutamine tract-dependent modulation of its structure and function

eLife, 2016
The polyglutamine expansion in huntingtin protein causes Huntington’s disease. Here, we investigated structural and biochemical properties of huntingtin and the effect of the polyglutamine expansion using various biophysical experiments including ...
R. Vijayvargia, R. Epand, A. Leitner, T. Jung, Baehyun Shin, Roy Jung, Alejandro Lloret, Randy Singh Atwal, Hyeongseok Lee, Jong-Min Lee, R. Aebersold, H. Hebert, Ji-Joon Song, I. Seong   +13 more
semanticscholar   +1 more source

Polyglutamine inclusion body toxicity [PDF]

Movement Disorders, 2017
Peer Reviewed ; https://deepblue.lib.umich.edu/bitstream/2027.42/141741/1/mds27226_am.pdf ; https://deepblue.lib.umich.edu/bitstream/2027.42/141741/2/mds27226 ...
openaire   +4 more sources

The most prevalent genetic cause of ALS-FTD, C9orf72 synergizes the toxicity of ATXN2 intermediate polyglutamine repeats through the autophagy pathway

Autophagy, 2016
The most common genetic cause for amyotrophic lateral sclerosis and frontotemporal dementia (ALS-FTD) is repeat expansion of a hexanucleotide sequence (GGGGCC) within the C9orf72 genomic sequence.
S. Ciura, C. Sellier, María-Letizia Campanari, N. Charlet-Berguerand, E. Kabashi   +4 more
semanticscholar   +1 more source

Evening complex component ELF3 interacts with LUX proteins to repress soybean root nodulation

Plant Biotechnology Journal, Volume 23, Issue 6, Page 2194-2206, June 2025.
Summary Formation of root nodules is a unique hallmark of the symbiotic interaction between legume host plants and rhizobia and is governed by a complex regulatory framework that balances the appropriate orchestration of rhizobial infection and subsequent nodule organogenesis. In contrast to prominent model species such as Medicago truncatula and Lotus
Bohong Su, Hong Li, Ke Zhang, Haiyang Li, Caiyun Fan, Meiling Zhong, Hui Zou, Rujie Li, Liyu Chen, Jing Bo Jin, Mingkun Huang, Baohui Liu, Fanjiang Kong, Zhihui Sun   +13 more
wiley   +1 more source