[HTML][HTML] Molecular pathophysiological mechanisms in Huntington's disease

A Jurcau - Biomedicines, 2022 - mdpi.com
Huntington's disease is an inherited neurodegenerative disease described 150 years ago
by George Huntington. The genetic defect was identified in 1993 to be an expanded CAG …

Proteasome in action: substrate degradation by the 26S proteasome

I Sahu, MH Glickman - Biochemical Society Transactions, 2021 - portlandpress.com
Ubiquitination is the major criteria for the recognition of a substrate-protein by the 26S
proteasome. Additionally, a disordered segment on the substrate—either intrinsic or induced …

Lynch syndrome, molecular mechanisms and variant classification

AB Abildgaard, SV Nielsen, I Bernstein, A Stein… - British Journal of …, 2023 - nature.com
Patients with the heritable cancer disease, Lynch syndrome, carry germline variants in the
MLH1, MSH2, MSH6 and PMS2 genes, encoding the central components of the DNA …

[HTML][HTML] Molecular determinants of the crosstalk between endosomal microautophagy and chaperone-mediated autophagy

GJ Krause, P Kirchner, B Stiller, K Morozova, A Diaz… - Cell reports, 2023 - cell.com
Chaperone-mediated autophagy (CMA) and endosomal microautophagy (eMI) are
pathways for selective degradation of cytosolic proteins in lysosomes and late endosomes …

PRKN-linked familial Parkinson's disease: cellular and molecular mechanisms of disease-linked variants

L Clausen, J Okarmus, V Voutsinos, M Meyer… - Cellular and Molecular …, 2024 - Springer
Parkinson's disease (PD) is a common and incurable neurodegenerative disorder that
arises from the loss of dopaminergic neurons in the substantia nigra and is mainly …

Folding of heterologous proteins in bacterial cell factories: Cellular mechanisms and engineering strategies

Y Rong, SI Jensen, K Lindorff-Larsen… - Biotechnology Advances, 2023 - Elsevier
The expression of correctly folded and functional heterologous proteins is important in many
biotechnological production processes, whether it is enzymes, biopharmaceuticals or …

[HTML][HTML] Paralogue-specific roles of SUMO1 and SUMO2/3 in protein quality control and associated diseases

W Wang, MJ Matunis - Cells, 2023 - mdpi.com
Small ubiquitin-related modifiers (SUMOs) function as post-translational protein
modifications and regulate nearly every aspect of cellular function. While a single ubiquitin …

Conserved degronome features governing quality control associated proteolysis

B Mashahreh, S Armony, KE Johansson… - Nature …, 2022 - nature.com
The eukaryotic proteome undergoes constant surveillance by quality control systems that
either sequester, refold, or eliminate aberrant proteins by ubiquitin-dependent mechanisms …

HSP70-binding motifs function as protein quality control degrons

AB Abildgaard, V Voutsinos, SD Petersen… - Cellular and Molecular …, 2023 - Springer
Protein quality control (PQC) degrons are short protein segments that target misfolded
proteins for proteasomal degradation, and thus protect cells against the accumulation of …

The Cys sense: thiol redox switches mediate life cycles of cellular proteins

M Radzinski, T Oppenheim, N Metanis, D Reichmann - Biomolecules, 2021 - mdpi.com
Protein homeostasis is an essential component of proper cellular function; however,
sustaining protein health is a challenging task, especially during the aerobic lifestyle …