Systemic light chain amyloidosis
V Sanchorawala - New England Journal of Medicine, 2024 - Mass Medical Soc
Key Points Systemic Light Chain Amyloidosis Immunoglobulin light chain (AL) amyloidosis
is a rare disease that occurs when a plasma cell disorder produces monoclonal light chains …
is a rare disease that occurs when a plasma cell disorder produces monoclonal light chains …
[HTML][HTML] The Hsp70 chaperone network
R Rosenzweig, NB Nillegoda, MP Mayer… - … reviews molecular cell …, 2019 - nature.com
The 70-kDa heat shock proteins (Hsp70s) are ubiquitous molecular chaperones that act in a
large variety of cellular protein folding and remodelling processes. They function virtually at …
large variety of cellular protein folding and remodelling processes. They function virtually at …
Small soluble α-synuclein aggregates are the toxic species in Parkinson's disease
Soluble α-synuclein aggregates varying in size, structure, and morphology have been
closely linked to neuronal death in Parkinson's disease. However, the heterogeneity of …
closely linked to neuronal death in Parkinson's disease. However, the heterogeneity of …
Skin aging, cellular senescence and natural polyphenols
E Csekes, L Račková - International journal of molecular sciences, 2021 - mdpi.com
The skin, being the barrier organ of the body, is constitutively exposed to various stimuli
impacting its morphology and function. Senescent cells have been found to accumulate with …
impacting its morphology and function. Senescent cells have been found to accumulate with …
[HTML][HTML] The proteostasis network and its decline in ageing
Ageing is a major risk factor for the development of many diseases, prominently including
neurodegenerative disorders such as Alzheimer disease and Parkinson disease. A hallmark …
neurodegenerative disorders such as Alzheimer disease and Parkinson disease. A hallmark …
Systemic immunoglobulin light chain amyloidosis
G Merlini, A Dispenzieri, V Sanchorawala… - Nature reviews Disease …, 2018 - nature.com
Systemic immunoglobulin light chain amyloidosis is a protein misfolding disease caused by
the conversion of immunoglobulin light chains from their soluble functional states into highly …
the conversion of immunoglobulin light chains from their soluble functional states into highly …
The nucleolus functions as a phase-separated protein quality control compartment
INTRODUCTION Cells have evolved quality control mechanisms that operate under normal
growth conditions and during stress to maintain protein homeostasis (proteostasis) and …
growth conditions and during stress to maintain protein homeostasis (proteostasis) and …
Diverse molecular mechanisms underlying pathogenic protein mutations: beyond the loss-of-function paradigm
L Backwell, JA Marsh - Annual review of genomics and human …, 2022 - annualreviews.org
Most known disease-causing mutations occur in protein-coding regions of DNA. While some
of these involve a loss of protein function (eg, through premature stop codons or missense …
of these involve a loss of protein function (eg, through premature stop codons or missense …
Pathways of cellular proteostasis in aging and disease
CL Klaips, GG Jayaraj, FU Hartl - Journal of Cell Biology, 2018 - rupress.org
Ensuring cellular protein homeostasis, or proteostasis, requires precise control of protein
synthesis, folding, conformational maintenance, and degradation. A complex and adaptive …
synthesis, folding, conformational maintenance, and degradation. A complex and adaptive …
In vivo aspects of protein folding and quality control
BACKGROUND Proteins are synthesized on ribosomes as linear chains of amino acids and
must fold into unique three-dimensional structures to fulfill their biological functions. Protein …
must fold into unique three-dimensional structures to fulfill their biological functions. Protein …