Multifunctional roles of gelsolin in health and diseases

GH Li, PD Arora, Y Chen… - Medicinal research …, 2012 - Wiley Online Library
Abstract Gelsolin, a Ca2+‐regulated actin filament severing, capping, and nucleating
protein, is an ubiquitous, multifunctional regulator of cell structure and metabolism. More …

Gelsolin amyloidosis: genetics, biochemistry, pathology and possible strategies for therapeutic intervention

JP Solomon, LJ Page, WE Balch… - Critical reviews in …, 2012 - Taylor & Francis
Protein misassembly into aggregate structures, including cross-β-sheet amyloid fibrils, is
linked to diseases characterized by the degeneration of post-mitotic tissue. While amyloid …

Uncovering the universality of self-replication in protein aggregation and its link to disease

G Meisl, CK Xu, JD Taylor, TCT Michaels, A Levin… - Science …, 2022 - science.org
Fibrillar protein aggregates are a hallmark of a range of human disorders, from prion
diseases to dementias, but are also encountered in several functional contexts. Yet, the …

Crystal-to-crystal synthesis of triazole-linked pseudo-proteins via topochemical azide–alkyne cycloaddition reaction

BP Krishnan, R Rai, A Asokan… - Journal of the American …, 2016 - ACS Publications
Isosteric replacement of amide bond (s) of peptides with surrogate groups is an important
strategy for the synthesis of peptidomimetics (pseudo-peptides). Triazole is a well …

Renal amyloidosis associated with a novel sequence variant of gelsolin

S Sethi, JD Theis, P Quint, W Maierhofer… - American journal of …, 2013 - Elsevier
We present a case of a 75-year-old woman who presented with progressive kidney failure.
Kidney biopsy performed to determine the cause of kidney failure showed amyloidosis of …

Chaperone nanobodies protect gelsolin against MT1-MMP degradation and alleviate amyloid burden in the gelsolin amyloidosis mouse model

W Van Overbeke, A Verhelle, I Everaert… - Molecular Therapy, 2014 - cell.com
Gelsolin amyloidosis is an autosomal dominant incurable disease caused by a point
mutation in the GSN gene (G654A/T), specifically affecting secreted plasma gelsolin …

Heparin binds 8 kDa gelsolin cross-β-sheet oligomers and accelerates amyloidogenesis by hastening fibril extension

JP Solomon, S Bourgault, ET Powers, JW Kelly - Biochemistry, 2011 - ACS Publications
Glycosaminoglycans (GAGs) are highly sulfated linear polysaccharides prevalent in the
extracellular matrix, and they associate with virtually all amyloid deposits in vivo. GAGs …

Molecular and clinical insights into protein misfolding and associated amyloidosis

M Pande, R Srivastava - European Journal of Medicinal Chemistry, 2019 - Elsevier
The misfolding of normally soluble proteins causes their aggregation and deposition in the
tissues which disrupts the normal structure and function of the corresponding organs. The …

[HTML][HTML] Nanobody interaction unveils structure, dynamics and proteotoxicity of the Finnish-type amyloidogenic gelsolin variant

T Giorgino, D Mattioni, A Hassan, M Milani… - … et Biophysica Acta (BBA …, 2019 - Elsevier
AGel amyloidosis, formerly known as familial amyloidosis of the Finnish-type, is caused by
pathological aggregation of proteolytic fragments of plasma gelsolin. So far, four mutations …

[HTML][HTML] Clinical, biopsy, and mass spectrometry findings of renal gelsolin amyloidosis

S Sethi, S Dasari, MS Amin, JA Vrana, JD Theis… - Kidney international, 2017 - Elsevier
Gelsolin amyloidosis is a rare type of amyloidosis typically involving the cranial and
peripheral nerves, but rarely the kidney. Here we report the clinical, kidney biopsy, and mass …