China
Africa
Explore chapters and articles related to this topic
Stimulus-Secretion Coupling: Intracellular Proteins and Nucleotides
Published in Stephen W. Carmichael, Susan L. Stoddard, The Adrenal Medulla 1986 - 1988, 2017
Stephen W. Carmichael, Susan L. Stoddard
Bader, Trifaró, Langley et al. (1986) identified a gelsolin-like protein in adrenal chromaffin cells obtained from cow and pig. Actin- and calcium-sensitive actin-binding proteins were purified on affinity columns using DNase I as ligand. A 91 kDa actin-binding protein was eluted and shown to be reactive against an antibody to gelsolin. A 93 kDa protein, found to be similar to brevin, was shown to be a component of blood but not chromaffin cells. An 85 kDa protein also was found but it apparently is not related to gelsolin. They suggested that calcium may activate the 91 kDa protein, and this may be important for the movement of chromaffin vesicles to the plasma membrane.
Mechanisms of Fibril Formation and Cellular Response
Published in Martha Skinner, John L. Berk, Lawreen H. Connors, David C. Seldin, XIth International Symposium on Amyloidosis, 2007
Martha Skinner, John L. Berk, Lawreen H. Connors, David C. Seldin
Hereditary gelsolin amyloidosis (AGel amyloidosis), also known as Meretoja Syndrome, was originally reported in Finland by Meretoja (1). Later, several kindred’s were found in other European countries, United States, Japan, and recently an Iranian case was characterized (2-4). Principal clinical signs are corneal lattice dystrophy, cranial neuropathy, and cutis laxa, but also other signs such as peripheral and autonomic neuropathy, cardiac, and renal involvement can occur (2). Gelsolin is a calcium- and polyphosphoinositide-regulated principal actin-modulating protein encoded by a locus on chromosome 9 at q32-q34 (5). Circulating gelsolin takes part in the clearance of actin filaments. AGel amyloidosis is caused by a guanine to adenine or very rarely, thymidine transversion at nucleotide 654 of the gelsolin gene (G654A or G654T gelsolin mutation) resulting in, respectively, asparagine or tyrosine for aspartic acid substitution at codon 187 corresponding to position 15 of mutant gelsolin (2). The mutant gelsolin is unable to bind calcium, which renders it susceptible to furin cleavage (6). In the evolution of clinical disease, not only amyloid deposition but also even impaired gelsolin function may contribute (7). All reported kindreds have been found to carry the variant G654A gelsolin, except the Danish and Czech who show a G654T gelsolin mutation (8). This study presents the first Iranian family with amyloidosis due to a G654A gelsolin mutation.
LC-MS/MS: A sensitive and selective analytical technique to detect COVID-19 protein biomarkers in the early disease stage
Published in Expert Review of Proteomics, 2023
Siva Nageswara Rao Gajula, Ankita Sahebrao Khairnar, Pallavi Jock, Nikita Kumari, Kendre Pratima, Vijay Munjal, Pavan Kalan, Rajesh Sonti
Gelsolin, an actin-binding protein, is a potent inflammatory regulator and plays a vital role in the body’s innate immunity. As the most widely expressed member of the actin capping and severing family of proteins exists in two isoforms: isoform 1 (a secretory protein) and isoform 2 (a cytosolic protein) [156] and provides information on disease severity, treatment efficiency, and clinical outcomes [157]. It is present in the cytoplasmic matrix and mitochondria, and calcium ions regulate its activity [158,159]. The prominent role of gelsolin is to scavenge actin that is released during cell injury. In case of severe injury, the level of gelsolin in the body depletes, resulting in a harsh inflammatory response and further leading to multiple organ damage [160]. The lower level of plasma gelsolin is a biomarker of prognosis in different pathological conditions such as diabetes, various cancers, and infection. Furthermore, gelsolin is associated with impaired lung function, and therefore determining its levels is vital in COVID-19 positive patients with diabetes [157,161]. The amount of gelsolin rapidly decreases as the severity of COVID-19 infection increases [160]. Gelsolin can be detected by Nano Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS). The peptide fraction was analyzed on a quadrupole time-of-flight system and validated further using ELISA (enzyme-linked immunosorbent assay) [162].
Bilateral facial palsy
Published in Acta Oto-Laryngologica, 2019
Junyang Jung, Dong Choon Park, Su Young Jung, Myung Jin Park, Sang Hoon Kim, Seung Geun Yeo
Studies have shown that the etiology of facial palsy can include trauma, infectious, systemic, autoimmune, idiopathic, toxic, iatrogenic, metabolic, genetic, congenital, and other causes. Causes differ between children and adults (Table 2) [12–14] and differ by region, research institution, researchers, and research subjects. In South Africa, Lyme disease, sarcoidosis, and sclerosteosis were the most frequent causes of bilateral facial palsy [15]. Patients with bilateral facial paralysis who required plastic surgery for bilateral facial neuropathy; or who had lattice dystrophy of the cornea, a positive family history and/or a G654A mutation in the gelsolin gene were found to have gelsolin amyloidosis [16]. In children, the incidence of bilateral Bell's palsy was low; causes included acute otitis media, Lyme disease, recurrent central nervous system leukemia, and acute disseminated encephalomyelitis. More exact history taking, physical examination, and directed laboratory studies are needed to diagnose the incidence and causes of bilateral facial paralysis [11].
Amyloid in parenchymal organs in gelsolin (AGel) amyloidosis
Published in Amyloid, 2019
Eeva-Kaisa Schmidt, Sari Kiuru-Enari, Sari Atula, Maarit Tanskanen
Gelsolin is a calcium-activated, actin-modulating protein that has a role in several biological processes such as cell motility, apoptosis, ion-channel regulation and carcinogenesis, and is present both in cytosolic and secretary forms in most cells [8–10]. The symptoms of AGel amyloidosis are suggested to be caused by the deposition of gelsolin-based amyloid (AGel) fibrils and pre-amyloid oligomers that originate in the misfolded gelsolin fragments and accumulate in various tissues [4]. The most common clinical findings in AGel amyloidosis are progressive ophthalmologic, neurological and dermatological signs, manifesting in patients close to their forties [4,11]. The penetrance of the 640 G>A gene variant is 100%, but for as yet unknown reasons the manifestations of the disease and the severity of symptoms may vary significantly between patients [4].