A5089649845- Muscle Physiology and Disorders
- Hemoglobin structure and function
- Calpain Protease Function and Regulation
- Cell Adhesion Molecules Research
- Adipose Tissue and Metabolism
- Adrenal Hormones and Disorders
- Protease and Inhibitor Mechanisms
- Erythrocyte Function and Pathophysiology
- Ubiquitin and proteasome pathways
- Hormonal and reproductive studies
- Hormonal Regulation and Hypertension
- Exercise and Physiological Responses
- Virus-based gene therapy research
- Protein Structure and Dynamics
- Estrogen and related hormone effects
- Signaling Pathways in Disease
- Eicosanoids and Hypertension Pharmacology
- Biotin and Related Studies
- Telomeres, Telomerase, and Senescence
- Silk-based biomaterials and applications
- Genetic Neurodegenerative Diseases
- RNA Research and Splicing
- Metabolism and Genetic Disorders
- Steroid Chemistry and Biochemistry
- Porphyrin Metabolism and Disorders
University of Bristol
2016-2025
Istituto di Scienze e Tecnologie Chimiche "Giulio Natta"
2020-2025
National Research Council
2023-2024
At Bristol
2023
Università Cattolica del Sacro Cuore
2008-2019
Institute of Chemistry of Molecular Recognition
2009-2019
Washington Center
2018
University of Washington
2018
Catholic University of America
2005-2014
Institute of Molecular Biology and Pathology
2008
alpha-Dystroglycan has been isolated from chicken cardiac muscle and its molecular weight was estimated to be approximately 135 kDa. The avian protein interacts with murine Engelbreth-Holm-Swarm (EHS) tumor laminin via interaction the C-terminal LG4 LG5 domains (fragment E3) of alpha-chain. This binding is calcium-dependent can competed by heparin. Electron microscopy investigation on shape alpha-dystroglycan suggests that core consists two roughly globular connected a segment which most...
Agrin is a basement membrane-associated proteoglycan that induces the formation of postsynaptic specializations at neuromuscular junction. This activity modulated by alternative splicing and thought to be mediated receptors expressed in muscle fibers. An isoform agrin does not induce binds with high affinity dystroglycan, component dystrophin-glycoprotein complex. Transcripts encoding this are variety non-muscle tissues. Here, we analyzed tissue distribution dystroglycan on protein level...
The structural factors governing azide and cyanide binding have been examined by measuring the effects of 46 mutations at key topological positions in distal pocket sperm whale, pig, human myoglobin. Replacement His64 (E7) with smaller amino acids results dramatic increases association rate constant for primarily due to relief steric hindrance imposed imidazole side chain. Gln64 (native) metmyoglobins abnormally low constants dissociation (0.1-0.3 s-1) direct hydrogen bonding between N...
A missense amino acid mutation of valine to aspartic in 567 position alpha-dystroglycan (DG), identified dag1-mutated zebrafish, results a reduced transcription and complete absence the protein. Lacking experimental structural data for zebrafish DG domains, detailed mechanism observed mutation-induced destabilization complex membrane damage, remained unclear. With aim contribute better clarification structure-function relationships featuring complex, three-dimensional models wild-type mutant...
Dystroglycan is a receptor responsible for crucial interactions between extracellular matrix and cytoplasmic space. We provide the first evidence that dystroglycan truncated. In HC11 normal murine 184B5 non‐tumorigenic mammary human cell lines, expected β‐dystroglycan 43 kDa band was found but breast T47D, BT549, MCF7, colon HT29, HCT116, SW620, prostate DU145 cervical HeLa cancer cells expressed an anomalous ≈31 band. α‐Dystroglycan udetectable in most of lines which as species. An also...
Abstract Altered aquaporin‐4 (AQP4) expression has been reported in brain edema, tumors, muscular dystrophy, and neuromyelitis optica. However, the plasma membrane organization of AQP4 its interaction with proteins such as dystrophin‐associated protein complex are not well understood. In this study, we used sucrose density gradient ultracentrifugation 2D blue native/sodium dodecyl sulfate–polyacrylamide gel electrophoresis showed several multi‐subunit complexes (pools) different sizes,...
Mitochondrial ATP synthases form rows of dimers, which induce membrane curvature to give cristae their characteristic lamellar or tubular morphology. The angle formed between the central stalks synthase dimers varies species. Using cryo-electron tomography and sub-tomogram averaging, we determined structure dimer from nematode worm C. elegans show that differs previously structures. consequences this species-specific difference at interface were investigated by comparing S. cerevisiae...
Introduction Dystroglycan (DG) is an adhesion complex comprising two subunits, α-DG and β-DG, which interact non-covalently at the plasma membrane. As a component of dystrophin-glycoprotein DGC, DG plays crucial role in linking cytoskeleton to surrounding basement membranes. Rare primary point mutations DAG1 gene have been identified patients with various forms neuromuscular dystrophy, ranging phenotype from mild severe. Methods To gain deeper understanding molecular mechanisms underlying...
Basement membrane (BM) extracellular matrices are crucial for the coordination of different tissue layers. A matrix adhesion receptor that is important BM function and stability in many mammalian tissues dystroglycan (DG) complex. This comprises non-covalently-associated α-DG, interacts with laminin BM, transmembrane β-DG, principally dystrophin to connect actin cytoskeleton. Mutations dystrophin, DG, or several enzymes glycosylate α-DG underlie severe forms human muscular dystrophy....
Muscle weakness and muscle loss characterize many physio-pathological conditions, including sarcopenia forms of muscular dystrophy, which are often also associated with mitochondrial dysfunction. Verbascoside, a phenylethanoid glycoside plant origin, named acteoside, has shown strong antioxidant anti-fatigue activity in different animal models, but the molecular mechanisms underlying these effects not completely understood. This study aimed to investigate influence verbascoside on function...
The structure of the N‐terminal region mouse α‐dystroglycan (DGN) was investigated by expression two protein fragments (residues 30–180 and 30–438) in Escherichia coli cells. Trypsin susceptibility experiments show presence a stable (approximately from residue 30 to 315). In addition, guanidinium hydrochloride (Gdn/HCl) denaturation DGN‐(30–438)‐peptide, monitored means tryptophan fluorescence, produces cooperative transition typical folded structures. These results strongly suggest that is...
Dystroglycan (DG) is a cell surface receptor consisting of two subunits: α-dystroglycan, extracellular and highly glycosylated, β-dystroglycan, spanning the membrane. It pivotal member dystrophin-glycoprotein complex involved in wide variety important cellular processes such as stabilization muscle fiber sarcolemma or clustering acetylcholine receptors. We report 2.3-Å resolution crystal structure murine skeletal N-terminal α-DG region, which confirms presence autonomous domains; first...
ABSTRACT α-Dystroglycan (α-DG) was identified as a common receptor for lymphocytic choriomeningitis virus (LCMV) and several other arenaviruses including the human pathogenic Lassa fever virus. Initial work postulated that interactions between arenavirus glycoproteins α-DG are based on protein-protein interactions. We found, however, susceptibility toward LCMV infection differed in various cell lines despite them expressing comparable levels of DG, suggesting posttranslational modifications...