Results 81 to 90 of about 211,407 (207)

Cell-to-cell transmission of p53 aggregates: a novel player in oncology?

Molecular & Cellular Oncology, 2021
The mutants of the tumor suppressor protein p53 form protein aggregates. It has been proposed that these aggregates propagate like prions, albeit the detailed mechanism of the propagation is unclear.
Naoyuki Iwahashi, Midori Ikezaki, Hiroyuki Saito, Kenji Uchimura, Kazuchika Nishitsuji   +4 more
doaj   +1 more source

Increased Disarray of Extracellular Matrix Collagen‐I Fiber Network and Compromised Biomechanics in Aortae From Marfan‐Syndrome Mice Assessed Through Combined Opto‐Biomechatronics

Advanced Science, EarlyView.
Combined structure‐function assessment in aortic rings from Marfan mice using MechaMorph Opto‐Biomechatronics technology relates increased elasticity (stiffness) and dynamic viscosity and less ordered extracellular matrix 3D‐structure (collagen) as potential causes for impaired Windkessel function and compromised haemodynamics in Marfan's syndrome ...
Dominik Schneidereit, Anika Nagel, Vanessa I. T. Zwaans, Laureen Schurr, Jonas Ahr, Michael Haug, Anna Piluso, Alexandra Sporkova, Matthias Karck, Andreas H. Wagner, Oliver Friedrich   +10 more
wiley   +1 more source

CRISPR and Gene Augmentation Rescue Trabecular Meshwork Dysfunction in iPSC Models of Lowe Syndrome

Advanced Science, EarlyView.
By modeling Lowe syndrome using patient‐derived iPSCs, this study establishes a human disease model that faithfully recapitulates OCRL deficiency‐associated ciliary and cytoskeletal defects. The model enables evaluation of both mutation‐agnostic DNA augmentation and CRISPR‐mediated mutation correction strategies, both of which restore OCRL function and
Siyu Chen, Zhiquan Liu, Wenmin Wang, Qing Wang, Tia J. Kowal, Fan Zhang, Grzegorz Walkiewicz, Faruk Hossen, Herbert M. Lachman, Jinqiong Zhou, Yiting Wang, Yang Sun   +11 more
wiley   +1 more source

Characterization of two heparan sulphate-binding sites in the mycobacterial adhesin Hlp

BMC Microbiology, 2008
Background The histone-like Hlp protein is emerging as a key component in mycobacterial pathogenesis, being involved in the initial events of host colonization by interacting with laminin and glycosaminoglycans (GAGs).
Previato Jose O, Mendonça-Previato Lucia, Ottenhoff Tom HM, Mohana-Borges Ronaldo, Silva Carlos AM, de Lima Cristiana S, Todeschini Adriane R, Portugal Michelle I, Pessolani Maria CV   +8 more
doaj   +1 more source

Control of cell division in the adult brain by heparan sulfates in fractones and vascular basement membranes [PDF]

, 2010
Regulation of cell division in adult tissues and organs requires the coordination of growth factors at the surface of potentially-dividing cells in specific anatomic loci named germinal niches.
Frederic Mercier, Vanessa Douet
core  

Heparan sulfate-dependent ERK activation contributes to the overexpression of fibrotic proteins and enhanced contraction by scleroderma fibroblasts

, 2008
Objective. To investigate the contribution of heparan sulfate proteoglycan and Ras/MEK/ERK to the overexpression of profibrotic proteins and the enhanced contractile ability of dermal fibroblasts from patients with systemic sclerosis (SSc; scleroderma ...
Wilcox-Adelman, S, Black, C M, Wilcox-Adelman, S., Khan, K., Chen, Y L, Pitsillides, A A, Goetinck, P, Sarraf, C., Denton, C P, Leask, A., Khan, K, Pala, D, Xu, S W, Pitsillides, A.A., Carter, D E, Denton, C.P., Goetinck, P.F., Chen, Y., Carter, D.E., Abraham, D.J., Abraham, D J, Liu, S., Shiwen, X., Liu, S X, Pala, D., Leask, A, Eastwood, M, Eastwood, M., Black, C.M., Sarraf, C E   +29 more
core   +1 more source

An amino-terminal extension is required for the secretion of chick agrin and its binding to extracellular matrix. [PDF]

, 1995
Agrin is an extracellular matrix (ECM) protein with a calculated relative molecular mass of more than 200 kD that induces the aggregation of acetylcholine receptors (AChRs) at the neuromuscular junction. This activity has been mapped to its COOH terminus.
Ruegg, M. A., A J Denzer, B Schumacher, Denzer, A J, M A Ruegg, Gesemann, M., Schumacher, B, M Gesemann, Schumacher, B., Gesemann, M, Ruegg, M A, Denzer, A. J.   +11 more
core   +1 more source

A Novel NR3C1 Frameshift Variant Associated With Familial Glucocorticoid Resistance Syndrome: An Integrated Analysis of Steroid Profiling and Structural Modeling

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Glucocorticoid resistance syndrome (GRS) is a rare hereditary disorder caused by pathogenic variants in NR3C1, characterized by marked phenotypic heterogeneity and frequent misdiagnosis as primary aldosteronism or subclinical Cushing's syndrome.
Sufang Yun, Zhifeng Zhu, Fengye Zhou, Ying Liu, Chunfang Liang, Haitao Ju, Lin Lu, Zhaoli Yan   +7 more
wiley   +1 more source

Review of the Molecular and Developmental Basis of Myhre Syndrome, Bench Research

American Journal of Medical Genetics Part C: Seminars in Medical Genetics, EarlyView.
ABSTRACT Myhre syndrome (MS) is a connective‐tissue disorder within the acromelic dysplasia spectrum. It is characterized by congenital craniofacial, skeletal, cutaneous anomalies, respiratory, cardiovascular along with intellectual disability, deafness, and progressive fibrosis.
Camille Viaut, Valerie Cormier‐Daire
wiley   +1 more source

Classification and Molecular Functions of Heparan Sulfate Proteoglycans and Their Molecular Mechanisms with the Receptor

Biologics
Heparan sulfate proteoglycans are highly glycosylated proteins in which heparan sulfate, a glycosaminoglycan sugar chain, is an acidic sugar chain consisting of a repeating disaccharide structure of glucuronic acid and N-acetylglucosamine is locally ...
Yasunari Matsuzaka, Ryu Yashiro
doaj   +1 more source