Results 131 to 140 of about 57,365 (244)

Novel α-galactosidase A gene mutation in a Chinese Fabry disease family: A case report. [PDF]

World J Clin Cases, 2022
Fu AY, Jin QZ, Sun YX.
europepmc   +1 more source

Cryo‐EM structure of GH43 β‐Xylosidase from Enterobacter cloacae provides insights into substrate specificity and the role of an auxiliary domain in enzymatic activity

The FEBS Journal, EarlyView.
Xylan, the second most abundant plant cell wall polysaccharide, is degraded by β‐xylanases and β‐xylosidases. Here, we present the 2.65 Å cryo‐EM structure of Enterobacter cloacae β‐xylosidase (EcXyl43, GH43) and the 2.4 Å X‐ray structure of its inactive F507A mutant.
Lorenzo Briganti, Andre Schutzer Godoy, Caio Cesar Mello Capetti, Rafaela Sachetto Fernandes, Vanessa O. A. Pellegrini, Ornella M. Ontañon, Eleonora Campos, Rodrigo V. Portugal, Igor Polikarpov   +8 more
wiley   +1 more source

Reduced α-galactosidase A activity in zebrafish (Danio rerio) mirrors distinct features of Fabry nephropathy phenotype. [PDF]

Mol Genet Metab Rep, 2022
Elsaid HOA, Furriol J, Blomqvist M, Diswall M, Leh S, Gharbi N, Anonsen JH, Babickova J, Tøndel C, Svarstad E, Marti HP, Krause M.   +11 more
europepmc   +1 more source

Molecular dynamics simulations elucidate the misfolding mechanisms of secretion‐defective pancreatic lipase variants

The FEBS Journal, EarlyView.
Misfolding mutations in pancreatic lipase have been identified as potential contributors of chronic pancreatitis, an inflammatory disease of the human pancreas. Here, we describe the effect of these misfolding mutations on pancreatic lipase structure using molecular dynamics simulations and structural modeling.
Gyula Hoffka, András Szabó
wiley   +1 more source

Purification, Characterization, and Immobilization of a Novel Protease-Resistant α-Galactosidase from Oudemansiella radicata and Its Application in Degradation of Raffinose Family Oligosaccharides from Soymilk. [PDF]

Foods, 2022
Geng X, Lei J, Bau T, Guo D, Chang M, Feng C, Xu L, Cheng Y, Zuo N, Meng J.   +9 more
europepmc   +1 more source

Structural insights into xyloglucan recognition by an ABC transporter from a Gram‐positive, thermophilic bacterium

The FEBS Journal, EarlyView.
Athe_2052 is an ABC transporter substrate‐binding protein that recognizes xyloglucan heptasaccharide (XXXG), a soluble breakdown product of plant xyloglucan. The crystal structure of Athe_2052, together with binding assays and computational modeling, shows how XXXG is recognized by this sugar transporter.
Hansen Tjo, Virginia Jiang, Philip D. Jeffrey, Angela Zhu, A. James Link, Jerelle A. Joseph, Jonathan M. Conway   +6 more
wiley   +1 more source

Serum Agalactosyl IgG Predicts Hepatocellular Carcinoma and All‐Cause Mortality After SVR in Advanced Chronic Hepatitis C

Hepatology Research, EarlyView.
ABSTRACT Background and Aims Patients with chronic hepatitis C and advanced fibrosis remain at substantial risk of hepatocellular carcinoma (HCC) and non‐liver–related death even after sustained virological response (SVR) to direct‐acting antivirals (DAA).
Daisuke Sakon, Jumpei Kondo, Yuki Tahata, Hayato Hikita, Maki Iwaisako, Muya Matsumoto, Asuka Ogata, Shinji Takamatsu, Yasutoshi Nozaki, Naruyasu Kakita, Hisashi Ishida, Fumihiko Nakanishi, Yuichi Yoshida, Masanori Nakahara, Kazuho Imanaka, Mitsuru Sakakibara, Takayuki Yakushijin, Tetsuo Takehara, Takahiro Kodama, Eiji Miyoshi   +19 more
wiley   +1 more source

α-Galactosidase A Augmentation by Non-Viral Gene Therapy: Evaluation in Fabry Disease Mice. [PDF]

Pharmaceutics, 2021
Rodríguez-Castejón J, Alarcia-Lacalle A, Gómez-Aguado I, Vicente-Pascual M, Solinís Aspiazu MÁ, Del Pozo-Rodríguez A, Rodríguez-Gascón A.   +6 more
europepmc   +1 more source

Bakuchiol: From structural mimicry to systems‐level skin biology

International Journal of Cosmetic Science, EarlyView.
Convergent outcomes, divergent mechanisms: bakuchiol vs. retinol. Bakuchiol exhibits functional convergence with retinol in improving extracellular matrix integrity, epidermal differentiation and barrier function, while operating through distinct mechanisms.
Ratan K. Chaudhuri
wiley   +1 more source

α-Galactosidase a Deficiency in Fabry Disease Leads to Extensive Dysregulated Cellular Signaling Pathways in Human Podocytes. [PDF]

Int J Mol Sci, 2021
Jehn U, Bayraktar S, Pollmann S, Van Marck V, Weide T, Pavenstädt H, Brand E, Lenders M.   +7 more
europepmc   +1 more source