Results 31 to 40 of about 13,344 (266)

Altered cytoskeletal arrangement in induced pluripotent stem cells and motor neurons from patients with riboflavin transporter deficiency

Disease Models & Mechanisms, 2021
The cytoskeletal network plays a crucial role in the differentiation, morphogenesis, function and homeostasis of the nervous tissue, so that alterations in any of its components may lead to neurodegenerative diseases.
Alessia Niceforo, Chiara Marioli, Fiorella Colasuonno, Stefania Petrini, Keith Massey, Marco Tartaglia, Enrico Bertini, Sandra Moreno, Claudia Compagnucci   +8 more
doaj   +1 more source

Study Protocol: Randomised controlled trial to investigate the functional significance of marginal riboflavin status in young women in the UK (RIBOFEM)

BMC Public Health, 2009
Background The functional significance of moderate riboflavin deficiency as it is currently assessed is not well understood. Animal and human studies have suggested a role for riboflavin in the absorption and mobilisation of iron and as such may be ...
Williams Elizabeth A, Mushtaq Sohail, Hill Marilyn HE, Dainty Jack R, Powers Hilary J   +4 more
doaj   +1 more source

Riboflavin transporter deficiency: <i>AAV9-SLC52A2</i> gene therapy as a new therapeutic strategy. [PDF]

Front Cell Neurosci
Mei C, Magliocca V, Chen X, Massey K, Gonzalez-Cordero A, Gray SJ, Tartaglia M, Bertini ES, Corti S, Compagnucci C.   +9 more
europepmc   +2 more sources

Bioinspired Design of a Wet‐Adhesive Cornea Glue Based on Recombinant Human Protein Networks

Advanced Functional Materials, EarlyView.
Natures protein‐based high performance materials e.g. elastin, silk and muscle proteins have been mimicked by a new protein‐hybrid material based on redesigned human partial sequences only, showing high wet‐adhesiveness and elasticity for biomedical applications.
Anna Resch, Matthias C. Huber, Alexander Resch, Andreas Schreiber, Thabo Lapp, Jan Dittrich, Frederic Eisen, Austin Julian Michalec, Viktoria Planz, Adrianna Kolberg, Mathaeus Tschaikowsky, Daniel Böhringer, Maike Windbergs, Thomas Reinhard, Bernd Rolauffs, Bizan N. Balzer, Günther Schlunck, Stefan M. Schiller   +17 more
wiley   +1 more source

New Insights into the Neurodegeneration Mechanisms Underlying Riboflavin Transporter Deficiency (RTD): Involvement of Energy Dysmetabolism and Cytoskeletal Derangement

Biomedicines, 2022
Riboflavin transporter deficiency (RTD) is a rare genetic disorder characterized by motor, sensory and cranial neuropathy. This childhood-onset neurodegenerative disease is caused by biallelic pathogenic variants in either SLC52A2 or SLC52A3 genes ...
Fiorella Colasuonno, Chiara Marioli, Marco Tartaglia, Enrico Bertini, Claudia Compagnucci, Sandra Moreno   +5 more
doaj   +1 more source

3D Printing of Conducting Polymer Hydrogels for Electrostimulation‐Assisted Tissue Engineering

Advanced Materials, EarlyView.
This review highlights nanosized inherently conducting polymers (ICPs) as promising ink constituents for 3D printing of conducting hydrogel (CH)‐based electrostimulation (ES) devices. ICP‐based ink formulation, 3D printing, and solidification strategies are discussed, along with the application of 3D‐printed ICP‐based CHs as ES platforms for regulating
Chien Minh Tran, Zhilian Yue, Chunyan Qin, Kusuma B. C. Imani, Mirella Dottori, Robert J. Forster, Gordon G. Wallace   +6 more
wiley   +1 more source

Demand-oriented riboflavin supply of organic broiler using a feed material from fermentation of Ashbya gossypii

Animal, 2021
Alternatives to riboflavin (vitamin B2) production by recombinant microorganisms are needed in organic poultry production, but are cost-intensive, so that a demand-oriented riboflavin supply is necessary. Details on the riboflavin requirements of organic
C. Lambertz, J. Leopold, S. Ammer, F. Leiber, B. Thesing, C. Wild, K. Damme   +6 more
doaj  

Flavin adenine dinucleotide rescues the phenotype of frataxin deficiency. [PDF]

PLoS ONE, 2010
BACKGROUND: Friedreich ataxia is a neurodegenerative disease caused by the lack of frataxin, a mitochondrial protein. We previously demonstrated that frataxin interacts with complex II subunits of the electronic transport chain (ETC) and putative ...
Pilar Gonzalez-Cabo, Sheila Ros, Francesc Palau   +2 more
doaj   +1 more source

Miniaturized Devices for On‐the‐Spot Generation of Small‐Diameter Vascular Grafts

Advanced Materials Technologies, EarlyView.
An intracorporeal extrusion device is developed for on‐the‐spot generation of vascular grafts with tunable diameters spanning from 1 to 6 mm. The device uses biomimetic polymers and light, to promptly obtaining biocompatible and high‐burst pressure‐resistant grafts.
Deyanira Hernandez‐Sanchez, Maxime‐Emmanuel Comtois‐Bona, Antony El‐Khoury, Sergio David Garcia Schejtman, Aidan Macadam, Juan‐David Figueroa‐Alegria, Jesus M. Calderon‐Torres, Irene Guzmán‐Soto, Marc Ruel, Erik J. Suuronen, Marcelo Muñoz, Emilio I. Alarcon   +11 more
wiley   +1 more source

Riboflavin transporter deficiency in young adults unmasked by dietary changes

JIMD Reports
Riboflavin transporter deficiency (RTD) is a genetic disorder of reduced riboflavin (vitamin B2) uptake that causes progressive, multifocal neurological dysfunction.
Bregje Jaeger, Mirjam Langeveld, Robert Brunkhorst, Felix Distelmaier, Ana Pop, Nicole I. Wolf, Annet M. Bosch   +6 more
doaj   +1 more source