CNS Neuroscience &Therapeutics, Volume 30, Issue 10, October 2024.The current study is the first to utilize tandem mass tag (TMT)‐labeled liquid chromatography‐mass spectrometry (LC–MS/MS)‐based proteomics in dysferlinopathy research. The study aimed to explore the proteomic landscape and disease‐related alterations in the muscle tissue proteome of 15 patients with dysferlinopathy with varying degrees of dystrophic ...
Di Wang +13 more
wiley +1 more source
Journal of Inherited Metabolic Disease, Volume 47, Issue 5, Page 1001-1017, September 2024.Abstract Dietary lipid manipulation has recently been proposed for managing glycogen storage disease (GSD) type IIIa. This study aimed to evaluate the myopathic, cardiac, and metabolic status, physical activity, growth, and dietary compliance of a personalized diet high in protein and fat for 24 months.
Sema Kalkan Uçar +9 more
wiley +1 more source
Genomic Reconstruction of an Uncultured Hydrothermal Vent Gammaproteobacterial Methanotroph (Family Methylothermaceae) Indicates Multiple Adaptations to Oxygen Limitation [PDF]
, 2015 Hydrothermal vents are an important contributor to marine biogeochemistry, producing large volumes of reduced fluids, gasses, and metals and housing unique, productive microbial and animal communities fueled by chemosynthesis.
Chan, Ken Y. +8 more
core +2 more sources
Bottom‐Up Synthesized Glucan Materials: Opportunities from Applied Biocatalysis
Advanced Materials, Volume 36, Issue 27, July 4, 2024.Linear d‐glucans, simple yet versatile polysaccharides, shape nanostructures crucial for biomaterial development. Shifting from top‐down to bottom‐up synthesis using natural and engineered enzymes provides meticulous control over polymer structures, fostering diversification for macromolecular materials.
Chao Zhong, Bernd Nidetzky
wiley +1 more source
Characterization of function of the GlgA2 glycogen/starch synthase in Cyanobacterium sp. Clg1 highlights convergent evolution of glycogen metabolism into starch granule aggregation [PDF]
, 2017 At variance with the starch-accumulating plants and most of the glycogen-accumulating cyanobacteria, Cyanobacterium sp. CLg1 synthesizes both glycogen and starch. We now report the selection of a starchless mutant of this cyanobacterium that retains wild-
Arias, M.C. +14 more
core +1 more source
Thermoconditional modulation of the pleiotropic sensitivity phenotype by the Saccharomyces cerevisiae PRP19 mutant allele pso4-1 [PDF]
, 2002 The conditionally-lethal pso4-1 mutant allele of the spliceosomal-associated PRP19 gene allowed us to study this gene’s influence on pre-mRNA processing, DNA repair and sporulation.
Bonatto, Diego +7 more
core
Investigation of Starch Binding Domains for Improvement of Starch degradation [PDF]
, 2009 Dansk resume Stivelse er planternes primære energilager og et vigtigt næringsmiddel for pattedyr,svampe og bakterier. Stivelse deponeres i højt organiserede semi-krystallinske stivelseskorn i plastider: kloroplaster i blade (transitorisk stivelse) og ...
Blennow, Anders +3 more
core
UEG Week 2025 DDM Summit Presentations
United European Gastroenterology Journal, Volume 13, Issue S8, Page S1477-S1538, October 2025.
wiley +1 more source
Effects of available dietary carbohydrate and preslaughter treatment on glycolytic potential, protein degradation, and quality traits of pig muscles [PDF]
, 2006 The current study was conducted to determine the interactive effects of a glycogen-reducing diet fed to finishing pigs and length of preslaughter transportion on muscle metabolic traits, proteolysis of intermediate filament and costameric proteins, and ...
Bee, G. +7 more
core +2 more sources
When <i>pull</i>ulanase needs a little push: MyoAAV capsids enhance gene therapy for GSD IIIa. [PDF]
Mol Ther Methods Clin DevVenigalla S, Pacak CA.
europepmc +1 more source