McArdle disease does not affect skeletal muscle fibre type profiles in humans
Patients suffering from glycogen storage disease V (McArdle disease) were shown to have higher surface electrical activity in their skeletal muscles when exercising at the same intensity as their healthy counterparts, indicating more muscle fibre ...
Tertius Abraham Kohn+9 more
doaj +1 more source
Novel Approaches in Glycogen Storage Disease Type I Management: Harnessing the Potential of Micronutrients and Macro Molecules [PDF]
Santosh B. Patil, Pramod C. Gadad
openalex +1 more source
The Expanding Clinical and Genetic Spectrum of Muscle Glycogen Storage Disease 0, (GSD0B)
ABSTRACT Glycogen storage disorders are a group of genetic disorders affecting glucose homeostasis in the body. Muscular glycogen stores are essential for liberating glucose for energy supply during bursts of activity and sustained muscle work. Muscle glycogen storage disease 0 (GSD0B) is associated with biallelic variants in GYS1 causing muscular ...
Sarah Donoghue+16 more
wiley +1 more source
X-linked Liver Glycogenosis in a Taiwanese Family: Transmission From Undiagnosed Males
X-linked liver glycogenosis (XLG), also known as glycogen storage disease type-IXa, is characterized by hepatomegaly, abnormal liver functions and growth retardation. It is caused by mutations in the PHKA2 gene that encodes the α-subunit of phosphorylase
Szu-Ta Chen+6 more
doaj +1 more source
Strategies for optimization of hypoglycemia rat models
This review focuses on rat models for studying the short‐term and long‐term effects of mild and severe hypoglycemia. We explored the physiological mechanisms to understand the consequences of hypoglycemia in rat experimental models. This study sheds light on uncovering the therapeutic potential for hypoglycemic patients and its efficacy in mitigating ...
Lee Yeong Zher+4 more
wiley +1 more source
Nanostructured Biomaterial‐Based Approaches to Support Induced Pluripotent Stem Cell Differentiation
Induced pluripotent stem cells (iPSCs), reprogrammed from adult somatic cells, represent an innovative approach for regenerative medicine and biomedical applications. This review highlights the importance of nanostructured biomaterials, utilized as delivery systems or scaffolds, in supporting iPSC differentiation and fate through structural and ...
Beatriz A. B. R. Passos+2 more
wiley +1 more source
ApuA, a multifunctional x-glucan-degrading enzyme of Streptococcus suis, mediates adhesion to porcine epithelium and mucus [PDF]
We have identified apuA in Streptococcus suis, which encodes a bifunctional amylopullulanase with conserved -amylase and pullulanase substrate-binding domains and catalytic motifs.
Ferrando, M.L.+4 more
core +2 more sources
The transition period in dairy cows, spanning 3 weeks before and after calving, is a critical phase characterized by increased nutrient demands, reduced dry matter intake (DMI), and elevated risk of metabolic disorders such as negative nutrient balance (NNB), lipolysis, proteolysis, and oxidative stress.
Mohammed S. Seleem+5 more
wiley +1 more source
Hepatic stress associated with pathologies characterized by disturbed glucose production
The liver is an organ with many facets, including a role in energy production and metabolic balance, detoxification and extraordinary capacity of regeneration. Hepatic glucose production plays a crucial role in the maintenance of normal glucose levels in
Monika Gjorgjieva+2 more
doaj +1 more source
Is acetylation a metabolic rheostat that regulates skeletal muscle insulin action? [PDF]
Skeletal muscle insulin resistance, which increases the risk for developing various metabolic diseases, including type 2 diabetes, is a common metabolic disorder in obesity and aging.
LaBarge, Samuel+2 more
core +2 more sources