Results 51 to 60 of about 42,767 (197)

Clinical efficacy of Myozyme on one ventilator dependent patient with late-onset glycogen storage disease typeⅡ

open access: yesChinese Journal of Contemporary Neurology and Neurosurgery, 2014
Objective To evaluate the effect of Myozyme on one ventilator dependent patient with late-onset glycogen storage disease typeⅡ (GSDⅡ).  Methods Myozyme infusion was administered based on manufacturer's recommendations at 20 mg/kg every 2 to 4 weeks ...
Juan YANG   +10 more
doaj  

Huge aneurysm of the ascending aorta in a patient with adult-type Pompe's disease: histological findings mimicking fibrillinopathy [PDF]

open access: yes, 2017
Adult-type Pompe's disease (glycogen storage disease type II) has rarely been shown to present with dilatative arteriopathy, suggesting potential smooth muscle involvement in addition to lysosomal glycogen deposits usually restricted to skeletal muscle ...
Banz, Yara   +3 more
core  

Programming Skeletal Muscle Metabolic Flexibility in Offspring of Male Rats in Response to Maternal Consumption of Slow Digesting Carbohydrates during Pregnancy [PDF]

open access: yes, 2020
Skeletal muscle plays a relevant role in metabolic flexibility and fuel usage and the associated muscle metabolic inflexibility due to high-fat diets contributing to obesity and type 2 diabetes.
Andújar, Eloísa   +7 more
core   +1 more source

Hepatotoxicity of Nonesterified Fatty Acids to Dairy Cows: Pathophysiological Mechanisms and Prospective Solutions

open access: yesAnimal Research and One Health, EarlyView.
Unregulated inflammation increases non‐esterified fatty acids (NEFAs), and triggers multi‐pathway hepatocyte damage including oxidative stress, mitochondrial dysfunction, and metabolic disorders in dairy cows. ABSTRACT Circulating concentrations of nonesterified fatty acids (NEFAs) are elevated due to lipid mobilization from adipose tissue in ...
Siqing Mao   +12 more
wiley   +1 more source

Neuronal differentiation and tissue engineering strategies for central neurous system injury repair

open access: yesBMEMat, EarlyView.
This review outlines tissue engineering advances for central nervous system (CNS) injury treatment, focusing on three core components: seed cells, inductive factors, and scaffold materials, with evaluation of their respective strengths and limitations. Tissue engineering for CNS injury repair.
Zhuqing Xia   +9 more
wiley   +1 more source

Targeting neutrophil extracellular traps in metabolic and immune niche: Nanomaterials for diabetes tissue regeneration

open access: yesBMEMat, EarlyView.
The effects of NETs on regeneration of various diabetic tissues, and strategies targeting NETs for diabetes tissue regeneration. In the diabetic environment, NETs undergo complex metabolic and immune reprogramming, leading to dynamic changes in antibacterial and proinflammatory functions, and affecting regeneration of multiple systemic tissues.
Xinyi Jiang   +6 more
wiley   +1 more source

CoA protects against the deleterious effects of caloric overload in Drosophila [PDF]

open access: yes, 2016
We developed a Drosophila model of T2D in which high sugar (HS) feeding leads to insulin resistance. In this model, adipose TG storage is protective against fatty acid toxicity and diabetes.
Baranski, Thomas J   +2 more
core   +2 more sources

Streptozotocin induced hyperglycemia in the axolotl

open access: yesDevelopmental Dynamics, EarlyView.
Abstract Background Diabetes is a group of diseases characterized by loss of β cell mass and/or function, resulting in hyperglycemia. With no established curative treatment, this has initiated research in β cell regeneration. Current animal models have either limited regenerative capacity (mice) or small size and evolutionary distance from humans ...
Pernille Lajer Sørensen   +2 more
wiley   +1 more source

Rising Inpatient Demands for Inherited Metabolic Disorders: Impact on Pediatric Capacity

open access: yes
American Journal of Medical Genetics Part A, EarlyView.
Maria Paula Silva   +7 more
wiley   +1 more source

Inherited metabolic epilepsies–established diseases, new approaches

open access: yesEpilepsia Open, EarlyView.
Abstract Inherited metabolic epilepsies (IMEs) represent the inherited metabolic disorders (IMDs) in which epilepsy is a prevailing component, often determining other neurodevelopmental outcomes associated with the disorder. The different metabolic pathways affected by individual IMEs are the basis of their rarity and heterogeneity.
Itay Tokatly Latzer, Phillip L. Pearl
wiley   +1 more source

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