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Two-year follow-up of gait and postural control following initiation of recombinant human tripeptidyl intracerebroventricular enzyme replacement therapy in two atypical CLN2 patients.
Sci Rep
January 2025
Division of Metabolic Disorders, CHOC Children's Hospital, Orange, CA, 92868, USA.
Neuronal ceroid lipofuscinosis type 2 (CLN2) is a rapidly progressive neurodegenerative disorder leading to premature mortality. Ambulatory CLN2 patients typically receive standard of care treatment through biweekly intracerebroventricular (ICV) enzyme replacement therapy (ERT) involving recombinant human tripeptidyl peptidase 1, known as cerliponase alfa (Brineura, Biomarin Pharmaceuticals). This study longitudinally assessed the impact of ICV cerliponase alfa ERT on gait, and postural control across a two-year span in two siblings diagnosed with atypical CLN2 disease.
View Article and Find Full Text PDFNew Hydrogenated Phenanthrene Glycosides from the Edible Vegetable W.T.Wang with DPP-IV Inhibitory and Hepatoprotective Activity.
J Agric Food Chem
January 2025
Ph.D. Program in Clinical Drug Development of Herbal Medicine, College of Pharmacy, Taipei Medical University, Taipei 11031, Taiwan.
Based on molecular networking-guided isolation, 15 previously undescribed hydrogenated phenanthrene glycosides, including eight hexahydro-phenanthrenone glycosides, four tetrahydro-phenanthrenone glycosides, one dihydro-phenanthrenol glycoside, two dimers, and two known dihydrophenanthrene glycosides, were isolated from W.T.Wang, a popular regional edible vegetable at the northwest region of Vietnam.
View Article and Find Full Text PDFMulti Targeted Activity of Cocculus hirsutus through Modulation of DPP-IV and PTP-1B Leading to Enhancement of Glucose Uptake and Attenuation of Lipid Accumulation.
Appl Biochem Biotechnol
January 2025
Tissue Culture and Drug Discovery Laboratory, Department of Biotechnology, Anna University, Chennai, 600 025, India.
Multi-targeted therapies are gaining attention in the management of multifactorial diseases due to their poly pharmacology, enhanced potency and reduced toxicity. Metabolic disorders like Type 2 diabetes mellitus (T2DM) and obesity necessitate multi-targeted therapy to improve insulin sensitivity, regulate glucose homeostasis and support weight loss. Medicinal plants rich in bioactive compounds exhibit multi-targetted action with minimal side effects.
View Article and Find Full Text PDFDPPX antibody-mediated disease mimicking Wernicke's encephalopathy.
BMJ Case Rep
January 2025
Neurology, Joondalup Health Campus, Joondalup, Western Australia, Australia.
Anti-dipeptidyl-peptidase-like protein 6 antibody-mediated disease is a rare autoimmune encephalitis typically presenting with diarrhoea and/or weight loss, central nervous system hyperexcitability and cognitive dysfunction. We present a case of a young woman with 10 days of diplopia and unsteadiness in the context of dysthymia and significant weight loss over 2 months. Initial examination demonstrated mixed dysconjugate nystagmus and ataxic gait.
View Article and Find Full Text PDFEffect of cooking treatment on protein digestibility, peptide profile and potential bioactive peptides of beef tripe during in vitro gastrointestinal digestion.
Food Chem
December 2024
College of Food Science, Southwest University, No.2 Tiansheng Road, Beibei District, Chongqing 400715, China; Chongqing Key Laboratory of Speciality Food Co-Built by Sichuan and Chongqing, No.2 Tiansheng Road, Beibei District, Chongqing 400715, China; Chongqing Engineering Research Center of Regional Food, No.2 Tiansheng Road, Beibei District, Chongqing 400715, China. Electronic address:
The purpose of this study was to understand the effects of cooking treatment on the protein hydrolysis of beef tripe and the release of potentially bioactive peptides using an in vitro gastrointestinal model. The results showed that digestion promoted the hydrolysis of proteins and release of free amino acids in beef tripe, but cooking treatment significantly reduced them. The sample of the cooked beef tripe after gastrointestinal digestion had the highest antioxidant activity.
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