Results 71 to 80 of about 2,600,769 (206)

Clinical characteristics of children with lysosomal acid lipase deficiency

, 2022
Lysosomal acid lipase deficiency (LAL-D) is a rare hereditary disorder, caused by pathogenic variant in the LIPA gene. LAL-D is screened as a secondary disorder among other rare dyslipidemias exhibiting with hypercholesterolemia as part of the Slovenian ...
Sustar, U (via Mendeley Data)
core   +1 more source

Characterization of lysosomal acid lipase mutations in the signal peptide and mature polypeptide region causing Wolman disease

Journal of Lipid Research, 2001
Wolman disease results from an inherited deficiency of lysosomal acid lipase (LAL; EC 3.1.1.13). This enzyme is essential for the hydrolysis of cholesteryl esters and triacylglycerols derived from endocytosed lipoproteins.
Oliver Zschenker, Nikola Jung, Julia Rethmeier, Sabine Trautwein, Stefanie Hertel, Marsha Zeigler, Detlev Ameis   +6 more
doaj   +1 more source

Bisphenol A and Its Analogs: Toxicity Analysis in Mouse and Rat Liver—A Review

Journal of Applied Toxicology, EarlyView.
ABSTRACT Bisphenol A (BPA) and its analogs are widely recognized for their use in industry and plastic manufacturing. Consequently, humans and animals are continually exposed to various substances in their daily lives. This review evaluates the hepatic toxicity of BPA and its analogs in mice and rats, with particular emphasis on molecular mechanisms ...
Sevda Bagdatli, Muhammed Canik, Gizem Gencay, Nurbanu Zengin, Tarık Dinc, Nazan Deniz Yön   +5 more
wiley   +1 more source

Analysis of Carbohydrates and Glycoconjugates by Matrix‐Assisted Laser Desorption/Ionization Mass Spectrometry: An Update for 2023–2024

Mass Spectrometry Reviews, EarlyView.
ABSTRACT The use of MALDI mass spectrometry for the analysis of carbohydrates and glycoconjugates is a well‐established technique and this comprehensive review is the twelfth update of the original article published in 1999 and brings coverage of the literature to the end of 2024.
David J. Harvey
wiley   +1 more source

Novel treatment options for lysosomal acid lipase deficiency: critical appraisal of sebelipase alfa

, 2016
Kim Su,1 Emma Donaldson,1 Reena Sharma2 1Division of Gastroenterology/Hepatology, 2The Mark Holland Metabolic Unit, Salford Royal Hospital NHS Foundation Trust, Salford, UK Abstract: Lysosomal acid lipase deficiency (LAL-D) is a rare disorder of ...
Sharma R, Su K, Donaldson E
core  

Aspartic Protease Inhibition Induces Proteomic Remodeling in Paracoccidioides brasiliensis

PROTEOMICS, EarlyView.
ABSTRACT Paracoccidioidomycosis (PCM) is a major systemic mycosis in Latin America caused by Paracoccidioides brasiliensis, yet the contribution of aspartic proteases to fungal physiology and pathogenicity remains poorly understood. Here, we employed data‐independent acquisition (DIA)‐based quantitative proteomics to investigate the impact of pepstatin
Sarah Fernandes Lima, Wilson Dias Segura, Leonardo Brogliato de Moraes, Reinaldo Souza Oliveira, Pedro Henrique Corteletti Manfio, Alison Felipe Alencar Chaves, Wagner Luiz Batista   +6 more
wiley   +1 more source

Reprogramming of lysosomal gene expression by interleukin-4 and Stat6. [PDF]

, 2013
BACKGROUND: Lysosomes play important roles in multiple aspects of physiology, but the problem of how the transcription of lysosomal genes is coordinated remains incompletely understood.
Meira, LB, Czimmerer, Z, Louise M Brignull, Hafida Saidi, Saidi, H, Bartlett, Nathan W., Bence Daniel, Nagy, László, Villela, Izabel, Nohturfft, A, Brignull, LM, Izabel Villela, Johnston, Sebastian L., Meira, Lisiane B., Villela, I, Axel Nohturfft, Nohturfft, Axel, Zsolt Czimmerer, Brignull, L. M., Nathan W Bartlett, Saidi, H., Dániel, Bence, Czimmerer, Zsolt, Daniel, B, Lisiane B Meira, Saidi, Hafida, Brignull, Louise M., Nagy, L, Villela, I., Laszlo Nagy, Bartlett, NW, Johnston, SL, Sebastian L Johnston   +32 more
core   +1 more source

Liquiritigenin Ameliorates High‐Fat Diet‐Induced Hepatic Steatosis by Promoting Lipid Droplet Catabolism via PLIN2‐ATGL Regulation

Phytotherapy Research, EarlyView.
Liquiritigenin promotes expression of CGI‐58 and enhances lipolysis by down‐regulating expression of PLIN2, thereby facilitating the catabolism of larger‐sized lipid droplets and producing smaller‐sized lipid droplets, resulting in enhanced activity of lipophagy and ameliorated hepatic steatosis. ABSTRACT Metabolic‐associated fatty liver disease (MAFLD)
Zhuoya Xu, Jingmiao Chen, Hong Qin, Yansong Fu, Cunqi Lv, Zhipeng Wang, Xi Liu, Wenya Zheng   +7 more
wiley   +1 more source

Dyslipidaemia, Chronic Kidney Disease and Diabetes Mellitus: A Triple Threat to Cardiovascular Health

Diabetes, Obesity and Metabolism, EarlyView.
ABSTRACT Diabetes mellitus (DM) and chronic kidney disease (CKD) frequently coexist and together create a ‘triple threat’ with dyslipidaemia, enhancing the risk for cardiovascular morbidity and mortality. Diabetic kidney disease (DKD) leads to altered lipid metabolism through insulin resistance, inflammation and oxidative stress resulting in an ...
Ann S. Forrest, Priyanka Majety, Stéphanie B. Mayer, Om P. Ganda   +3 more
wiley   +1 more source

Non-canonical lysosomal lipolysis drives mobilization of adipose tissue energy stores with fasting

Nature Communications
Physiological adaptations to fasting enable humans to survive for prolonged periods without food and involve molecular pathways that may drive life-prolonging effects of dietary restriction in model organisms.
GV Naveen Kumar, Rui-Sheng Wang, Ankit X. Sharma, Natalie L. David, Tânia Amorim, Daniel S. Sinden, Nandini K. Doshi, Martin Wabitsch, Sebastien Gingras, Asim Ejaz, J. Peter Rubin, Bradley A. Maron, Pouneh K. Fazeli, Matthew L. Steinhauser   +13 more
doaj   +1 more source