Results 201 to 210 of about 23,025 (248)
PTA alleviates atherosclerosis by regulating the MDM2/GPX4 axis‐mediated endothelial ferroptosis. ABSTRACT Endothelial ferroptosis is a crucial pathogenic driver of atherosclerosis (AS) progression. Protosappanin A (PTA), a bioactive compound from Caesalpinia sappan L., protects cardiovascular vessels by regulating ferroptosis.
Jiamei Fu +6 more
wiley +1 more source
Alpha-Mannosidosis in a 3.5-Year-Old Girl: A Case Report. [PDF]
Bonilla Fornes S +4 more
europepmc +1 more source
Stability of alglucosidase alfa in 0.9% sodium chloride for enzyme replacement therapy in patients with Pompe disease: insights from enzyme activity and cellular uptake measurements. [PDF]
Barzel I +5 more
europepmc +1 more source
Naturally occurring lysosomal storage disease consistent with neuronal ceroid lipofuscinosis in a group of 5 related captive rhesus macaques (<i>Macaca mulatta</i>). [PDF]
Olstad K +5 more
europepmc +1 more source
Fetal Hepatosplenomegaly: Stepwise Diagnostic Framework, Diagnostic Approach to Fetal Hepatosplenomegaly. [PDF]
Mazek M, Ciebiera M, Massalska D.
europepmc +1 more source
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Treatment for Lysosomal Storage Disorders
Current Pharmaceutical Design, 2020Lysosomal storage disorders comprise a group of approximately 70 types of inherited diseases resulting due to lysosomal gene defects. The outcome of the defect is a deficiency in either of the three: namely, lysosomal enzymes, activator protein, or transmembrane protein, as a result of which there is an unwanted accumulation of biomolecules inside ...
Jayesh Sheth, Aadhira Nair
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Lysosomal Biogenesis in Lysosomal Storage Disorders
Experimental Cell Research, 1997Lysosomal biogenesis is an orchestration of the structural and functional elements of the lysosome to form an integrated organelle and involves the synthesis, targeting, functional residence, and turnover of the proteins that comprise the lysosome. We have investigated lysosomal biogenesis during the formation and dissipation of storage vacuoles in two
Karageorgos, L. +6 more
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2021
Abstract Lysosomes are membrane-bound organelles that degrade various macromolecules. Lysosomal storage diseases are a clinically, enzymatically, and genetically heterogeneous group of disorders resulting from intracellular accumulation of substrates.
Angela Sun +3 more
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Abstract Lysosomes are membrane-bound organelles that degrade various macromolecules. Lysosomal storage diseases are a clinically, enzymatically, and genetically heterogeneous group of disorders resulting from intracellular accumulation of substrates.
Angela Sun +3 more
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Autophagy and Lysosome Storage Disorders
2020Lysosomal storage disorders (LSDs) are one of the most common human genetic metabolic diseases caused by gene mutations. Up to now, more than 70 LSDs have been identified and mainly divided into five categories. LSDs are mainly caused by defects in the function of enzymes or lysosomal-related proteins in lysosomes, which causes progressive accumulation
Haigang, Ren, Guanghui, Wang
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Seminars in Respiratory and Critical Care Medicine
Abstract Lysosomes are intracellular organelles that are responsible for degrading and recycling macromolecules. Lysosomal diseases (LDs) are a group of rare inherited diseases caused by deleterious variants affecting genes that encode the lysosomal enzymes, their transporter or their cofactor.
Jacopo, Cefalo +6 more
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Abstract Lysosomes are intracellular organelles that are responsible for degrading and recycling macromolecules. Lysosomal diseases (LDs) are a group of rare inherited diseases caused by deleterious variants affecting genes that encode the lysosomal enzymes, their transporter or their cofactor.
Jacopo, Cefalo +6 more
openaire +2 more sources

