Výsledky vyhledávání - "Naresh K Meena"

Akademický článek

Autor: Hung Do, Naresh K. Meena, Nina Raben

Publikováno v: Biomolecules, Vol 14, Iss 5, p 573 (2024)

Autophagy is an evolutionarily conserved lysosome-dependent degradation of cytoplasmic constituents. The system operates as a critical cellular pro-survival mechanism in response to nutrient deprivation and a variety of stress conditions. On top of t

Externí odkaz: https://doaj.org/article/28155909c15e4f089e699522abd860da

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Akademický článek

AAV-mediated delivery of secreted acid α-glucosidase with enhanced uptake corrects neuromuscular pathology in Pompe mice

Autor: Naresh K. Meena, Davide Randazzo, Nina Raben, Rosa Puertollano

Publikováno v: JCI Insight, Vol 8, Iss 16 (2023)

Gene therapy is under advanced clinical development for several lysosomal storage disorders. Pompe disease, a debilitating neuromuscular illness affecting infants, children, and adults with different severity, is caused by a deficiency of lysosomal g

Externí odkaz: https://doaj.org/article/bbcc172f43894f12a05e44541ce7a48f

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Akademický článek

MiT/TFE Family of Transcription Factors: An Evolutionary Perspective

Autor: Martina La Spina, Pablo S. Contreras, Alberto Rissone, Naresh K. Meena, Eutteum Jeong, José A. Martina

Publikováno v: Frontiers in Cell and Developmental Biology, Vol 8 (2021)

Response and adaptation to stress are critical for the survival of all living organisms. The regulation of the transcriptional machinery is an important aspect of these complex processes. The members of the microphthalmia (MiT/TFE) family of transcri

Externí odkaz: https://doaj.org/article/0863426872194a7a8652458cf7e0a48a

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Akademický článek

Pompe Disease: New Developments in an Old Lysosomal Storage Disorder

Autor: Naresh K. Meena, Nina Raben

Publikováno v: Biomolecules, Vol 10, Iss 9, p 1339 (2020)

Pompe disease, also known as glycogen storage disease type II, is caused by the lack or deficiency of a single enzyme, lysosomal acid alpha-glucosidase, leading to severe cardiac and skeletal muscle myopathy due to progressive accumulation of glycoge

Externí odkaz: https://doaj.org/article/0b18017493b341e99df24257190de271

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Chemoenzymatic glycan-selective remodeling of a therapeutic lysosomal enzyme with high-affinity M6P-glycan ligands. Enzyme substrate specificity is the name of the game†

Autor: Huiying Liu, Naresh K Meena, Rosa Puertollano, Guanghui Zong, Xiao Zhang, Lai-Xi Wang, Nina Raben, Chao Li

Publikováno v: Chemical Science

Functionalization of therapeutic lysosomal enzymes with mannose-6-phosphate (M6P) glycan ligands represents a major strategy for enhancing the cation-independent M6P receptor (CI-MPR)-mediated cellular uptake, thus improving the overall therapeutic e

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::0b9cf81ca0b87488d7f3bee7db598ece
http://europepmc.org/articles/PMC8480326

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Nutritional co-therapy with 1,3-butanediol and multi-ingredient antioxidants enhances autophagic clearance in Pompe disease

Autor: Mats I. Nilsson, Michael Crozier, Alessia Di Carlo, Donald Xhuti, Katherine Manta, Liza J. Roik, Adam L. Bujak, Joshua P. Nederveen, Milla G. Tarnopolsky, Bart Hettinga, Naresh K. Meena, Nina Raben, Mark A. Tarnopolsky

Publikováno v: Molecular genetics and metabolism. 137(1-2)

Alglucosidase alpha is an orphan drug approved for enzyme replacement therapy (ERT) in Pompe disease (PD); however, its efficacy is limited in skeletal muscle because of a partial blockage of autophagic flux that hinders intracellular trafficking and

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3104501cd3d0663612231fe7934137d6
https://pubmed.ncbi.nlm.nih.gov/35718712

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Enzyme Replacement Therapy Can Reverse Pathogenic Cascade in Pompe Disease

Autor: Rosa Puertollano, Evelyn Ralston, Naresh K Meena, Nina Raben

Publikováno v: Molecular Therapy: Methods & Clinical Development, Vol 18, Iss, Pp 199-214 (2020)
Molecular Therapy. Methods & Clinical Development

Pompe disease, a deficiency of glycogen-degrading lysosomal acid alpha-glucosidase (GAA), is a disabling multisystemic illness that invariably affects skeletal muscle in all patients. The patients still carry a heavy burden of the disease, despite th

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::535c96e9e7770a64909cbaba2a0f2c29
http://www.sciencedirect.com/science/article/pii/S2329050120301133

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