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- Department of Pathology (3)
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- DES (1)
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- Desmin-related myopathy (1)
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- Mitochondrial DNA depletion (1)
- Mitochondrial disease (1)
- Sidney Kimmel Medical College (1)
- Thomas Jefferson University; Gene regulation; Genetic disorder; Intestine; Neural development (1)
- Thomas Jefferson University; adult; Alcaligenes; antibiotic sensitivity; Article; asthma; bacterial infection; biopsy; Bordetella; Bordetella petrii; Bordetella petrii infection; case report; cystic fibrosis; endoscopic sinus surgery; female; gastroesophageal reflux; human; human tissue; matrix assisted laser desorption ionization time of flight mass spectrometry; minimum inhibitory concentration; nose septum reconstruction; phenotype; priority journal; RNA sequence; sinusitis; species identification; stomach paresis (1)
- Thomas Jefferson University; animal cell; animal experiment; animal model; animal tissue; Article; brain third ventricle; chemical modification; controlled study; corpus striatum; dopamine brain level; dopaminergic nerve cell; drug infusion; drug mechanism; enzyme activity; male; mouse; neuroprotection; nonhuman; osmotic minipump; Parkinson disease; substantia nigra; systemic therapy; treatment duration; treatment response; Vibrio cholerae (1)
- Whole exome sequencing (1)
Articles 1 - 4 of 4
Full-Text Articles in Pathology
Intraventricular Sialidase Administration Enhances Gm1 Ganglioside Expression And Is Partially Neuroprotective In A Mouse Model Of Parkinson's Disease., Jay S Schneider, Thomas N Seyfried, Hyo-S Choi, Sarah Kidd
Intraventricular Sialidase Administration Enhances Gm1 Ganglioside Expression And Is Partially Neuroprotective In A Mouse Model Of Parkinson's Disease., Jay S Schneider, Thomas N Seyfried, Hyo-S Choi, Sarah Kidd
Department of Pathology, Anatomy, and Cell Biology Faculty Papers
BACKGROUND: Preclinical and clinical studies have previously shown that systemic administration of GM1 ganglioside has neuroprotective and neurorestorative properties in Parkinson's disease (PD) models and in PD patients. However, the clinical development of GM1 for PD has been hampered by its animal origin (GM1 used in previous studies was extracted from bovine brains), limited bioavailability, and limited blood brain barrier penetrance following systemic administration.
OBJECTIVE: To assess an alternative therapeutic approach to systemic administration of brain-derived GM1 to enhance GM1 levels in the brain via enzymatic conversion of polysialogangliosides into GM1 and to assess the neuroprotective potential of this approach. …
Desmin Common Mutation Is Associated With Multi-Systemic Disease Manifestations And Depletion Of Mitochondria And Mitochondrial Dna., Elizabeth M. Mccormick, Lawrence C. Kenyon, Marni J. Falk
Desmin Common Mutation Is Associated With Multi-Systemic Disease Manifestations And Depletion Of Mitochondria And Mitochondrial Dna., Elizabeth M. Mccormick, Lawrence C. Kenyon, Marni J. Falk
Department of Pathology, Anatomy, and Cell Biology Faculty Papers
Desmin (DES) is a major muscle scaffolding protein that also functions to anchor mitochondria. Pathogenic DES mutations, however, have not previously been recognized as a cause of multi-systemic mitochondrial disease. Here, we describe a 45-year-old man who presented to The Children's Hospital of Philadelphia Mitochondrial-Genetics Diagnostic Clinic for evaluation of progressive cardiac, neuromuscular, gastrointestinal, and mood disorders. Muscle biopsy at age 45 was remarkable for cytoplasmic bodies, as well as ragged red fibers and SDH positive/COX negative fibers that were suggestive of a mitochondrial myopathy. Muscle also showed significant reductions in mitochondrial content (16% of control mean for citrate synthase …
Slit2/Robo1-Mir-218-1-Ret/Plag1: A New Disease Pathway Involved In Hirschsprung's Disease., Weibing Tang, Junwei Tang, Jun He, Zhigang Zhou, Yufeng Qin, Jingjing Qin, Bo Li, Xiaoqun Xu, Qiming Geng, Weiwei Jiang, Wei Wu, Xinru Wang, Yankai Xia
Slit2/Robo1-Mir-218-1-Ret/Plag1: A New Disease Pathway Involved In Hirschsprung's Disease., Weibing Tang, Junwei Tang, Jun He, Zhigang Zhou, Yufeng Qin, Jingjing Qin, Bo Li, Xiaoqun Xu, Qiming Geng, Weiwei Jiang, Wei Wu, Xinru Wang, Yankai Xia
Department of Pathology, Anatomy, and Cell Biology Faculty Papers
Hirschsprung's disease (HSCR) is a rare congenital disease caused by impaired proliferation and migration of neural crest cells. We investigated changes in expression of microRNAs (miRNAs) and the genes they regulate in tissues of patients with HSCR. Quantitative real-time PCR and immunoblot analyses were used to measure levels of miRNA, mRNAs, and proteins in colon tissues from 69 patients with HSCR and 49 individuals without HSCR (controls). Direct interactions between miRNAs and specific mRNAs were indentified in vitro, while the function role of miR-218-1 was investigated by using miR-218 transgenic mice. An increased level of miR-218-1 correlated with increased levels …
Bordetella Petrii Recovered From Chronic Pansinusitis In An Adult With Cystic Fibrosis, Laura Biederman, Marc Rosen Md, Brent S. Bobik, Amity L. Roberts, Phd, D(Abmm)
Bordetella Petrii Recovered From Chronic Pansinusitis In An Adult With Cystic Fibrosis, Laura Biederman, Marc Rosen Md, Brent S. Bobik, Amity L. Roberts, Phd, D(Abmm)
Department of Pathology, Anatomy, and Cell Biology Faculty Papers
No abstract provided.