A5075109208- Retinal Diseases and Treatments
- Retinal Development and Disorders
- Peroxisome Proliferator-Activated Receptors
- Oral and Maxillofacial Pathology
- Viral Infectious Diseases and Gene Expression in Insects
- Biochemical Acid Research Studies
- Optimal Experimental Design Methods
- Neuroinflammation and Neurodegeneration Mechanisms
- 3D Printing in Biomedical Research
- Autophagy in Disease and Therapy
- Adipose Tissue and Metabolism
- Calcium signaling and nucleotide metabolism
- Bone and Dental Protein Studies
- Alzheimer's disease research and treatments
- Periodontal Regeneration and Treatments
- Mitochondrial Function and Pathology
- Sphingolipid Metabolism and Signaling
- Advanced Glycation End Products research
- Cancer, Hypoxia, and Metabolism
- Adenosine and Purinergic Signaling
University of Pennsylvania
2017-2021
Abstract Late-onset retinal degeneration (L-ORD) is an autosomal dominant disorder caused by a missense substitution in CTRP5 . Distinctive clinical features include sub-retinal pigment epithelium (RPE) deposits, choroidal neovascularization, and RPE atrophy. In induced pluripotent stem cells-derived from L-ORD patients (L-ORD-iRPE), we show that the pathogenic variant leads to reduced secretion. silico modeling suggests lower binding of mutant adiponectin receptor 1 (ADIPOR1). Downstream...
Like other neurons, retinal cells utilize autophagic pathways to maintain cell homeostasis. The mammalian retina relies on heterophagy and selective autophagy efficiently degrade metabolize ingested lipids with disruption in associated degradation contributing age related disorders. pigment epithelium (RPE) supports photoreceptor renewal by daily phagocytosis of shed outer segments (OS). ingestion these lipid-rich OS imposes a constant degradative burden terminally differentiated cells....
The retinal pigment epithelium (RPE) supports the outer retina through essential roles in retinoid cycle, nutrient supply, ion exchange, and waste removal. Each day RPE removes oldest ~10% of photoreceptor segment (OS) disk membranes phagocytic uptake, which peaks following light onset. Impaired degradation phagocytosed OS material by can lead to toxic accumulation lipids, oxidative tissue damage, inflammation, cell death. OSs are rich very long chain fatty acids, preferentially catabolized...
Visual function depends on the intimate structural, functional and metabolic interactions between retinal pigment epithelium (RPE) neural retina. The daily phagocytosis of photoreceptor outer segment tips by overlaying RPE provides essential nutrients for itself photoreceptors through intricate synergy. Age-related changes are often characterized dysregulation contributing to increased lipid accumulation peroxidation as well release proinflammatory cytokines. LGM2605 is a synthetic lignan...
In cell culture process development, we rely largely on an iterative, one-factor-at-a-time procedure based experiments that explore a limited space. Design of (DoE) addresses this issue by allowing us to analyze the effects inputs responses systematically and efficiently. However, DoE cannot be applied directly study time-varying unless impractically large number bioreactors is used. Here, adopt methodology design dynamic (DoDE) incorporate feeding profiles efficiently in late-stage...
Abstract The retinal pigment epithelium (RPE) supports the outer retina through essential roles in retinoid visual cycle, nutrient supply, ion exchange and waste removal. Each day RPE removes oldest ∼10% of photoreceptor segments phagocytic uptake, which peaks a synchronous burst following light onset. Impaired degradation phagocytosed OS material by can lead to toxic accumulation lipids, oxidative tissue damage, inflammation cell death. OSs are rich very long chain fatty acids...