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Articles 1 - 8 of 8
Full-Text Articles in Biology
The Decision Maker: Understanding The Role Of Ifrd1 In Urothelial Plasticity And Regeneration, Bisiayo Erionmwon Fashemi
The Decision Maker: Understanding The Role Of Ifrd1 In Urothelial Plasticity And Regeneration, Bisiayo Erionmwon Fashemi
Arts & Sciences Electronic Theses and Dissertations
The bladder urothelium forms a highly specialized watertight barrier to urinary wastes. The urothelium offers an unusual example of tissue regeneration: although urothelial cells do not rapidly turn over under physiological conditions, they have an impressive capacity to regenerate tissue upon injury. Even more remarkable, depending on the modality of injury (sterile, infectious) there appear to be two distinctive modes of urothelial regeneration. We have previously shown that in response to a urinary tract infection (UTI), the urothelial stem cell niche becomes activated and induces rapid restoration of the urothelium, whereas, regeneration following sterile injury does not involve stem cell …
Mechanisms Of Cross-Presentation By Cdc1s, Derek James Theisen
Mechanisms Of Cross-Presentation By Cdc1s, Derek James Theisen
Arts & Sciences Electronic Theses and Dissertations
Classical dendritic cells (cDCs) are specialized antigen presenting cells that can be divided into distinct subsets based on the types of pathogens they respond to and the type of immune response they generate. The cDC1 subset is specialized in priming CD8 T cell responses through the process of cross-presentation. During cross-presentation, exogenous protein antigens are taken up by cDC1 and presented on MHCI molecules, allowing for the priming of CD8 T cells during conditions when DCs themselves are not directly infected. The ability to cross-present in vivo is unique to cDC1, and is essential for anti-viral responses and rejection of …
Pathophysiology And Treatment Of Murine Globoid Cell Leukodystrophy, Yedda Li
Pathophysiology And Treatment Of Murine Globoid Cell Leukodystrophy, Yedda Li
Arts & Sciences Electronic Theses and Dissertations
Infantile globoid cell leukodystrophy (GLD, Krabbe disease) is a rapidly progressing, invariably fatal pediatric disorder first described in 1916. Krabbe disease is caused by a deficiency in the lysosomal enzyme, galactosylceramidase (GALC), and is characterized clinically by failure to thrive, limb stiffness, seizures, developmental regression, and death by 2-4 years of age. Galactosylceramidase degrades the cytotoxic glycolipid, galactosylsphingosine (psychosine). In the absence of GALC activity, psychosine accumulates primarily in oligodendrocytes and Schwann cells, resulting in profound demyelination. In 1972, psychosine was hypothesized to be responsible for the clinical signs associated with Krabbe disease. However, the ‘Psychosine Hypothesis’ has never been …
Notch-Mediated Regulation Of Atrial Arrhythmogenesis, Catherine Lipovsky
Notch-Mediated Regulation Of Atrial Arrhythmogenesis, Catherine Lipovsky
Arts & Sciences Electronic Theses and Dissertations
Abnormalities in electrical impulse generation and/or propagation that affect the heartճ normal rhythm are extremely common. Clinically, cardiac arrhythmias are prevalent worldwide, yet the molecular mechanisms underlying their pathology remain largely unknown. Current treatments for arrhythmias primarily target symptoms rather than the underlying cause and these treatments have limited efficacy. The most common risk factor for developing an arrhythmia is a previous cardiac injury; however, the mechanisms underlying this are not well described. My thesis work has demonstrated that the Notch signaling pathway, which is crucial for cardiac patterning and development and is normally quiescent in adult cardiomyocytes (CMs), is …
Elucidating The Effect Of Myopathy-Causing Mutations And Second-Site Suppressors On Client Processing By J-Domain Proteins, Melanie Y. Pullen
Elucidating The Effect Of Myopathy-Causing Mutations And Second-Site Suppressors On Client Processing By J-Domain Proteins, Melanie Y. Pullen
Arts & Sciences Electronic Theses and Dissertations
Defects in protein quality control may lead to protein misfolding and aggregation often associated with protein conformational disorders such as Alzheimerճ Disease and Limb Girdle Muscular Dystrophy, among others. Molecular chaperones protect against protein misfolding and aggregation. A chaperone of interest is the ubiquitously expressed type II Hsp40 co-chaperone DNAJB6, which assists in protein folding and disaggregation. Mutations within the DNAJB6 G/F domain have been associated with the dominantly inherited disease Limb-Girdle Muscular Dystrophy type 1D (LGMD1D), now referred to as LGMDD1. Our collaborators recently discovered novel LGMDD1-associated mutations in the J-domain of DNAJB6. In the enclosed body of work, …
Nonsense Mediated Rna Decay Promotes Survival Of Cells With Defective Splicing, Abigael Jeruto Cheruiyot
Nonsense Mediated Rna Decay Promotes Survival Of Cells With Defective Splicing, Abigael Jeruto Cheruiyot
Arts & Sciences Electronic Theses and Dissertations
Nonsense mediated RNA decay (NMD) is an RNA surveillance pathway present in all eukaryotes that detects and degrades nonsense mRNAs, which contain pre-mature translation termination codons. Nonsense mRNAs are prevalent when pre-mRNA splicing is altered or defective. Interestingly, defective pre-mRNA splicing is emerging as a major driver of cancer development, including development of myelodysplastic syndrome (MDS), leukemia, and some solid tumors. Moreover, pre-mRNA splicing is also thought to enhance NMD in human cells, although itճ still unclear whether and how splicing or splicing factors promote NMD. The role of NMD in regulating mis-spliced mRNA and the link between NMD and …
The Role Of Apolipoprotein E In Alzheimer Disease: From Therapy To Mechanism, Tien-Phat Vuong Huynh
The Role Of Apolipoprotein E In Alzheimer Disease: From Therapy To Mechanism, Tien-Phat Vuong Huynh
Arts & Sciences Electronic Theses and Dissertations
Alzheimer’s disease (AD) is a neurodegenerative disorder associated with irreversible damage to the brain, which manifests in cognitive dysfunction, memory loss, and eventual death. The pathological hallmarks of AD are amyloid plaques, which are cerebral aggregates consisting of fibrils of the amyloid β-protein (Aβ), and filamentous lesions of the microtubule-associated protein tau known as neurofibrillary tangles. In the early 1990s, the apolipoprotein E (apoE) was found to co-localize with amyloid plaques. The ε4 allele of the APOE gene was sequentially identified as the strongest genetic risk factor for AD, increasing the risk by 4 – 12-fold, whereas the ε2 allele …
Development Of An In Vitro Culture System For Cryptosporidium Parvum, Georgia Wilke
Development Of An In Vitro Culture System For Cryptosporidium Parvum, Georgia Wilke
Arts & Sciences Electronic Theses and Dissertations
Cryptosporidium is a genus of protozoan parasites that causes diarrheal disease in humans and other animals. There are two major species that cause disease in humans: C. parvum, which infects both humans and animals, and C. hominis, which primarily infects humans. A recent study investigating the etiologies of pediatric diarrheal illness in Africa and South Asia found that Cryptosporidium is the 2nd most prevalent cause of diarrhea in infants and may be a contributing factor to chronic malnutrition. This discovery has led to renewed interest in studying this parasite and a reexamination of the barriers to studying Cryptosporidium. The main …