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2011

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Two-Dimensional Computer Model Of Human Atrial Ablation, Samineh R. Esfahani Jan 2011

Two-Dimensional Computer Model Of Human Atrial Ablation, Samineh R. Esfahani

USF Tampa Graduate Theses and Dissertations

A finite-element model of a two-dimensional slice of human atrial tissue for the study of the electrograms and propagation of action potentials is presented. Action potential propagation is described by a reaction-diffusion model coupled with the complex Courtemanche et al. atrial cell model. The effects of recording electrode size and location on electrograms are presented. Action potential propagation as a result of atrial fibrillation ablation therapy is also modeled by defining a lesion area with decreased electrical conductivity. The effect of electrical conductivity and geometry of the lesion was also studied. It is shown that the success rate of atrial …


Does D-Cycloserine Augmentation Of Cbt Improve Therapeutic Homework Compliance For Pediatric Obsessive Compulsive Disorder?, Jennifer M. Park Jan 2011

Does D-Cycloserine Augmentation Of Cbt Improve Therapeutic Homework Compliance For Pediatric Obsessive Compulsive Disorder?, Jennifer M. Park

USF Tampa Graduate Theses and Dissertations

D-cycloserine (DCS), a partial agonist that acts on the N-methyl-D-aspartate (NMDA) receptor of the glutamatergic receptor complex, may enhance fear extinction learning during exposure-based therapy. Clinical studies in adults with obsessive-compulsive disorder (OCD) and non-OCD anxiety disorders - and a recent trial in pediatric OCD - have shown that DCS can improve treatment response to exposure therapy relative to placebo and exposure therapy. Some have hypothesized that improved treatment response is a function of increased compliance and engagement in therapeutic homework tasks, a core component of behavioral treatment. The present study examined the relationship between DCS and homework compliance in …


Informal Karst Education In The United States And Internationally, Leslie A. North Jan 2011

Informal Karst Education In The United States And Internationally, Leslie A. North

USF Tampa Graduate Theses and Dissertations

Despite the abundance of karst terrains and the important role they play in a wide variety of roles including supplying freshwater drinking supplies, no single, comprehensive study investigates the role of informal education for the improved understanding and protection of the terrains. Commonly overlooked anthropogenic karst disturbances partially occur because of the poor dissemination of scientific information to the general populace and policymakers and budgetary and time constraints of municipalities, thus generating a need to use informal education to fill these shortcomings. The purpose of this study was to: 1) establish the status of and quantify the amount of karst-related …


An Examination Of Demographic Variables And Their Relationships With Perceived Stress Among Caregivers Beginning A Parent Training Program, Amy Heath Patenaude Jan 2011

An Examination Of Demographic Variables And Their Relationships With Perceived Stress Among Caregivers Beginning A Parent Training Program, Amy Heath Patenaude

USF Tampa Graduate Theses and Dissertations

The purpose of this study was to investigate how levels of stress among caregivers beginning a behavioral parent training program are related to caregiver and child variables. Research questions were answered using archival data collected from 474 caregivers who participated in HOT DOCS, a behavioral parent training program, between January 2009 through July 2010. The three objectives of the study were to (a) examine caregivers' perceived stress in relation to caregiver demographic variables (i.e., gender, marital status, level of education); (b) examine caregivers' perceived stress in relation to child demographic variables (i.e., levels of externalizing and internalizing behavior and …


"We Have Never Known What Death Was Before"--A Just War Doctrine Critique Of U.S. History Textbooks, Mark Pearcy Jan 2011

"We Have Never Known What Death Was Before"--A Just War Doctrine Critique Of U.S. History Textbooks, Mark Pearcy

USF Tampa Graduate Theses and Dissertations

Textbooks are a significant element of the social studies curriculum and teacher pedagogical choice (Apple, 2004; Apple & Christian-Smith, 1991). Students' views of American history are dramatically affected by the textbook narratives to which they are exposed, and teachers often tilt their curricular choices based on the textbooks available to them (Luke, 2006; Schug, Western & Enochs, 1997). The history of our nation's armed conflicts is often presented, through our textbooks and our pedagogy, as a history of reluctant violence, which promotes a particular moral agenda that exerts control over our students' future beliefs and decisions. This is particularly important …


My Bad Romance: Exploring The Queer Sublimity Of Diva Reception, Blake Paxton Jan 2011

My Bad Romance: Exploring The Queer Sublimity Of Diva Reception, Blake Paxton

USF Tampa Graduate Theses and Dissertations

This study explores the historic relationship between pop music divas and gay male fandom. It charts fan experiences from the early 60s with Judy Garland to contemporary times with pop diva Lady Gaga. This project also gives a description of the embodied experience of Brett Farmer’s “queer sublimity of diva reception.” Farmer (2005) argues that diva worship among gay men has become a queer sublimity, “the transcendence of a limiting heteronormative materiality and the sublime reconstruction, at least in fantasy, of a more capacious, kinder, queerer world” (p. 170). Using the methods of participant observation in drag performance and karaoke …


Captivating The Captors: Re-Defining Masculinity, Identity And Post-Colonialism In Plutarch's Parallel Lives, Andrea Lea Pittard Jan 2011

Captivating The Captors: Re-Defining Masculinity, Identity And Post-Colonialism In Plutarch's Parallel Lives, Andrea Lea Pittard

USF Tampa Graduate Theses and Dissertations

This thesis investigates 1st-2nd century CE biographer and philosopher, Plutarch's, manipulation and construction of gender ideals in three sets of his Parallel Lives, Coriolanus and Alcibiades, Pelopidas and Marcellus, and Phocion and Cato the Younger in which he presented his particular version of the ideal man and route to manhood. Plutarch discouraged traditional paths to gaining masculine status and simultaneously promoted a type of masculinity that benefited other aspects of his identity, particularly promoting his social and economic position and ethnicity. He asserted throughout that martial men were not in control of their emotions and therefore were incomplete men. …


Role Of Protein Kinase C-Iota In Neuroblastoma And The Effect Of Ica-1, A Novel Protein Kinase C-Iota Inhibitor On The Proliferation And Apoptosis Of Neuroblastoma Cells, Prajit P. Pillai Jan 2011

Role Of Protein Kinase C-Iota In Neuroblastoma And The Effect Of Ica-1, A Novel Protein Kinase C-Iota Inhibitor On The Proliferation And Apoptosis Of Neuroblastoma Cells, Prajit P. Pillai

USF Tampa Graduate Theses and Dissertations

Protein Kinase C-iota (PKC-é), an atypical protein kinase C isoform manifests its potential as an oncogene by targeting various aspects of cancer cells such as growth, invasion and survival. PKC-é confers resistance to drug-induced apoptosis in cancer cells. The acquisition of drug resistance is a major obstacle to good prognosis in neuroblastoma. The focus of the dissertation was three-fold: First to study the role of PKC-é in the proliferation of neuroblastoma. Secondly, to identify the efficacy of [4-(5-amino-4-carbamoylimidazol-1-yl)-2,3-dihydroxycyclopentyl] methyl dihydrogen phosphate (ICA-1) as a novel PKC-é inhibitor in neuroblastoma cell proliferation and apoptosis. Finally, to analyze whether PKC-é could self-regulate …


The Relationship Of Principal Resiliency To Job Satisfaction And Work Commitment: An Exploratory Study Of K-12 Public School Principals In Florida, Jason Pepe Jan 2011

The Relationship Of Principal Resiliency To Job Satisfaction And Work Commitment: An Exploratory Study Of K-12 Public School Principals In Florida, Jason Pepe

USF Tampa Graduate Theses and Dissertations

The purpose of this study was to investigate characteristics associated with resilient school leaders. Principals juggle multiple responsibilities and work under increasingly stressful conditions. Despite recent role changes, added job responsibilities, and increased accountability, some principals remain remarkably resilient while working in a tumultuous environment. Using Henderson and Milstein's (2003) definition, principal resiliency was described as "the capacity to spring back, rebound, successfully adapt in the face of adversity, and develop social, academic, and vocational competence despite exposure to severe stress or simply to the stress that is inherent in today's world" (p. 7). This empirical study tested the theory …


A Case Study Of Women Educational Administrators And Their Perspectives On Work And Life Roles, Krissy Perkins Jan 2011

A Case Study Of Women Educational Administrators And Their Perspectives On Work And Life Roles, Krissy Perkins

USF Tampa Graduate Theses and Dissertations

Women are persistent in their pursuits to obtain advanced degrees in higher education, as evidenced by the growing percentage (53% in 2009 according to NCES, 2010). Their purposes for degree attainment are multiple and varied, as are their experiences in higher education. This case study investigated the perspectives of five females managing the roles of woman, mother, educational administrator, and doctoral student. Previous research has paid little attention to women who manage three roles, let alone four. Feminist Standpoint Theory undergirds this study and allows a conversation about power relations within the broader social order, allows the asking of questions …


Relations Among Classroom Support, Academic Self-Efficacy, And Perceived Stress During Early Adolescence, Krystle Kuzia Preece Jan 2011

Relations Among Classroom Support, Academic Self-Efficacy, And Perceived Stress During Early Adolescence, Krystle Kuzia Preece

USF Tampa Graduate Theses and Dissertations

The purpose of this study was to gain insight into the relations between support, academic self-efficacy, and stress during the transition into middle school. Research suggests that early adolescents experience an increase in stress across the middle school transition (e.g., Chung, et al., 1998), due to a mismatch between the individuals' developmental needs and the environment (Eccles et al., 1993). Stress has been found to be a risk factor for mental health disorders among adolescents (Grant et al., 2003). The current study examined if teacher and classmate support and academic self-efficacy served as external and internal resources for buffering stress …


Guiding Electric Fields For Electroporation Applications, Jose Rey Jan 2011

Guiding Electric Fields For Electroporation Applications, Jose Rey

USF Tampa Graduate Theses and Dissertations

Electroporation is the critical step in an electric field mediated drug or gene delivery protocol. Electroporation based protocols have been successfully demonstrated in cancer clinical trials, however, its impact in other applications is still under investigation. A significant roadblock to long term functioning of implantable biosensors in vivo is the tissue reaction in the form of fibrous encapsulation that results in reduced transport to the sensing element of the biosensor. In vivo gene electroporation has a great potential as a means to modify the transport properties of tissues in the proximity of the sensing element of implantable biosensors.

This dissertation …


Sequential Anaerobic And Algal Membrane Bioreactor (A2mbr) System For Sustainable Sanitation And Resource Recovery From Domestic Wastewater, Ana Lucia Prieto Jan 2011

Sequential Anaerobic And Algal Membrane Bioreactor (A2mbr) System For Sustainable Sanitation And Resource Recovery From Domestic Wastewater, Ana Lucia Prieto

USF Tampa Graduate Theses and Dissertations

An innovative wastewater treatment technology was developed to recover renewable resources, such as water, energy and nutrients, from sewage. First, a novel synthetic sewage was evaluated for its suitability to serve as an alternative substrate for lab-scale wastewater treatment (WWT) research. Based on granular dried cat food, Complex Organic Particulate Artificial Sewage (COPAS) is a commercially-available, flexible, and easy to preserve feed. Characteristics of COPAS, namely chemical composition, disintegration/dissolution kinetics, and anaerobic biodegradability, were determined. Anaerobic bioassays indicate that COPAS is highly biodegradable at the concentration used to simulate household sewage (1000 mg/L), with more than 72% of the theoretical …


Biological Effective Dose (Bed) Distribution Matching For Obtaining Brachytherapy Prescription Doses & Dosimetric Optimization For Hybrid Seed Brachytherapy, Jakub Pritz Jan 2011

Biological Effective Dose (Bed) Distribution Matching For Obtaining Brachytherapy Prescription Doses & Dosimetric Optimization For Hybrid Seed Brachytherapy, Jakub Pritz

USF Tampa Graduate Theses and Dissertations

Radioactive seed implant brachytherapy is a common radiotherapy treatment method for prostate cancer. In current clinical practice, a seed consists of a single isotope, such as 125I or 103Pd. A seed containing a mixture of two isotopes has been proposed for prostate cancer treatment. This study investigates a method for defining a prescription dose for new seed compositions based on matching the biological equivalent dose (BED) of a reference plan.

Ten prostate cancer cases previously treated using single isotope seeds (5 using 125I seeds and 5 using 103Pd seeds) were selected for this study. Verification of …


Synthesis And Antimicrobial Activities Of S,S'-Heterosubstituted Disulfides, Praveen Ramaraju Jan 2011

Synthesis And Antimicrobial Activities Of S,S'-Heterosubstituted Disulfides, Praveen Ramaraju

USF Tampa Graduate Theses and Dissertations

Antibiotic resistance is a particularly critical health concern and has increased dramatically over the past two decades. For over a decade the Turos laboratory has been designing small molecules to target pathogenic microbes such as Staphylococcus aureus and the resistant variants like methicillin-resistant Staphylococcus aureus (MRSA). Previously, N-thiolated Β-lactams, N-thiolated 2-oxazolidinones and aromatic disulfides that were synthesized in Dr. Turos' lab have shown strong activity against these bacteria. The present work describes the synthesis and antimicrobial activities of a related structural class called S,S'-heterosubstituted disulfides. For ages, sulfur (elemental) has been used as an antibacterial for controlling infestation and bacterial …


Egyptian Body Size: A Regional And Worldwide Comparison, Michelle H. Raxter Jan 2011

Egyptian Body Size: A Regional And Worldwide Comparison, Michelle H. Raxter

USF Tampa Graduate Theses and Dissertations

Human body size and limb proportions can provide important information about adaptation, population movements, and health disparities. This study investigated changes in body size and limb proportions of adult Egyptians temporally and geographically in relation to climatological, sociopolitical and economic developments. It was predicted that Egyptian groups that experienced more environmental stress would be shorter and exhibit less sexual dimorphism. It was also predicted that Egyptians would be intermediate between higher and lower latitude populations in body form and limb length ratios. The main skeletal sample consisted of 492 males and 528 females, all adults from the Predynastic and Dynastic …


Characterization Of Aminopeptidase Pepz In Staphylococcus Aureus Virulence, Tiffany Marie Robison Jan 2011

Characterization Of Aminopeptidase Pepz In Staphylococcus Aureus Virulence, Tiffany Marie Robison

USF Tampa Graduate Theses and Dissertations

Staphylococcus aureus is a remarkably successful pathogen, accounting for an estimated 95,000 invasive infections annually in the U.S. alone. The burden of MRSA infections on public healthcare continues to rise, particularly with the continued spread of antibiotic resistant strains and the hyper-virulent CA-MRSA strains. The pathogenic nature of S. aureus can be attributed to the cache of virulence factors encoded within the genome of this organism. Typically, these are secreted toxins which directly interact with the host during infection, and facilitate pathogenesis. A previous screen in our laboratory investigating proteases in S. aureus identified a mutant in aminopeptidase Z as …


The Culture Of Mean: Gender, Race, And Class In Mediated Images Of Girls' Bullying, Emily Davis Ryalls Jan 2011

The Culture Of Mean: Gender, Race, And Class In Mediated Images Of Girls' Bullying, Emily Davis Ryalls

USF Tampa Graduate Theses and Dissertations

This dissertation examines narratives about female bullying and aggression through mediated images of "mean girls." Through textual analysis of popular media featuring mean girls (television shows such as Gossip Girl and films like Mean Girls), as well as national news coverage of the case of Phoebe Prince, who reportedly committed suicide after being bullied by girls from her school, this feminist examination questions how the image of the mean girl is raced and classed. This dissertation values an interdisciplinary approach to research that works to make sense of the forces that produce bodies as gendered, raced, and classed.

One of …


Secreted Factors From Human Umbilical Cord Blood Cells Protect Oligodendrocytes From Ischemic Insult, Derrick Rowe Jan 2011

Secreted Factors From Human Umbilical Cord Blood Cells Protect Oligodendrocytes From Ischemic Insult, Derrick Rowe

USF Tampa Graduate Theses and Dissertations

Oligodendrocytes (OL)s are the dominant cell type in the white matter and are integral for synaptic transmission essential for proper neuronal communication between brain areas. Previous studies have shown that intravenous administration of the mononuclear fraction of human umbilical cord blood (HUCB) cells in rat models of stroke reduced white matter injury, gray matter injury and behavioral deficits. Yet the mechanisms used by HUCB cells remain unknown in ischemic injury. These studies will investigate both in vitro and in vivo approaches to elucidate this mechanism in OLs. When mature primary OLs were coincubated with HUCB cells, HUCB cells secreted soluble …


Computational Discovery Of Phenotype Related Biochemical Processes For Engineering, Andrea M. Rocha Jan 2011

Computational Discovery Of Phenotype Related Biochemical Processes For Engineering, Andrea M. Rocha

USF Tampa Graduate Theses and Dissertations

Application of bioengineering technologies for enhanced biological hydrogen production is a promising approach that may play a vital role in sustainable energy. Due to the ability of several naturally occurring microorganisms to generate hydrogen through varying metabolic processes, biological hydrogen has become an attractive alternative energy and fuel source.

One area of particular interest is the production of biological hydrogen in organically-rich engineered systems, such as those associated with waste treatment. Despite the potential for high energy yields, hydrogen yields generated by bacteria in waste systems are often limited due to a focus on microbial utilization of organic material towards …


Visual Search For Smoking Stimuli: Detection And Distraction, Jason A. Oliver Jan 2011

Visual Search For Smoking Stimuli: Detection And Distraction, Jason A. Oliver

USF Tampa Graduate Theses and Dissertations

Extensive research has shown that the attentional systems of addicted individuals are biased towards drug-related stimuli, but despite several decades of effort these results have frequently been inconsistent. Though commonly believed to result from addiction and dependence, cognitive research would suggest that frequent exposure to drug-related stimuli could affect the attentional processing of drug-related cues even if no actual drug use occurs. The present investigation examined attentional bias for smoking cues using a novel visual search paradigm amongst smokers currently in nicotine withdrawal and fully satiated smokers, as well as a non-smoker control group. Variables related to smoking behavior, as …


Evaluating The Get Into Fitness Today (Gift) Program: Weight Loss And The Roles Of Education And Empowerment, Emily Koby Novicki Jan 2011

Evaluating The Get Into Fitness Today (Gift) Program: Weight Loss And The Roles Of Education And Empowerment, Emily Koby Novicki

USF Tampa Graduate Theses and Dissertations

This study investigated the effects of Get Into Fitness Today (GIFT), a health program for adults that promotes balanced nutrition, physical activity, and weight loss through weekly meetings that provide education and social support. In addition to investigating the effects of GIFT, this evaluation sought to better understand explanatory models used by participants and program staff, and the roles of education and empowerment in weight loss and health education. This mixed methods, case study evaluation consisted of quantitative analysis of existing program records for 664 participants, 40 hours of participant observation of class sessions with ten GIFT groups, and follow-up …


Development Of A Vessel Mimicking Material For Use In Anatomically Realistic Doppler Flow Phantoms, Deirdre King, Carmel Moran, John Mcnamara, Andrew Fagan, Jacinta Browne Jan 2011

Development Of A Vessel Mimicking Material For Use In Anatomically Realistic Doppler Flow Phantoms, Deirdre King, Carmel Moran, John Mcnamara, Andrew Fagan, Jacinta Browne

Articles

Polyvinyl alcohol cryogel, (PVA-C) is presented as a vessel mimicking material for use in anatomically realistic Doppler flow phantoms. Three different batches of 10 % wt PVA-C containing (i) PVA-C alone, (ii) PVA-C with anti-bacterial agent and (iii) PVA-C with silicon carbide particles were produced, each with 1 to 6 freeze-thaw cycles. The resulting PVA-C samples were characterized acoustically (over a range 2.65 - 10.5 MHz) and mechanically in order to determine the optimum mixture and preparation for mimicking the properties of healthy and diseased arteries found in vivo. This optimum mix was reached with the PVA-C with anti-bacterial agent …


Comparative Imaging Study In Ultrasound, Mri, Ct And Dsa Using A Multi-Modality Renal Artery Phantom, Deirdre King, Andrew Fagan, Carmel Moran, Jacinta Browne Jan 2011

Comparative Imaging Study In Ultrasound, Mri, Ct And Dsa Using A Multi-Modality Renal Artery Phantom, Deirdre King, Andrew Fagan, Carmel Moran, Jacinta Browne

Articles

A range of anatomically realistic multimodality renal artery phantoms consisting of vessels with varying degrees of stenosis was developed and evaluated using four imaging techniques currently used to detect renal artery stenosis (RAS). The spatial resolution required to visualize vascular geometry and the velocity detection performance required to adequately characterize blood flow in patients suffering from RAS are currently ill-defined, with the result that no one imaging modality has emerged as a gold standard technique for screening for this disease.

Methods:

The phantoms, which contained a range of stenosis values (0%, 30%, 50%, 70%, and 85%), were designed for use …


Mitophagy And Mitochondrial Morphology In Human Melanoma-Derived Cells Post Exposure To Simulated Sunlight, Luciene M. Zanchetta, Amaya Garcia, Fiona Lyng, James Walsh, James E.J. Murphy Jan 2011

Mitophagy And Mitochondrial Morphology In Human Melanoma-Derived Cells Post Exposure To Simulated Sunlight, Luciene M. Zanchetta, Amaya Garcia, Fiona Lyng, James Walsh, James E.J. Murphy

Articles

Purpose: To assess changes in mitochondrial morphology and mitophagy induced by simulated sunlight irradiation (SSI) and how these changes are modulated by mitochondrial activity and energy source. Materials and methods: Human malignant amelanotic melanoma A375 cells were pre-treated with either a mitochondrial activity enhancer, uncoupler or were either melanin or glutamine supplemented/starved for 4 hours pre-exposure to sunlight. A Q-Sun Solar Simulator (Q-Lab, Homestead, FL, USA) was employed to expose cells to simulated sunlight. Confocal microscopy imaging of A375 cells co-loaded with mitochondria and lysosome-specific fluorescent dyes was used to identify these organelles and predict mitophagic events. Results: SSI induces …


What Role For “Women,” “Men,” And Transpeople/Intersex People In Gender Equality: A Commentary, Edward D. Stein Jan 2011

What Role For “Women,” “Men,” And Transpeople/Intersex People In Gender Equality: A Commentary, Edward D. Stein

Articles

This has been a very rich opening panel to what promises to be a terrific conference. The panelists' comments go in such varied directions that it is a challenge to be both a commentator and summarizer, especially when I have just a few minutes to speak. What I plan to do is say a little bit to draw together some of the themes that the panelists have been talking about that are connected to a couple of topics I have been working on while at the same time trying to look forward to some of the things I hope we …


Is The "Post" In Post-Racial The "Blind" In Colorblind?, Ian F. Haney López Jan 2011

Is The "Post" In Post-Racial The "Blind" In Colorblind?, Ian F. Haney López

Cardozo Law Review

No abstract provided.


Insecure Attachment, Self-Discrepancy, Suicidal Tendency In A Sample Of Korean And Korean-American: A Path Model Of Suicide, Joongkeun Joseph Yoo Jan 2011

Insecure Attachment, Self-Discrepancy, Suicidal Tendency In A Sample Of Korean And Korean-American: A Path Model Of Suicide, Joongkeun Joseph Yoo

Doctoral Dissertations and Projects

Suicide, an intended self-destructive behavior, has various etiological risk factors related to social, biochemical, psychological, and even political components. Due to the need of psychological investigations for the multidisciplinary assessment of suicide, the current study was designed to investigate the relationship between attachment and suicide tendency with the mediator, self-discrepancy. A path analysis was utilized in this study to evaluate for the relationship among variables in the model. For the study, the Experiences in Close Relationships Scale-Short Form (ECR-S), the Integrated Self-Discrepancy Index (ISDI), and the Positive and Negative Suicide Ideation Inventory (PANSI) were employed as assessment instruments. The sample …


Volume 36, No. 2 Jan 2011

Volume 36, No. 2

The Weldon Times

A student-run newspaper published at the Schulich School of Law at Dalhousie University.

Editor in Chief: Geoff Franklin

Layout Editor: Lorne Graburn

Assistant Editors: Alex Gorlewski, Victoria Gergan, Patrick MacDonald, Max Rothschild


Protein Replacement Therapy Partially Corrects The Cholesterol-Storage Phenotype In A Mouse Model Of Niemann-Pict Type C2 Disease, Gitte Krogh Nielsen, Frederik Dagnaes-Hansen, Ida Elisabeth Holm, Steve Meaney, Derek Symula, Niels Trolle Andersen, Christian Wurtz Heegaard Jan 2011

Protein Replacement Therapy Partially Corrects The Cholesterol-Storage Phenotype In A Mouse Model Of Niemann-Pict Type C2 Disease, Gitte Krogh Nielsen, Frederik Dagnaes-Hansen, Ida Elisabeth Holm, Steve Meaney, Derek Symula, Niels Trolle Andersen, Christian Wurtz Heegaard

Articles

Niemann-Pick type C2 (NPC2) disease is a fatal autosomal recessive neurovisceral degenerative disorder characterized by late endosomal-lysosomal sequestration of low-density lipoprotein derived cholesterol. The breach in intracellular cholesterol homeostasis is caused by deficiency of functional NPC2, a soluble sterol binding protein targeted to the lysosomes by binding the mannose-6-phosphate receptor. As currently there is no effective treatment for the disorder, we have investigated the efficacy of NPC2 replacement therapy in a murine gene-trap model of NPC2-disease generated on the 129P2/OlaHsd genetic background. NPC2 was purified from bovine milk and its functional competence assured in NPC2-deficient fibroblasts using the specific cholesterol …