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2014

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Articles 7561 - 7590 of 9579

Full-Text Articles in Medicine and Health Sciences

Modulation Of Cns Neurotransmitter Levels And Associated Behaviors In Organic Anion Transporter 1 (Slc22a6) And Organic Anion Transporter 3 (Slc22a8) Knockout Mice, Christine Farthing Jan 2014

Modulation Of Cns Neurotransmitter Levels And Associated Behaviors In Organic Anion Transporter 1 (Slc22a6) And Organic Anion Transporter 3 (Slc22a8) Knockout Mice, Christine Farthing

Theses and Dissertations

According to the World Health Organization, mental disorders represent the leading cause of disability in the US generating ~58 billion dollars in medical costs annually. Additionally, among the US population, ~40 million adults suffer from an anxiety disorder and ~14 million suffer from a major depressive disorder. The association between the persistence of these neurobehavioral conditions and central nervous system (CNS) levels of biogenic amines and metabolites has been studied for half a century. Further, a number of drugs interfering with neurotransmission/metabolism are used clinically for treatment of these disorders. Recently, some members of the solute carrier (SLC) superfamily, the …


Quantitative Mass Spectrometric Investigations Of Protein Biomarkers: Serum Thymidine Kinase 1 And Human Osteopontin, Morse Faria Jan 2014

Quantitative Mass Spectrometric Investigations Of Protein Biomarkers: Serum Thymidine Kinase 1 And Human Osteopontin, Morse Faria

Theses and Dissertations

Mass spectrometry is being increasingly used in biomarker research mainly due to its ability to achieve high selectivity coupled with high sensitivity. This dissertation focuses on quantitative mass spectrometric investigations of two protein biomarkers i.e. serum thymidine kinase 1 (TK1) and human osteopontin (OPN).

First part of this research was focused on developing a liquid chromatography coupled with tandem mass spectrometry (LC-MS/MS) method for measuring the activity of TK1 in serum by monitoring the conversion of a TK1 specific exogenous substrate, 3’-deoxy-3’-fluorothymidine (FLT), to its mono-phosphorylated form 3’-deoxy-3’-fluorothymidine monophosphate (FLT-MP). A method to quantify FLT-MP on LC-MS/MS was developed and …


Statistical Modeling Of Interfractional Tissue Deformation And Its Application In Radiation Therapy Planning, Douglas J. Vile Jan 2014

Statistical Modeling Of Interfractional Tissue Deformation And Its Application In Radiation Therapy Planning, Douglas J. Vile

Theses and Dissertations

In radiation therapy, interfraction organ motion introduces a level of geometric uncertainty into the planning process. Plans, which are typically based upon a single instance of anatomy, must be robust against daily anatomical variations. For this problem, a model of the magnitude, direction, and likelihood of deformation is useful. In this thesis, principal component analysis (PCA) is used to statistically model the 3D organ motion for 19 prostate cancer patients, each with 8-13 fractional computed tomography (CT) images. Deformable image registration and the resultant displacement vector fields (DVFs) are used to quantify the interfraction systematic and random motion. By applying …


Examining The Sensory Characteristics Of Preschool Children With Retentive Fecal Incontinence, Isabelle Beaudry-Bellefeuille Jan 2014

Examining The Sensory Characteristics Of Preschool Children With Retentive Fecal Incontinence, Isabelle Beaudry-Bellefeuille

Theses and Dissertations

Occupational therapists are concerned with individuals´ abilities to engage in daily occupations. When a daily activity such as bowel management is problematic, participation in key occupations can be limited. Retentive fecal incontinence is a common disorder in children. Behavior seems to be partly responsible for this condition. Occupational therapists have hypothesized that some behaviors could be related to sensory over-responsivity. This study investigated the relationship between retentive fecal incontinence and sensory over-responsivity and examined the Toileting Habit Profile Questionnaire, a tool designed to screen for toileting difficulties. The study showed that a group of children (n=16) with retentive fecal incontinence …


The Scoop, Vol. 1 Issue 4, Summer 2014, Health Sciences Library Jan 2014

The Scoop, Vol. 1 Issue 4, Summer 2014, Health Sciences Library

Volume 1

Latest news and events from the Health Sciences Library in our monthly newsletter for Summer 2014.


Completely Intracorporeal Robotic-Assisted Laparoscopic Ileovesicostomy, Maryellen T. Dolat, Greg Wade, B. Mayer Grob, Lance J. Hampton, Adam P. Klausner Jan 2014

Completely Intracorporeal Robotic-Assisted Laparoscopic Ileovesicostomy, Maryellen T. Dolat, Greg Wade, B. Mayer Grob, Lance J. Hampton, Adam P. Klausner

Surgery Publications

We present a report of a completely intracorporeal robotic-assisted laparoscopic ileovesicostomy with long term follow-up. The patient was a 55-year-old man with paraplegia secondary to tropical spastic paresis resulting neurogenic bladder dysfunction. The procedure was performed using a da Vinci Surgical system (Intuitive Surgical, Sunnyvale, CA) and took 330 minutes with an estimated blood loss of 100 mL. The patient recovered without perioperative complications. He continues to have low pressure drainage without urethral incontinence over two years postoperatively.


Donor Heart Utilization Following Cardiopulmonary Arrest And Resuscitation: Influence Of Donor Characteristics And Wait Times In Transplant Regions, Mohammed Quader, Luke Wolfe, Gundars Katlaps, Vigneshwar Kasirajan Jan 2014

Donor Heart Utilization Following Cardiopulmonary Arrest And Resuscitation: Influence Of Donor Characteristics And Wait Times In Transplant Regions, Mohammed Quader, Luke Wolfe, Gundars Katlaps, Vigneshwar Kasirajan

Surgery Publications

Background. Procurement of hearts from cardiopulmonary arrest and resuscitated (CPR) donors for transplantation is suboptimal. We studied the influences of donor factors and regional wait times on CPR donor heart utilization. Methods. From UNOS database (1998 to 2012), we identified 44,744 heart donors, of which 4,964 (11%) received CPR. Based on procurement of heart for transplantation, CPR donors were divided into hearts procured (HP) and hearts not procured (HNP) groups. Logistic regression analysis was used to identify predictors of heart procurement. Results. Of the 4,964 CPR donors, 1,427 (28.8%) were in the HP group. Donor characteristics that favored heart procurement …


Is The Consistency More Important Than The Ingredients For Treatment? Case Report For Eosinophilic Esophagitis, Dan W. Parrish, Shashank Sharma, Santhosh Kumar Jan 2014

Is The Consistency More Important Than The Ingredients For Treatment? Case Report For Eosinophilic Esophagitis, Dan W. Parrish, Shashank Sharma, Santhosh Kumar

Surgery Publications

Eosinophilic Esophagitis seems to be an ever-changing disease, from patient-to-patient and from year-to-year in the same patient. Improvements in these patients’ lives have been made with the realization that elimination of dietary antigens helped with symptom relief and treatment with topical steroids assisted in eradication of esophageal eosinophils.

This case study has two very important aspects that should be emphasized. The first is the realization that it may be consistency of the steroid mixture rather than the ingredients that infer the effectiveness of the EoE treatment, and the second aspect is the willingness of the medical team to listen to …


Perspectives Volume 1: Number 1, Winter 2014, College Of Health Care Sciences Jan 2014

Perspectives Volume 1: Number 1, Winter 2014, College Of Health Care Sciences

Perspectives

No abstract provided.


Cyclic Peptide-Capped Gold Nanoparticles For Enhanced Sirna Delivery, Amir Nasrolahi Shirazi, Karissa L. Paquin, Niall G. Howlett, Dindyal Mandal, Keykavous Parang Jan 2014

Cyclic Peptide-Capped Gold Nanoparticles For Enhanced Sirna Delivery, Amir Nasrolahi Shirazi, Karissa L. Paquin, Niall G. Howlett, Dindyal Mandal, Keykavous Parang

Pharmacy Faculty Articles and Research

Previously, we have reported the synthesis of a homochiral L-cyclic peptide [WR]5 and its use for delivery of anti-HIV drugs and biomolecules. A physical mixture of HAuCl4 and the peptide generated peptide-capped gold nanoparticles. Here, [WR]5 and [WR]5-AuNPs were tested for their efficiency to deliver a small interfering RNA molecule (siRNA) in human cervix adenocarcinoma (HeLa) cells. Flow cytometry investigation revealed that the intracellular uptake of a fluorescence-labeled non-targeting siRNA (200 nM) was enhanced in the presence of [WR]5 and [WR]5-AuNPs by 2- and 3.8-fold when compared with that of siRNA alone after 24 h incubation. Comparative toxicity results showed …


Catalog (2014-2015), Philadelphia College Of Osteopathic Medicine Jan 2014

Catalog (2014-2015), Philadelphia College Of Osteopathic Medicine

Catalogs

A snapshot of the PCOM curriculum through the decades.


Applying Well-Being Assessment For Service Design, Margeret A. Hall, Steven O. Kimbrough, Christof Weinhardt Jan 2014

Applying Well-Being Assessment For Service Design, Margeret A. Hall, Steven O. Kimbrough, Christof Weinhardt

Interdisciplinary Informatics Faculty Proceedings & Presentations

Service design is transformative when it has a measurable, even optimizing, positive affect on human well-being. Any prospect for such felicitous outcomes, however, requires accurate assessment or measurement of well-being in and for target populations. Such assessment raises two immediate issues: conceptualization (How should well-being be conceptually operationalized?) and measurement (Given an operationalization of well-being, how can it be measured?). We begin to explore and address both questions in this paper by reviewing existing conceptualizations of well-being and then by describing the relevance of well-being measurement (and it methodologies) which are presently available.


An Extended Conceptual Framework For Transformative Service Research, Margeret A. Hall, Christian Haas, Steven O. Kimbrough, Christof Weinhardt Jan 2014

An Extended Conceptual Framework For Transformative Service Research, Margeret A. Hall, Christian Haas, Steven O. Kimbrough, Christof Weinhardt

Interdisciplinary Informatics Faculty Proceedings & Presentations

Transformative service research (TSR), a recently-envisioned branch of service science, is about understanding connections between service offerings and well-being. It has at the core of its conceptualization the goal of improving the well-being of individuals. A founding statement characterizes TSR as: “the integration of consumer and service research that centers on creating uplifting changes and improvements in the well-being of consumer entities: individuals (consumers and employees), communities and the ecosystem” (Anderson et al. 2013). It is also clear that service touches innumerable aspects of daily life. It is then natural that the field of service science explores mitigation of negative …


Solubility Enhancement And Precipitation Inhibition Of Poorly Water Soluble Compounds Utilizing Hot Melt Extrusion Technology, Jiannan Lu Jan 2014

Solubility Enhancement And Precipitation Inhibition Of Poorly Water Soluble Compounds Utilizing Hot Melt Extrusion Technology, Jiannan Lu

Electronic Theses and Dissertations

Soluplus® (SOL), a graft amorphous copolymer, composed of polyethylene glycol, vinyl acetate and vinylcaprolactam in a ratio of 13: 30: 57, was utilized to prepare solid dispersions containing felodipine (FEL) or ketoconazole (KTZ) using hot-melt extrusion technology. The melting point depression approach was utilized to determine the miscibility and solubility of the model compounds within Soluplus® , of which felodipine demonstrated higher solubility when compared to ketoconazole (14% vs 4.3% w/w) at room temperature (298K). Moreover, the solubility parameters of FEL, KTZ and SOL were calculated as 21.76, 26.51 and 21.64, respectively.

Polarized light microscopy, Fourier transform infrared spectroscopy (FT-IR), …


Firesetting And Maltreatment, Amy L. Burnett, Hatim A. Omar Jan 2014

Firesetting And Maltreatment, Amy L. Burnett, Hatim A. Omar

Pediatrics Faculty Publications

In this chapter we try to determine if a link exists between firesetting offenders and a past history of maltreatment. A literature review was performed using research from various studies and populations. Firesetting is a significant problem that affects the world's youth today. Even though there is substantial loss of property and life from juvenile firesetters, there is not a great deal of research to provide insight into early identification of those at risk. In the literature review performed, 6 of 7 studies found a link with maltreatment and firesetting behaviors. There does seem to be a correlation between the …


Children, Violence And Bullying, Joav Merrick, Isack Kandel, Hatim A. Omar Jan 2014

Children, Violence And Bullying, Joav Merrick, Isack Kandel, Hatim A. Omar

Pediatrics Faculty Publications

Adolescent violence is a very visible phenomenon in our modern society, where you just have to open the newspaper or the television and you find yourself right in the middle of it. In order to understand the scope of the problem, we need to look at the epidemiology of global violence. Each year, more than 1.6 million people worldwide lose their lives to violence and for every person who dies as a result of violence, many more are injured and suffer from a range of physical, sexual, reproductive and mental health problems. Violence places a massive burden on national economies …


Adolescent Violence Prevention, Hatim A. Omar Jan 2014

Adolescent Violence Prevention, Hatim A. Omar

Pediatrics Faculty Publications

Violence in adolescence has seen an increase since the 1990s with dramatic statistics on violent death and risk behaviors. School violence has been focused upon by a huge media coverage of especially violent cases that could have had some endemic consequences worldwide. We present a case of a 14 year old white male with change in school behavior, strategies for the case investigation, its results and long term prevention. Other research has shown that preventive measures during pregnancy, infancy and childhood can prevent adolescent and adult delinquency.


Prevention: Sexual Violence Against Adolescent And Young Adult Women, Kimberly K. Mcclanahan, Marlene B. Huff, Hatim A. Omar, Joav Merrick Jan 2014

Prevention: Sexual Violence Against Adolescent And Young Adult Women, Kimberly K. Mcclanahan, Marlene B. Huff, Hatim A. Omar, Joav Merrick

Pediatrics Faculty Publications

No abstract provided.


A Grassroots Program For Youth Suicide Prevention, Stephanie Stockburger, Hatim A. Omar Jan 2014

A Grassroots Program For Youth Suicide Prevention, Stephanie Stockburger, Hatim A. Omar

Pediatrics Faculty Publications

Suicide is a major public health problem. It affects people of all ages. Youth, in particular, have a disturbingly high suicide rate. Suicide is the third leading cause of death for young people between the ages of 10 and 24 (1). Approximately 4600 young people die each year from a suicide attempt (1). The most common methods used in suicides are firearms (45%), suffocation (which may be in the form of hanging) (40%), and poisoning (8%) (1). However, more young people survive suicide attempts than actually die from the attempt. A national survey of youth in both private and public …


Assessment Of Apparent Diffusion Coefficient Values As Predictor Of Aggressiveness In Peripheral Zone Prostate Cancer: Comparison With Gleason Score, Shayan Sirat Maheen Anwar, Zahid Anwar Khan, Rana Shoaib Hamid, Fahd Haroon, Raza Sayani, Madiha Beg, Yasir Jamil Khattak Jan 2014

Assessment Of Apparent Diffusion Coefficient Values As Predictor Of Aggressiveness In Peripheral Zone Prostate Cancer: Comparison With Gleason Score, Shayan Sirat Maheen Anwar, Zahid Anwar Khan, Rana Shoaib Hamid, Fahd Haroon, Raza Sayani, Madiha Beg, Yasir Jamil Khattak

Department of Radiology

Purpose. To determine association between apparent diffusion coefficient value on diffusion-weighted imaging and Gleason score in patients with prostate cancer. Methods. This retrospective case series was conducted at Radiology Department of Aga Khan University between June 2009 and June 2011. 28 patients with biopsy-proven prostate cancer were included who underwent ultrasound guided sextant prostate biopsy and MRI. MRI images were analyzed on diagnostic console and regions of interest were drawn. Data were entered and analyzed on SPSS 20.0. ADC values were compared with Gleason score using one-way ANOVA test. Results. In 28 patients, 168 quadrants were biopsied and 106 quadrants …


The Journal Of Undergraduate Research: Volume 12 Jan 2014

The Journal Of Undergraduate Research: Volume 12

The Journal of Undergraduate Research

This is the complete issue of the South Dakota State University Journal of Undergraduate Research, Volume 12.


Testing The Delivery Of An Interactive Second Grade Nutrition Education Curriculum, Kaitlyn Hemmingson Jan 2014

Testing The Delivery Of An Interactive Second Grade Nutrition Education Curriculum, Kaitlyn Hemmingson

The Journal of Undergraduate Research

Childhood obesity rates in the United States are alarming. Interventions incorporating nutrition education and physical activity are important for initiating behavior changes that may reduce the risk of childhood obesity and future health complications. The availability of nutrition curriculum for use in a second grade audience is limited. For this project, a nutrition education curriculum for second grade students was developed and tested over a two-week period in the fall of 2013. Six second grade classrooms (N=118) received four 20-minute interactive nutrition lessons. Lesson topics included: My Plate, Handy Portions, Sugar Shockers, and MyTray. Pre and post assessments of nutrition …


Evaluation And Characterization Of The Biomechanical Properties Of Tissue Engineered Vascular Grafts Implanted In The Arterial Circulation, Brooks Van Udelsman Jan 2014

Evaluation And Characterization Of The Biomechanical Properties Of Tissue Engineered Vascular Grafts Implanted In The Arterial Circulation, Brooks Van Udelsman

Yale Medicine Thesis Digital Library

We used a murine model to assess the in vivo evolving biomechanical properties of tissue engineered vascular grafts (TEVGs) implanted in the arterial circulation. The initial polymeric tubular scaffold was fabricated from (poly)lactic acid (PLA) and coated with a 50:50 copolymer of (poly)caprolactone and (poly)lactic acid (P[PC/LA]). Following seeding with syngeneic bone marrow derived mononuclear cells, the TEVGs (n=50) were implanted as aortic interposition grafts in wild-type mice and monitored serially using ultrasound. A custom biaxial mechanical testing device was used to quantify in vitro the circumferential and axial mechanical properties of grafts explanted at 3 or 7 months. At …


Singing An Imaginal Dialogue: A Study Of A Bereavement-Specific Music Therapy Intervention, Yasmine A. Iliya Jan 2014

Singing An Imaginal Dialogue: A Study Of A Bereavement-Specific Music Therapy Intervention, Yasmine A. Iliya

Expressive Therapies Dissertations

The purpose of this research was to understand the experience of singing an imaginal dialogue with a deceased loved one. This bereavement-specific music therapy intervention was an adaptation of Shear, Frank, Houck, and Reynolds’ (2005) imaginal dialogue intervention and was heavily influenced by Austin’s (2008) method of vocal psychotherapy. Following Shear’s (2006) use of the spoken imaginal dialogue with therapists, the guiding question of the present study was: What are creative arts therapists’ experiences of singing the intervention? The sample consisted of nine female creative arts therapists with a minimum of three years of clinical experience. It included women who …


Altered Mucins (Muc) Trafficking In Benign And Malignant Conditions., Suhasini Joshi, Sushil Kumar, Amit Choudhury, Moorthy P. Ponnusamy, Surinder K. Batra Jan 2014

Altered Mucins (Muc) Trafficking In Benign And Malignant Conditions., Suhasini Joshi, Sushil Kumar, Amit Choudhury, Moorthy P. Ponnusamy, Surinder K. Batra

Journal Articles: Biochemistry & Molecular Biology

Mucins are high molecular weight O-glycoproteins that are predominantly expressed at the apical surface of epithelial cells and have wide range of functions. The functional diversity is attributed to their structure that comprises of a peptide chain with unique domains and multiple carbohydrate moieties added during posttranslational modifications. Tumor cells aberrantly overexpress mucins, and thereby promote proliferation, differentiation, motility, invasion and metastasis. Along with their aberrant expression, accumulating evidence suggest the critical role of altered subcellular localization of mucins under pathological conditions due to altered endocytic processes. The mislocalization of mucins and their interactions result in change in the density …


Chewing Through The Miocene: An Examination Of The Feeding Musculature In The Ground Sloth Hapalops From South America (Mammalia: Pilosa), Virginia L Naples, Robert Mcafee Jan 2014

Chewing Through The Miocene: An Examination Of The Feeding Musculature In The Ground Sloth Hapalops From South America (Mammalia: Pilosa), Virginia L Naples, Robert Mcafee

PCOM Scholarly Works

Hapalops, a smaller-sized and early sloth of the Megatheroidea, appeared in the middle Miocene Santa Cruz formation of Argentina. This genus is part of the group from which later, larger megatheroids arose, i.e., Nothrotheriops and Megatherium. Many cranial characters support this idea; however Hapalops is not merely a smaller antecedent of the later forms. Specifically, Hapalops retains short anterior caniniform teeth, and a temporomandibular joint elevated above the cheek tooth row; a combination distinct among sloths. An elevated temporomandibular joint occurs in Bradypus, a tree sloth with anterior chisel-shaped teeth instead of caniniforms, and the tree sloth Choloepus, which is …


Safety And Efficacy Of Ceftaroline Fosamil In The Management Of Community-Acquired Bacterial Pneumonia, Heather F. Debellis, Kimberly L. Barefield Jan 2014

Safety And Efficacy Of Ceftaroline Fosamil In The Management Of Community-Acquired Bacterial Pneumonia, Heather F. Debellis, Kimberly L. Barefield

PCOM Scholarly Works

Ceftaroline fosamil is a new fifth-generation cephalosporin indicated for the treatment of community-acquired bacterial pneumonia (CABP). It possesses antimicrobial effects against both Gram-positive and Gram-negative bacteria, including methicillin-resistant Staphylococcus aureus (MRSA), but not against anaerobes. Organisms covered by this novel agent that are commonly associated with CABP are Streptococcus pneumoniae, Staphylococcus aureus, Haemophilus influenzae, Moraxella catarrhalis, and Klebsiella pneumoniae; however, ceftaroline fosamil lacks antimicrobial activity against Pseudomonas and Acinetobacter species. FOCUS 1 and FOCUS 2 clinical trials evaluated the use of ceftaroline fosamil in the treatment of CABP as compared to ceftriaxone. These non-inferiority trials provided evidence that ceftaroline fosamil …


Antibiotic Lock Therapy: Review Of Technique And Logistical Challenges, Julie Ann Justo, P Brandon Bookstaver Jan 2014

Antibiotic Lock Therapy: Review Of Technique And Logistical Challenges, Julie Ann Justo, P Brandon Bookstaver

Faculty Publications

Antibiotic lock therapy (ALT) for the prevention and treatment of catheter-related bloodstream infections is a simple strategy in theory, yet its real-world application may be delayed or avoided due to technical questions and/or logistical challenges. This review focuses on these latter aspects of ALT, including preparation information for a variety of antibiotic lock solutions (ie, aminoglycosides, beta-lactams, fluoroquinolones, folate antagonists, glycopeptides, glycylcyclines, lipopeptides, oxazolidinones, polymyxins, and tetracyclines) and common clinical issues surrounding ALT administration. Detailed data regarding concentrations, additives, stability/compatibility, and dwell times are summarized. Logistical challenges such as lock preparation procedures, use of additives (eg, heparin, citrate, or ethylenediaminetetraacetic …


Alternative Splicing In The Variable Domain Of Camkiiβ Affects The Level Of F-Actin Association In Developing Neurons., Jun Zheng, Lori R. Hardy, Chengshi Xu, Jing Kuang, Weijing Liao Jan 2014

Alternative Splicing In The Variable Domain Of Camkiiβ Affects The Level Of F-Actin Association In Developing Neurons., Jun Zheng, Lori R. Hardy, Chengshi Xu, Jing Kuang, Weijing Liao

PCOM Scholarly Works

The Ca(2+)/calmodulin (CaM)-dependent protein kinase II (CaMKII) β has an essential function in dendritic spines via binding to and reorganization of the actin cytoskeleton during plasticity events not shared by CaMKIIα isoform. CaMKIIβ and CaMKIIα isoforms have remarkable structural differences within the variable region. Three exons (E1, E3, and E4) are present in CaMKIIβ but not in CaMKIIα gene. Four splice variants of CaMKIIβ isoforms (CaMKIIβ, β', βe and β'e) were discovered in embryonic and adult brains. Exons E1 (lacked in βe and β'e) and E4 (lacked in β' and β'e) are subject to differential alternative splicing. We hypothesized that …


Phenotype Of Transgenic Mice Carrying A Very Low Copy Number Of The Mutant Human G93a Superoxide Dismutase-1 Gene Associated With Amyotrophic Lateral Sclerosis, Jeffrey S. Deitch, Guillermo M. Alexander, Andrew Bensinger, Steven Yang, Juliann T. Jiang, Terry D. Heiman-Patterson Jan 2014

Phenotype Of Transgenic Mice Carrying A Very Low Copy Number Of The Mutant Human G93a Superoxide Dismutase-1 Gene Associated With Amyotrophic Lateral Sclerosis, Jeffrey S. Deitch, Guillermo M. Alexander, Andrew Bensinger, Steven Yang, Juliann T. Jiang, Terry D. Heiman-Patterson

PCOM Scholarly Works

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease of the motor neuron. While most cases of ALS are sporadic, 10% are familial (FALS) with 20% of FALS caused by a mutation in the gene that codes for the enzyme Cu/Zn superoxide dismutase (SOD1). There is variability in sporadic ALS as well as FALS where even within the same family some siblings with the same mutation do not manifest disease. A transgenic (Tg) mouse model of FALS containing 25 copies of the mutant human SOD1 gene demonstrates motor neuron pathology and progressive weakness similar to ALS patients, leading to death …