Medicine and Health Sciences Commons^™

Validating A New In Vivo Model To Study Als, Izabela J. Cimachowska

Student Theses and Dissertations

Buildup of oxidative stress and mitochondrial dysfunction are well known characteristics of both sporadic and hereditary amyotrophic lateral sclerosis (ALS). While both forms of the disease seem to arise from common cellular dysfunction, the genetic disease is studied to a much greater extent. Engineering novel animal models of the sporadic form of the disease is crucial for development of druggable targets to treat ALS and understand the underlying mechanisms. Interestingly, accumulation of oxidative stress by exacerbated emission of reactive oxygen species (ROS) from presynaptic mitochondria is a hallmark of both hereditary and sporadic ALS. Previous work by our laboratory showed …

Amyotrophic Lateral Sclerosis: The Disease Of The Peripheral Nervous System, Aisling G. Lavelle

Student Publications

A literary review of Amyotrophic Lateral Sclerosis summarizing the most up to date information about ALS. The most recent prevalence and incidence reports globally and specifically the USA are reported. Characteristics of the phenotypes are described and a break down of symptoms and effective diagnosis measures are reported and explained. Genetic and environmental factors are summarized and broken down in layman's terms. Current research and treatments being performed for ALS patients and their future impacts are described.

How Does The Social World Shape The Experience Of A Rare Disease? Social Position And The Development, Progression, And Medical Care For People With Amyotrophic Lateral Sclerosis, Jennifer A. Andersen

Department of Sociology: Dissertations, Theses, and Student Research

This dissertation focuses on the implications of social position and life course on the experience of Amyotrophic Lateral Sclerosis (ALS). Using a sociology in medicine frame, I test three theoretical perspectives (fundamental cause theory, social determinants of health, and life course theory) to determine the influence of social conditions on the development and progression of, and medical care for, people with ALS (pALS). Further, I use ALS as an exemplar of the need for a sociology of disease.

Using the Amyotrophic Lateral Sclerosis National Registry, I first assess the association of social position with the reported onset location at the …

The Hapless State Of Amyotrophic Lateral Sclerosis In India: A Comprehensive Look At Life And Medical Services For Als Patients In Rural Himachal Pradesh, Carson J. Bergström

Independent Study Project (ISP) Collection

Amyotrophic lateral sclerosis (ALS) or Lou Gehrig’s disease is a neurodegenerative disorder that leads to paralysis. The disease requires a high amount of medical intervention and interdisciplinary focus to achieve quality of life for patients. This study looks at ALS patient’s lifestyles, their access to these medical devices, Indian therapeutic approaches and policy that impacts patients in Shimla, Himachal Pradesh. As caretakers have a critical part in the care for this disease, their lives were also considered in the case studies. It was found that ALS patients are not experiencing access to medical devices necessary for life because of physical …

Amyotrophic Lateral Sclerosis, Anastasia M. Georgetson

Student Publications

The word amyotrophic is derived from Greek, and means “without nourishment to muscles”, lateral means to the sides and sclerosis means hardened (“What is ALS?,” n.d.). First described by Jean-Martin Charcot in the 1800s, Amyotrophic Lateral Sclerosis (ALS) is a progressive degenerative motor neuron disease. Motor neurons are very important cells, and extremely unique since they can be very long with some motor neurons having a length of over a meter (“Disease Mechanisms,” n.d.).

About 5-10% of the cases of ALS are inherited, which is known as familial ALS or fALS, and it is known as autosomal dominant in these …

Longitudinal Screening Detects Cognitive Stability And Behavioral Deterioration In Als Patients, Susan Woolley, Ray Goetz, Pam Factor-Litvak, Jennifer Murphy, Jonathan Hupf, Catherine Lomen-Hoerth, Howard Andrews, Daragh Heitzman, Richard Bedlack, Jonathan Katz, Richard Barohn, Eric Sorenson, Bjorn Oskarsson, Americo Fernandes Filho, Edward J. Kasarskis, Tahseen Mozaffar, Sharon Nations, Andrea Swenson, Agnes Koczon-Jaremko, Georgia Christodoulou, Hiroshi Mitsumoto

Neurology Faculty Publications

Objective. To evaluate longitudinal cognitive/behavioral change over 12 months in participants enrolled in the ALS Multicenter Cohort Study of Oxidative Stress (ALS COSMOS). Methods. We analyzed data from 294 ALS participants, 134 of whom were studied serially. Change over time was evaluated controlling for age, sex, symptom duration, education, race, and ethnicity. Using multiple regression, we evaluated associations among decline in ALS Functional Rating Scale-Revised (ALSFRS-R) scores, forced vital capacity (FVC), and cognitive/behavioral changes. Change in cognitive/behavioral subgroups was assessed using one-way analyses of covariance. Results. Participants with follow-up data had fewer baseline behavior problems compared to patients …

Diseases At Work, Alice Liu '19, Nicholas Opiola '19, Gunnar Bergmann '19, Femi Durodola '20, Suraj Sunkara '19

Human Diseases Graphic Novels

No abstract provided.

Hsp90 And Its Co-Chaperones Modify Tdp-43 Localization, Aggregation, And Toxicity, Lilian T. Lin

Electronic Thesis and Dissertation Repository

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease associated with protein misfolding and protein aggregation. In particular, the TAR DNA-binding protein (TDP-43) is often found in the pathological inclusions in neurons of ALS patient brains and spinal cords. This phenomenon is known as TDP-43 proteinopathy, the mislocalization of TDP-43 from the cell nucleus and the formation of aggregates in the cytoplasm. Numerous mutations in the gene encoding TDP-43 have also been linked to familial cases of ALS (fALS) and cause TDP-43 proteinopathy. This study attempts to decipher how the molecular chaperone Hsp90 and its co-chaperones, Aha1, Sti1, and Cdc37, modulate …

Three Damn Letters, Katherine Ammon

Honors Program Theses

Amyotrophic lateral sclerosis (ALS) is a terminal disease that destroys a patient's motor neurons. In the play Three Damn Letters, a sarcastic woman must accept her own ALS diagnosis while also balancing her relationships with her father, husband, and son. This seriocomedic play explores themes of family roles, disability, love, and death.

Cross-Sectional Survey Of Relevant Literatures As To The Current Proposed Disease Mechanisms And Treatments Of Amyotrophic Lateral Sclerosis (Als), Zachary Sanford

Marshall Journal of Medicine

Amyotrophic Lateral Sclerosis (ALS), more commonly referred to as Lou Gehrig’s disease, is a progressively degenerative neuromuscular disorder affecting both the upper and lower motor neurons and preferentially affecting males in their forties to seventies. Although the pathology of ALS has been clearly elucidated elsewhere, the precise mechanisms by which the disease progresses and the means by which it is acquired are still poorly understood areas of medicine. Current genomic and proteomic studies in human and animal models have yielded exciting and promising new findings that may help elucidate this pathology. It is the purpose of this review article to …

Stem Cells As A Cure For Amyotrophic Lateral Sclerosis, Chaya K. Hirsch

The Science Journal of the Lander College of Arts and Sciences

Amyotrophic Lateral Sclerosis (ALS) is a fatal motor neuron disease which affects approximately 30,000 Americans at any given time (alsa.org, 2010). The etiology of this terminal disease unfortunately remains an unsolved mystery and has therefore severely limited the ability to find a cure. The use of stem cells to regenerate neurons has been vastly studied and have produced very promising results. However, its practicality as a cure or treatment for neurodegenerative diseases, such as ALS, is greatly compromised. Three different therapies involving stem cells were examined, Embryonic Stem Cells (ESC), induced pluripotent stem cells, (iPSC) and direct reprogramming of adult …