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Displaying 1 to 9 of 9 results for ALS

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  • ALS Center

    The ALS Center for Cell Therapy and Regeneration Research at Johns Hopkins is committed to identifying the causes of the neurodegenerative disease, amyotrophic lateral sclerosis (ALS), and discovering new and effective treatment options. At the ALS Center, Johns Hopkins researchers work with other investigators, including those at the Robert Packard Center for ALS Research at Johns Hopkins and clinicians within the Johns Hopkins ALS Clinic to aggressively take groundbreaking scientific discoveries and turn them into clinical applications that will improve the quality of life of those diagnosed with ALS.

    Research Areas: ALS

    Lab Website

    Principal Investigator

    Nicholas Maragakis, M.D.

    Department

    Neurology

  • Brown Lab

    The Brown Lab is focused on the function of the cerebral cortex in the brain, which underlies our ability to interact with our environment through sensory perception and voluntary movement. Our research takes a bottom-up approach to understanding how the circuits of this massively interconnected network of neurons are functionally organized, and how dysfunction in these circuits contributes to neurodegenerative diseases like amyotrophic lateral sclerosis and neuropsychiatric disorders, including autism and schizophrenia. By combining electrophysiological and optogenetic approaches with anatomical and genetic techniques for identifying cell populations and pathways, the Brown Lab is defining the synaptic interactions among different classes of cortical neurons and determining how long-range and local inputs are integrated within cortical circuits. In amyotrophic lateral sclerosis, corticospinal and spinal motor neurons progressively degenerate. The Brown Lab is examining how abnormal ...activity within cortical circuits contributes to the selective degeneration of corticospinal motor neurons in an effort to identify new mechanisms for treating this disease. Abnormalities in the organization of cortical circuits and synapses have been identified in genetic and anatomical studies of neuropsychiatric disease. We are interested in the impact these abnormalities have on cortical processing and their contribution to the disordered cognition typical of autism and schizophrenia. view more

    Research Areas: autism, neurodegenerative diseases, brain, electrophysiology, ALS, schizophrenia, cerebral cortex, optogenetics

    Lab Website

    Principal Investigator

    Solange Brown, M.D., Ph.D.

    Department

    Neuroscience

  • Charles Wiener Lab

    The Charles Wiener Lab primarily conducts research on pulmonary circulation and hypoxia as well as respiratory muscle function in patients with neuromuscular diseases. Our recent studies have included investigating the treatment of pericardial effusions in patients with pulmonary arterial hypertension and examining the use of non-invasive ventilation in patients with amyotrophic lateral sclerosis (ALS). We also have an interest in medical education research. Our work in this area has included reviewing the role of academic medical centers in emerging health care markets.

    Research Areas: respiratory system, neuromuscular diseases, ALS, pulmonary medicine, hypertension, hypoxia

    Principal Investigator

    Charles Wiener, M.D.

    Department

    Medicine

  • Dwight Bergles Laboratory

    The Bergles Laboratory studies synaptic physiology, with an emphasis on glutamate transporters and glial involvement in neuronal signaling. We are interested in understanding the mechanisms by which neurons and glial cells interact to support normal communication in the nervous system. The lab studies glutamate transport physiology and function. Because glutamate transporters play a critical role in glutamate homeostasis, understanding the transporters' function is relevant to numerous neurological ailments, including stroke, epilepsy, and neurodegenerative diseases like amyotrophic lateral sclerosis (ALS). Other research in the laboratory focuses on signaling between neurons and glial cells at synapses. Understanding how neurons and cells communicate, may lead to new approaches for stimulating re-myelination following injury or disease. Additional research in the lab examines how a unique form of glia-to-neuron signaling in the cochlea influences auditory system development, whethe...r defects in cell communication lead to certain hereditary forms of hearing impairment, and if similar mechanisms are related to sound-induced tinnitus. view more

    Research Areas: epilepsy, synaptic physiology, ALS, stroke, neuronal signaling, glutamate transport physiology and function, audiology, neuroscience, neurology, nervous system, molecular biology

    Lab Website

    Principal Investigator

    Dwight Bergles, Ph.D.

    Department

    Neuroscience

  • Kata Design Studio

    We started Kata to bridge the gap between professional experiential production and neuroscience, clinical neurology, and medical hardware. We strive to build experiences and technology from the ground up, with a focus on mission, and at a level that is consistent with the best productions in the industry. We mirror the thousands of hours that go into a level design in a video game, but with the crucial difference that the focus is on the subtleties required for patient treatment or wellness. Our designs require high-frequency iterative development with patients and users in countless game-play sessions in which they provide crucial feedback. Characters have been painstakingly crafted to elicit profound emotional responses. Some of the requirements for patients or the elderly population in this space are qualitatively different from what is needed in the entertainment marketplace. That said we have also understood the critical artistic similarities.

    The core ethos of Kata is that the... challenge of complex movement has profound benefits for cognition, wellness, and brain repair. Specifically, there is growing evidence that complex motor movement can have cognitive benefits that go beyond what has been reported for exercise alone. When designing experiences to treat motor impairments after stroke, maximizing rigorous and dynamic motor input is a requirement. New interactive technologies will allow people to engage in diverse and complex motor movements, even in the home, which was previously impossible.

    Overall it has been a very exciting journey, combining art, medicine, technology, and neuroscience. We continue to build, discover, and craft immersive experiences, side by side with physicians, physical therapists, and scientists, with the common goal of pushing clinical care and wellness forward. We believe this is only possible by having a mission focused design group embedded in an academic hospital. Ultimately, we wish to scale and perfect these innovations into other hospitals. Kata is a true hybrid of academia, and industry, doing what neither can do in isolation. We hope the ethos and design philosophy behind Kata provides the impetus for its expansion, partnerships, and growth.
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    Research Areas: ALS, stroke, vestibular disorders

    Lab Website

    Principal Investigator

    John Krakauer, M.A., M.D.

    Department

    Neurology

  • Lee Martin Laboratory

    In the Lee Martin Laboratory, we are testing the hypothesis that selective vulnerability--the phenomenon in which only certain groups of neurons degenerate in adult onset neurological disorders like amyotrophic lateral sclerosis and Alzheimer's disease--is dictated by brain regional connectivity, mitochondrial function and oxidative stress. We believe it is mediated by excitotoxic cell death resulting from abnormalities in excitatory glutamatergic signal transduction pathways, including glutamate transporters and glutamate receptors as well as their downstream intracellular signaling molecules.

    We are also investigating the contribution of neuronal/glial apoptosis and necrosis as cell death pathways in animal (including transgenic mice) models of acute and progressive neurodegeneration. We use a variety of anatomical and molecular neurobiological approaches, including neuronal tract-tracing techniques, immunocytochemistry, immunoblotting, antipeptide antibody production, transmissi...on electron microscopy and DNA analysis to determine the precise regional and cellular vulnerabilities and the synaptic and molecular mechanisms that result in selective neuronal degeneration.
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    Research Areas: ALS, neurodegeneration, selective vulnerability, cell death, Alzheimer's disease

    Lab Website

    Principal Investigator

    Lee Martin, Ph.D.

    Department

    Pathology

  • Philip Wong Lab

    The Philip Wong Lab seeks to understand the molecular mechanisms and identification of new therapeutic targets of neurodegenerative diseases, particularly Alzheimer's disease (AD) and amyotrophic lateral sclerosis (ALS). Taking advantage of discoveries of genes linked to these diseases (mutant APP and PS in familial AD and mutant SOD1, dynactin p150glued ALS4and ALS2 in familial ALS), our laboratory is taking a molecular/cellular approach, including transgenic, gene targeting and RNAi strategies in mice, to develop models that facilitate our understanding of pathogenesis of disease and the identification and validation of novel targets for mechanism-based therapeutics. Significantly, these mouse models are instrumental for study of disease mechanisms, as well as for design and testing of therapeutic strategies for AD and ALS.

    Research Areas: neurodegenerative disorders, ALS, genomics, pathogenesis, Alzheimer's disease

    Lab Website

    Principal Investigator

    Philip Wong, Ph.D.

    Department

    Pathology

  • Rong Li Lab

    Research in the Rong Li Lab aims to better understand the fundamental laws that regulate the behavior and interactions of cellular systems. Our team is currently examining how cells consolidate their damaged proteins and prevent them from spreading freely — work aimed at understanding how to better treat diseases such as Alzheimer’s and ALS. We are also applying insights gained through basic research to better understand diseases such as cancer and polycystic kidney disease.

    Research Areas: cell biology, ALS, kidney diseases, cancer, cellular dynamics, molecular biology, Alzheimer's disease

    Lab Website

    Principal Investigator

    Rong Li, M.S., Ph.D.

    Department

    Cell Biology

  • The Sun Laboratory

    The nervous system has extremely complex RNA processing regulation. Dysfunction of RNA metabolism has emerged to play crucial roles in multiple neurological diseases. Mutations and pathologies of several RNA-binding proteins are found to be associated with neurodegeneration in both amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). An alternative RNA-mediated toxicity arises from microsatellite repeat instability in the human genome. The expanded repeat-containing RNAs could potentially induce neuron toxicity by disrupting protein and RNA homeostasis through various mechanisms.

    The Sun Lab is interested in deciphering the RNA processing pathways altered by the ALS-causative mutants to uncover the mechanisms of toxicity and molecular basis of cell type-selective vulnerability. Another major focus of the group is to identify small molecule and genetic inhibitors of neuron toxic factors using various high-throughput screening platforms. Finally, we are also highly i...nterested in developing novel CRISPR technique-based therapeutic strategies. We seek to translate the mechanistic findings at molecular level to therapeutic target development to advance treatment options against neurodegenerative diseases. view more

    Research Areas: ALS, neurodegeneration, RNA

    Lab Website

    Principal Investigator

    Shuying Sun, Ph.D.

    Department

    Pathology

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