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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.
The goal of the Johns Hopkins Alzheimer's Disease Research Center (ADRC) is to accelerate the discovery of new treatments that are directed at the basic mechanisms of disease, and to hasten the time when effective treatments for AD and related disorders become a reality. We have a strong commitment to basic research regarding the underlying mechanisms of Alzheimer's Disease and related disorders, and how this may translate into effective treatment. We perform clinical research seeking to identify medications to delay or treat the symptoms of dementia. We also provided many educational programs for family members and professionals.
Mitochondrial dysfunction has long been a consistent observation in Parkinson's disease. To understand the consequences of Parkinson's disease causing genetic mutations on the function of mitochondria, the Bioenergetics Core B will provide the following analyses to the projects in the Udall Center at Johns Hopkins: (1) Measuring rates of respiration, oxygen consumption and ATP generation, (2) Measuring calcium dynamics, (3) Measuring reactive oxygen and reactive nitrogen species, (4) Measuring the activity of the electron transport chain enzymes and metabolic enzymes, and (5) Measuring plasma versus mitochondrial membrane potential and mitochondrial membrane permeability
The goal of the Johns Hopkins Brain Cancer Biology and Therapy Laboratory is to locate the genetic and genomic changes that lead to brain cancer. These molecular changes are evaluated for their potential as therapeutic targets and are often mutated genes, or genes that are over-expressed during the development of a brain cancer. The brain cancers that the Riggins Laboratory studies are medulloblastomas and glioblastomas. Medulloblastomas are the most common malignant brain tumor for children and glioblastomas are the most common malignant brain tumor for adults. Both tumors are difficult to treat, and new therapies are urgently needed for these cancers. Our laboratory uses large-scale genomic approaches to locate and analyze the genes that are mutated during brain cancer development. The technologies we now employ are capable of searching nearly all of a cancer genome for molecular alterations that can lead to cancer. The new molecular targets for cancer therapy are first located by large scale gene expression analysis, whole-genome scans for altered gene copy number and high throughput sequence analysis of cancer genomes. The alterations we find are then studied in-depth to determine how they contribute to the development of cancer, whether it is promoting tumor growth, enhancing the ability for the cancer to invade into normal tissue, or preventing the various fail-safe mechanisms programmed into our cells.
Our laboratory investigates the neural bases of sound processing in the human brain. We combine electrophysiology recordings (intracranial, scalp), behavioral paradigms, and statistical modeling methods to study the cortical dynamics of normal and impaired auditory perception. We are interested in measuring and modeling variability in spatiotemporal cortical response patterns as a function of individual listening abilities and acoustic sound properties. Current studies are investigating the role of high-frequency (>30 Hz) neural oscillations in human auditory perception.
Gabsang Lee Lab
Human induced pluripotent stem cells (hiPSCs) provide unprecedented opportunities for cell replacement approaches, disease modeling and drug discovery in a patient-specific manner. The Gabsang Lee Lab focuses on the neural crest lineage and skeletal muscle tissue, in terms of their fate-determination processes as well as relevant genetic disorders.
Previously, we studied a human genetic disorder (familial dysautonomia, or FD) with hiPSCs and found that FD-specific neural crest cells have low levels of genes needed to make autonomous neurons--the ones needed for the "fight-or-flight" response. In an effort to discover novel drugs, we performed high-throughput screening with a compound library using FD patient-derived neural crest cells.
We recently established a direct conversion methodology, turning patient fibroblasts into "induced neural crest (iNC)" that also exhibit disease-related phenotypes, just as the FD-hiPSC-derived neural crest. We're extending our research to the neural crest's neighboring cells, somite. Using multiple genetic reporter systems, we identified sufficient cues for directing hiPSCs into somite stage, followed by skeletal muscle lineages. This novel approach can straightforwardly apply to muscular dystrophies, resulting in expandable myoblasts in a patient-specific manner.
The Johns Hopkins NIMH Center is comprised of an interdisciplinary research team who has pooled their talents to study the nature of HIV-associated neurocognitive disorders (HAND). Their aim is to translate discoveries of the pathophysiological mechanisms into novel therapeutics for HAND.Our objectives are to integrate aspects of ongoing research in HAND and SIV encephalitis; to develop high-throughput and screening assays for identifying novel therapeutic compounds; to use proteomics and lipidomics approaches to indentifying surrogate markers of disease activity; to disseminate information and education about HAND through existing and new educational systems, including the JHU AIDS Education Training Center and the JHU Center for Global Clinical Education and to facilitate the entry of new investigators into neuro-AIDS research, and to catalyze new areas of research, particularly where relevant for drug discovery or the development of validated surrogate markers.
Research in the Laboratory of Auditory Neurophysiology aims to understand brain mechanism responsible for auditory perception and vocal communication in a naturalistic environment. We are interested in revealing neural mechanisms operating in the cerebral cortex and how cortical representations of biologically important sounds emerge through development and learning.
We use a combination of state-of-the-art neurophysiological techniques and sophisticated computational and engineering tools to tackle our research questions.
Current research in our laboratory includes the following areas (1) neural basis of auditory perception, (2) neural mechanisms for vocal communication and social interaction, and (3) cortical processing of cochlear implant stimulation.
The Laboratory of Richard L. Huganir is interested in the mechanisms that regulate synaptic transmission and synaptic plasticity. Our general approach is to study molecular and cellular mechanisms that regulate neurotransmitter receptors and synapse function. We are currently focusing our efforts on the mechanisms that underlie the regulation of the glutamate receptors, the major excitatory neurotransmitter receptors in the brain.
Laboratory of Vestibular NeuroAdaptation
The Laboratory of Vestibular NeuroAdaptation investigates mechanisms of gaze stability in people with loss of vestibular sensation. A bulk of our research investigates motor learning in the vestibulo-ocular reflex (VOR) using different types of error signals. In addition, we investigate the synergistic relationship between the vestibular and saccadic oculomotor systems as trainable strategies for gaze stability. We are particularly interested in developing novel technologies to assess and deliver improved rehabilitation outcomes. We are validating a hand-held computer tablet for assessment of sensorimotor function and participating in a clinical trial comparing traditional vestibular rehabilitation against a device developed in our laboratory that can unilaterally or bilaterally strengthen the VOR.
Members of the lab include physical therapists, physicians, engineers, statisticians and post-doctoral fellows. The laboratory is supported by generous grant funding from NASA, the NIH, the DOD and grateful patients
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