scispace - formally typeset
Search or ask a question
Institution

Johns Hopkins University School of Medicine

HealthcareBaltimore, Maryland, United States
About: Johns Hopkins University School of Medicine is a healthcare organization based out in Baltimore, Maryland, United States. It is known for research contribution in the topics: Population & Cancer. The organization has 44277 authors who have published 79222 publications receiving 4788882 citations.


Papers
More filters
Journal ArticleDOI
TL;DR: It is concluded that excitatory amino acid metabolism is altered in patients with ALS and these changes may play a role in motor neuron loss in ALS.
Abstract: Recently, the excitatory amino acid neurotransmitter glutamate was implicated in the pathogenesis of a variety of chronic degenerative neurological diseases in humans and animals. This report describes abnormalities in excitatory amino acids in the central nervous system of 18 patients with amyotrophic lateral sclerosis (ALS). The concentration of the excitatory amino acids glutamate and aspartate in the cerebrospinal fluid were increased significantly (p less than 0.01) by 100 to 200% in patients with ALS. Similarly, the concentrations of the excitatory neuropeptide N-acetyl-aspartyl glutamate and its metabolite, N-acetyl-aspartate, were elevated twofold to threefold in the cerebrospinal fluid from the patients. There was no relationship between amino acid concentrations and duration of disease, clinical impairment, or patient age. In the ventral horns of the cervical region of the spinal cord, the level of N-acetyl-aspartyl glutamate and N-acetyl-aspartate was decreased by 60% (p less than 0.05) and 40% (p less than 0.05), respectively, in 8 patients with ALS. Choline acetyltransferase activity was also diminished by 35% in the ventral horn consistent with motor neuron loss. We conclude that excitatory amino acid metabolism is altered in patients with ALS. Based on neurodegenerative disease models, these changes may play a role in motor neuron loss in ALS.

653 citations

Journal ArticleDOI
TL;DR: It is shown that increased TGF-β activity leads to failed muscle regeneration in fibrillin-1–deficient mice and a similar therapeutic response in a dystrophin-deficient mouse model of Duchenne muscular dystrophy.
Abstract: Skeletal muscle has the ability to achieve rapid repair in response to injury or disease Many individuals with Marfan syndrome (MFS), caused by a deficiency of extracellular fibrillin-1, exhibit myopathy and often are unable to increase muscle mass despite physical exercise Evidence suggests that selected manifestations of MFS reflect excessive signaling by transforming growth factor (TGF)-beta (refs 2,3) TGF-beta is a known inhibitor of terminal differentiation of cultured myoblasts; however, the functional contribution of TGF-beta signaling to disease pathogenesis in various inherited myopathic states in vivo remains unknown Here we show that increased TGF-beta activity leads to failed muscle regeneration in fibrillin-1-deficient mice Systemic antagonism of TGF-beta through administration of TGF-beta-neutralizing antibody or the angiotensin II type 1 receptor blocker losartan normalizes muscle architecture, repair and function in vivo Moreover, we show TGF-beta-induced failure of muscle regeneration and a similar therapeutic response in a dystrophin-deficient mouse model of Duchenne muscular dystrophy

653 citations

Journal ArticleDOI
TL;DR: Data from this large series substantiate that autoantibody-associated CHB is not coincident with major structural abnormalities, is most often identified in the late second trimester, carries a substantial mortality in the neonatal period and frequently requires pacing.

652 citations

Journal ArticleDOI
TL;DR: Given the importance of assessment and evaluation for CBME, the medical education community will need more collaborative research to address several major challenges in assessment, including “best practices” in the context of systems and institutional culture and how to best to train faculty to be better evaluators.
Abstract: Competency-based medical education (CBME), by definition, necessitates a robust and multifaceted assessment system. Assessment and the judgments or evaluations that arise from it are important at the level of the trainee, the program, and the public. When designing an assessment system for CBME, medical education leaders must attend to the context of the multiple settings where clinical training occurs. CBME further requires assessment processes that are more continuous and frequent, criterion-based, developmental, work-based where possible, use assessment methods and tools that meet minimum requirements for quality, use both quantitative and qualitative measures and methods, and involve the wisdom of group process in making judgments about trainee progress. Like all changes in medical education, CBME is a work in progress. Given the importance of assessment and evaluation for CBME, the medical education community will need more collaborative research to address several major challenges in assessment, including ‘‘best practices’’ in the context of systems and institutional culture and how to best to train faculty to be better evaluators. Finally, we must remember that expertise, not competence, is the ultimate goal. CBME does not end with graduation from a training program, but should represent a career that includes ongoing assessment.

652 citations

Journal ArticleDOI
01 Oct 1998-Neuron
TL;DR: Observations support a model in which the dynamic expression of Homer 1a competes with constitutively expressed CC-Homers to modify synaptic mGluR properties.

652 citations


Authors

Showing all 44754 results

NameH-indexPapersCitations
Robert Langer2812324326306
Bert Vogelstein247757332094
Solomon H. Snyder2321222200444
Steven A. Rosenberg2181204199262
Kenneth W. Kinzler215640243944
Hagop M. Kantarjian2043708210208
Mark P. Mattson200980138033
Stuart H. Orkin186715112182
Paul G. Richardson1831533155912
Aaron R. Folsom1811118134044
Gonçalo R. Abecasis179595230323
Jie Zhang1784857221720
Daniel R. Weinberger177879128450
David Baker1731226109377
Eliezer Masliah170982127818
Network Information
Related Institutions (5)
University of California, San Francisco
186.2K papers, 12M citations

99% related

Baylor College of Medicine
94.8K papers, 5M citations

99% related

University of Texas Southwestern Medical Center
75.2K papers, 4.4M citations

98% related

National Institutes of Health
297.8K papers, 21.3M citations

98% related

University of Alabama at Birmingham
86.7K papers, 3.9M citations

97% related

Performance
Metrics
No. of papers from the Institution in previous years
YearPapers
2023149
2022622
20216,078
20205,107
20194,444
20183,848