Publications by authors named "John M Mislow"

How the activity of populations of cortical neurons generates coordinated multijoint actions of the arm, wrist, and hand is poorly understood. This study combined multielectrode recording techniques with full arm motion capture to relate neural activity in primary motor cortex (M1) of macaques (Macaca mulatta) to arm, wrist, and hand postures during movement. We find that the firing rate of individual M1 neurons is typically modulated by the kinematics of multiple joints and that small, local ensembles of M1 neurons contain sufficient information to reconstruct 25 measured joint angles (representing an estimated 10 functionally independent degrees of freedom).

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Neurosurgical diagnosis and intervention has evolved through improved neuroimaging, allowing better visualization of anatomy and pathology. This article discusses the various systems that have been designed over the last decade to meet the requirements of neurosurgical patients and opines on the potential future developments in the technology and application of intraoperative MRI. Because the greatest amount of experience with intraoperative MRI comes from its use in brain tumor resection, this article focuses on the origins of intraoperative MRI in relation to this field.

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Background. In today's fast-paced and high-acuity emergency departments, clinicians are often compelled to triage cases so rapidly that a differential diagnosis consistent with the history and physical examination is not comprehensive. Case Report.

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Neurosurgical diagnosis and intervention has evolved through improved neuroimaging, allowing better visualization of anatomy and pathology. This article discusses the various systems that have been designed over the last decade to meet the requirements of neurosurgical patients and opines on the potential future developments in the technology and application of intraoperative MRI. Because the greatest amount of experience with intraoperative MRI comes from its use in brain tumor resection, this article focuses on the origins of intraoperative MRI in relation to this field.

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Intervertebral disk herniation in pediatric patients is a rare but potentially disabling entity that is frequently difficult to diagnose. This article reviews the fundamentals of pediatric intervertebral disk herniation with the intention of presenting a rational and simple strategy for the evaluation and treatment of disk herniation in children, with specific emphasis on how it differs from adult disk disease in presentation, pathologic findings, and treatment options.

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Calvarial osteolysis is a relatively rare finding in patients with neurofibromatosis. The authors describe two patients with neurofibromatosis Type 1 (NF1) and extensive cranial defects associated with underlying dural ectasia. Cranioplasties were performed in both patients with mixed results.

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Muscle A-kinase anchoring protein (mAKAP) is a scaffold protein found principally at the nuclear envelope of striated myocytes. mAKAP maintains a complex consisting of multiple signal transduction molecules including the cAMP-dependent protein kinase A, the ryanodine receptor calcium release channel, phosphodiesterase type 4D3, and protein phosphatase 2A. By an unknown mechanism, a domain containing spectrin repeats is responsible for targeting mAKAP to the nuclear envelope.

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Mutations in the LMNA gene encoding nuclear lamins A and C are responsible for seven inherited disorders affecting specific tissues. We have analyzed skin fibroblasts from a patient with type 1B limb-girdle muscular dystrophy and from her deceased newborn grandchild carrying, respectively, a heterozygous (+/mut) and a homozygous (mut/mut) nonsense Y259X mutation. In fibroblasts(+/mut), the presence of only 50% lamins A and C promotes no detectable abnormality, whereas in fibroblasts(mut/mut) the complete absence of lamins A and C leads to abnormally shaped nuclei with lobules in which none of the analyzed nuclear proteins were detected, i.

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Genetic studies of cardiomyopathy and muscular dystrophy have emphasized the importance of the striated myocyte cytoskeleton. Cytoskeletal defects produce myopathies through a combination of structural and signaling mechanisms. Broadly, the cytoskeletal proteins defective in these myopathic syndromes can be classified into categories based on their intracellular locations.

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Nesprin-1alpha is a spectrin repeat (SR)-containing, transmembrane protein of the inner nuclear membrane, and is highly expressed in muscle cells. A yeast two-hybrid screen for nesprin-1alpha-interacting proteins showed that nesprin-1alpha interacted with itself. Blot overlay experiments revealed that nesprin-1alpha's third SR binds the fifth SR.

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Mutations in the genes encoding the inner nuclear membrane proteins lamin A/C and emerin produce cardiomyopathy and muscular dystrophy in humans and mice. The mechanism by which these broadly expressed gene products result in tissue-specific dysfunction is not known. We have identified a protein of the inner nuclear membrane that is highly expressed in striated and smooth muscle.

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