N lines in a myopathy with myosin loss. 1998

G Lopate, and A Pestronk, and W C Yee
Washington University Department of Neurology, St. Louis, Missouri 63110, USA.

N lines can be seen by electron microscopy within the I band of skeletal muscle, but are poorly visualized in conventional preparations. We present a case of acute quadriplegic myopathy with myosin loss and prominent N lines. The only other reported cases of N lines were also seen in patients with myosin loss from diverse etiologies. Myosin loss and the subsequent detachment of titin from myosin may result in the formation of prominent N lines.

UI MeSH Term Description Entries
D007366 Intercostal Muscles Respiratory muscles that arise from the lower border of one rib and insert into the upper border of the adjoining rib, and contract during inspiration or respiration. (From Stedman, 25th ed) Intercostal Muscle,Muscle, Intercostal,Muscles, Intercostal
D008297 Male Males
D008854 Microscopy, Electron Microscopy using an electron beam, instead of light, to visualize the sample, thereby allowing much greater magnification. The interactions of ELECTRONS with specimens are used to provide information about the fine structure of that specimen. In TRANSMISSION ELECTRON MICROSCOPY the reactions of the electrons that are transmitted through the specimen are imaged. In SCANNING ELECTRON MICROSCOPY an electron beam falls at a non-normal angle on the specimen and the image is derived from the reactions occurring above the plane of the specimen. Electron Microscopy
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009135 Muscular Diseases Acquired, familial, and congenital disorders of SKELETAL MUSCLE and SMOOTH MUSCLE. Muscle Disorders,Myopathies,Myopathic Conditions,Muscle Disorder,Muscular Disease,Myopathic Condition,Myopathy
D009218 Myosins A diverse superfamily of proteins that function as translocating proteins. They share the common characteristics of being able to bind ACTINS and hydrolyze MgATP. Myosins generally consist of heavy chains which are involved in locomotion, and light chains which are involved in regulation. Within the structure of myosin heavy chain are three domains: the head, the neck and the tail. The head region of the heavy chain contains the actin binding domain and MgATPase domain which provides energy for locomotion. The neck region is involved in binding the light-chains. The tail region provides the anchoring point that maintains the position of the heavy chain. The superfamily of myosins is organized into structural classes based upon the type and arrangement of the subunits they contain. Myosin ATPase,ATPase, Actin-Activated,ATPase, Actomyosin,ATPase, Myosin,Actin-Activated ATPase,Actomyosin ATPase,Actomyosin Adenosinetriphosphatase,Adenosine Triphosphatase, Myosin,Adenosinetriphosphatase, Actomyosin,Adenosinetriphosphatase, Myosin,Myosin,Myosin Adenosinetriphosphatase,ATPase, Actin Activated,Actin Activated ATPase,Myosin Adenosine Triphosphatase
D009460 Neurologic Examination Assessment of sensory and motor responses and reflexes that is used to determine impairment of the nervous system. Examination, Neurologic,Neurological Examination,Examination, Neurological,Examinations, Neurologic,Examinations, Neurological,Neurologic Examinations,Neurological Examinations
D011782 Quadriplegia Severe or complete loss of motor function in all four limbs which may result from BRAIN DISEASES; SPINAL CORD DISEASES; PERIPHERAL NERVOUS SYSTEM DISEASES; NEUROMUSCULAR DISEASES; or rarely MUSCULAR DISEASES. The locked-in syndrome is characterized by quadriplegia in combination with cranial muscle paralysis. Consciousness is spared and the only retained voluntary motor activity may be limited eye movements. This condition is usually caused by a lesion in the upper BRAIN STEM which injures the descending cortico-spinal and cortico-bulbar tracts. Quadriparesis,Spastic Quadriplegia,Tetraplegia,Flaccid Quadriplegia,Flaccid Tetraplegia,Paralysis, Spinal, Quadriplegic,Spastic Tetraplegia,Flaccid Quadriplegias,Flaccid Tetraplegias,Quadripareses,Quadriplegia, Flaccid,Quadriplegia, Spastic,Quadriplegias,Quadriplegias, Flaccid,Quadriplegias, Spastic,Spastic Quadriplegias,Spastic Tetraplegias,Tetraplegia, Flaccid,Tetraplegia, Spastic,Tetraplegias,Tetraplegias, Flaccid,Tetraplegias, Spastic
D004568 Electrodiagnosis Diagnosis of disease states by recording the spontaneous electrical activity of tissues or organs or by the response to stimulation of electrically excitable tissue. Electrodiagnoses
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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