Ultrastructural studies on the acetylcholine receptor at motor end plates of normal and pathologic muscles. 1976

C W Porter, and E A Barnard

The acetylcholine receptors at the mammalian motor end plate have been counted and their distribution there determined ultrastructurally. Electron microscope autoradiography applied to muscles labeled with alpha-(3H)bungarotoxin was used for this purpose. The receptors are distributed asymmetrically along the postsynaptic membrane, being concentrated at the fold crests-that portion nearest to the presynaptic membrane. The density of receptor sites at that region is estimated to be 20,000-25,000 per mum2 of membrane surface. This density holds for the several species and muscle types thus far examined and appears to be a constant parameter of the motor end plate. It determines the limit of responsiveness to acetylcholine. By contrast, the enzyme acetylcholinesterase is found to be distributed evenly over the folds and may reside in the intersynaptic matrix. When mouse diaphragm end plates from dystrophic animals or animals following 5-day denervation were similarly examined, no significant alterations in either the density or the distribution of the receptor sites were found. Similarly, dystrophic muscles in chickens possess an unaltered number of receptors at their end plates. A model is outlined to correlate receptor and cholinesterase concentrations with known aspects of transmitter release. These findings may have relevance to some of the electrophysiological abnormalities seen in myasthenia gravis.

UI MeSH Term Description Entries
D009045 Motor Endplate The specialized postsynaptic region of a muscle cell. The motor endplate is immediately across the synaptic cleft from the presynaptic axon terminal. Among its anatomical specializations are junctional folds which harbor a high density of cholinergic receptors. Motor End-Plate,End-Plate, Motor,End-Plates, Motor,Endplate, Motor,Endplates, Motor,Motor End Plate,Motor End-Plates,Motor Endplates
D009121 Muscle Denervation The resection or removal of the innervation of a muscle or muscle tissue. Denervation, Muscle,Denervations, Muscle,Muscle Denervations
D009132 Muscles Contractile tissue that produces movement in animals. Muscle Tissue,Muscle,Muscle Tissues,Tissue, Muscle,Tissues, Muscle
D009136 Muscular Dystrophies A heterogeneous group of inherited MYOPATHIES, characterized by wasting and weakness of the SKELETAL MUSCLE. They are categorized by the sites of MUSCLE WEAKNESS; AGE OF ONSET; and INHERITANCE PATTERNS. Muscular Dystrophy,Myodystrophica,Myodystrophy,Dystrophies, Muscular,Dystrophy, Muscular,Myodystrophicas,Myodystrophies
D009157 Myasthenia Gravis A disorder of neuromuscular transmission characterized by fatigable weakness of cranial and skeletal muscles with elevated titers of ACETYLCHOLINE RECEPTORS or muscle-specific receptor tyrosine kinase (MuSK) autoantibodies. Clinical manifestations may include ocular muscle weakness (fluctuating, asymmetric, external ophthalmoplegia; diplopia; ptosis; and weakness of eye closure) and extraocular fatigable weakness of facial, bulbar, respiratory, and proximal limb muscles. The disease may remain limited to the ocular muscles (ocular myasthenia). THYMOMA is commonly associated with this condition. Anti-MuSK Myasthenia Gravis,MuSK MG,MuSK Myasthenia Gravis,Muscle-Specific Receptor Tyrosine Kinase Myasthenia Gravis,Muscle-Specific Tyrosine Kinase Antibody Positive Myasthenia Gravis,Myasthenia Gravis, Generalized,Myasthenia Gravis, Ocular,Anti MuSK Myasthenia Gravis,Generalized Myasthenia Gravis,Muscle Specific Receptor Tyrosine Kinase Myasthenia Gravis,Muscle Specific Tyrosine Kinase Antibody Positive Myasthenia Gravis,Myasthenia Gravis, Anti-MuSK,Myasthenia Gravis, MuSK,Ocular Myasthenia Gravis
D009469 Neuromuscular Junction The synapse between a neuron and a muscle. Myoneural Junction,Nerve-Muscle Preparation,Junction, Myoneural,Junction, Neuromuscular,Junctions, Myoneural,Junctions, Neuromuscular,Myoneural Junctions,Nerve Muscle Preparation,Nerve-Muscle Preparations,Neuromuscular Junctions,Preparation, Nerve-Muscle,Preparations, Nerve-Muscle
D011955 Receptors, Drug Proteins that bind specific drugs with high affinity and trigger intracellular changes influencing the behavior of cells. Drug receptors are generally thought to be receptors for some endogenous substance not otherwise specified. Drug Receptors,Drug Receptor,Receptor, Drug
D002038 Bungarotoxins Neurotoxic proteins from the venom of the banded or Formosan krait (Bungarus multicinctus, an elapid snake). alpha-Bungarotoxin blocks nicotinic acetylcholine receptors and has been used to isolate and study them; beta- and gamma-bungarotoxins act presynaptically causing acetylcholine release and depletion. Both alpha and beta forms have been characterized, the alpha being similar to the large, long or Type II neurotoxins from other elapid venoms. alpha-Bungarotoxin,beta-Bungarotoxin,kappa-Bungarotoxin,alpha Bungarotoxin,beta Bungarotoxin,kappa Bungarotoxin
D002802 Cholinesterases Acylcholineacylhydrolase,Cholase,Cholinesterase
D006651 Histocytochemistry Study of intracellular distribution of chemicals, reaction sites, enzymes, etc., by means of staining reactions, radioactive isotope uptake, selective metal distribution in electron microscopy, or other methods. Cytochemistry

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