Biomaterial Database

Excitation-Contraction Coupling Alterations in Myopathies. 2016

Isabelle Marty, and Julien Fauré

University Grenoble Alpes, Grenoble Institut des Neurosciences, GIN, F-38000 Grenoble, France.

During the complex series of events leading to muscle contraction, the initial electric signal coming from motor neurons is transformed into an increase in calcium concentration that triggers sliding of myofibrils. This process, referred to as excitation-contraction coupling, is reliant upon the calcium-release complex, which is restricted spatially to a sub-compartment of muscle cells ("the triad") and regulated precisely. Any dysfunction in the calcium-release complex leads to muscle impairment and myopathy. Various causes can lead to alterations in excitation-contraction coupling and to muscle diseases. The latter are reviewed and classified into four categories: (i) mutation in a protein of the calcium-release complex; (ii) alteration in triad structure; (iii) modification of regulation of channels; (iv) modification in calcium stores within the muscle. Current knowledge of the pathophysiologic mechanisms in each category is described and discussed.

UI	MeSH Term	Description	Entries
D009046	Motor Neurons	Neurons which activate MUSCLE CELLS.	Neurons, Motor,Alpha Motorneurons,Motoneurons,Motor Neurons, Alpha,Neurons, Alpha Motor,Alpha Motor Neuron,Alpha Motor Neurons,Alpha Motorneuron,Motoneuron,Motor Neuron,Motor Neuron, Alpha,Motorneuron, Alpha,Motorneurons, Alpha,Neuron, Alpha Motor,Neuron, Motor
D009119	Muscle Contraction	A process leading to shortening and/or development of tension in muscle tissue. Muscle contraction occurs by a sliding filament mechanism whereby actin filaments slide inward among the myosin filaments.	Inotropism,Muscular Contraction,Contraction, Muscle,Contraction, Muscular,Contractions, Muscle,Contractions, Muscular,Inotropisms,Muscle Contractions,Muscular Contractions
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
D009210	Myofibrils	The long cylindrical contractile organelles of STRIATED MUSCLE cells composed of ACTIN FILAMENTS; MYOSIN filaments; and other proteins organized in arrays of repeating units called SARCOMERES .	Myofilaments,Myofibril,Myofilament
D002118	Calcium	A basic element found in nearly all tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes.	Coagulation Factor IV,Factor IV,Blood Coagulation Factor IV,Calcium-40,Calcium 40,Factor IV, Coagulation
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012519	Sarcoplasmic Reticulum	A network of tubules and sacs in the cytoplasm of SKELETAL MUSCLE FIBERS that assist with muscle contraction and relaxation by releasing and storing calcium ions.	Reticulum, Sarcoplasmic,Reticulums, Sarcoplasmic,Sarcoplasmic Reticulums
D053498	Sarcoplasmic Reticulum Calcium-Transporting ATPases	Calcium-transporting ATPases that catalyze the active transport of CALCIUM into the SARCOPLASMIC RETICULUM vesicles from the CYTOPLASM. They are primarily found in MUSCLE CELLS and play a role in the relaxation of MUSCLES.	Calcium-Transporting ATPases, Sarcoplasmic Reticulum,Sarcoplasmic Reticulum Calcium ATPase,SERCA Calcium ATPase,SERCA1 Calcium ATPase,SERCA2 Calcium ATPase,SERCA2a Calcium ATPase,SERCA3 Calcium ATPase,SR Ca(2+)-ATPase 1,SR Ca(2+)-ATPase 2,SR Ca(2+)-ATPase 3,Sarco-Endoplasmic Reticulum Ca2+-ATPase,Sarcoplasmic Reticulum Ca(2+)-ATPase,Sarcoplasmic Reticulum Calcium-Transporting ATPase 1,Sarcoplasmic Reticulum Calcium-Transporting ATPase 2,Sarcoplasmic Reticulum Calcium-Transporting ATPase 2a,Sarcoplasmic Reticulum Calcium-Transporting ATPase 3,Sarcoplasmic-Endoplasmic Reticulum Calcium ATPase 2,Sarcoplasmic-Endoplasmic Reticulum Calcium ATPase 2a,Sarcoplasmic-Endoplasmic Reticulum Calcium ATPase 3,Sarcoplasmic-endoplasmic Reticulum Calcium ATPase 1,Ca2+-ATPase, Sarco-Endoplasmic Reticulum,Calcium Transporting ATPases, Sarcoplasmic Reticulum,Reticulum Ca2+-ATPase, Sarco-Endoplasmic,Sarco Endoplasmic Reticulum Ca2+ ATPase,Sarcoplasmic Endoplasmic Reticulum Calcium ATPase 2,Sarcoplasmic Endoplasmic Reticulum Calcium ATPase 2a,Sarcoplasmic Endoplasmic Reticulum Calcium ATPase 3,Sarcoplasmic Reticulum Calcium Transporting ATPase 1,Sarcoplasmic Reticulum Calcium Transporting ATPase 2,Sarcoplasmic Reticulum Calcium Transporting ATPase 2a,Sarcoplasmic Reticulum Calcium Transporting ATPase 3,Sarcoplasmic Reticulum Calcium Transporting ATPases,Sarcoplasmic endoplasmic Reticulum Calcium ATPase 1
D056966	Excitation Contraction Coupling	A process fundamental to muscle physiology whereby an electrical stimulus or action potential triggers a myocyte to depolarize and contract. This mechanical muscle contraction response is regulated by entry of calcium ions into the cell.	Contraction Coupling, Excitation,Contraction Couplings, Excitation,Coupling, Excitation Contraction,Couplings, Excitation Contraction,Excitation Contraction Couplings
D018482	Muscle, Skeletal	A subtype of striated muscle, attached by TENDONS to the SKELETON. Skeletal muscles are innervated and their movement can be consciously controlled. They are also called voluntary muscles.	Anterior Tibial Muscle,Gastrocnemius Muscle,Muscle, Voluntary,Plantaris Muscle,Skeletal Muscle,Soleus Muscle,Muscle, Anterior Tibial,Muscle, Gastrocnemius,Muscle, Plantaris,Muscle, Soleus,Muscles, Skeletal,Muscles, Voluntary,Skeletal Muscles,Tibial Muscle, Anterior,Voluntary Muscle,Voluntary Muscles