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This discussion delves into the structural components of muscle fibers, including the sarcomere, its proteins, and their functions, focusing on titin, actinin, desmin, and the dystrophin-glycoprotein complex. Learn about the physiological anatomy of muscle and the clinical significance in muscular dystrophies.
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NERVE MUSCLE PHYSIOLOGY Dr FarzanaSalman
OBJECTIVES • At the end of this discussion you should be able to: • 1.Know the structural components of a muscle fiber • 2. Differentiate striated muscles & correlate it with structural components. • 3. Know the major types & functions of Proteins present in a muscle fiber
Types of Muscles SimilaritiesDifferences • Skeletal. Excitability. . Structure Contractility . Location • Cardiac. Elasticity. . Function Activation • Smooth. . Contraction
Physiological anatomy of MUSCLE Muscle Fascicle
Physiological anatomy of MUSCLE
SARCOMERE “Structural and functional Unit of the Muscle” Mainly Comprised of: Actin Filaments Arranged between two Z lines Myosin Filaments Besides these ?
SARCOPLASM • Spaces between the myofibrils are filled with intracellular fluid called sarcoplasm. • Potassium • Magnesium • Phosphate • Protein enzymes • Mitochondria
Sarcomere (Supportive Proteins) 1. Titin, 2. Actinin, 3. Desmin & 4. Dystrophin-Glycoprotein complex
SARCOMERE Proteins • Titin: MW= 3 million Largest springy protein Provides scaffolding for the sarcomere Connects Z line to M line • Actinin : MW=190,000 Binds actin to Z line • Desmin: Binds Z line to plasma membrane
Dystrophin-Glycoprotein Complex • Large Protein, MW = 427000 • Forms a Rod which connects the Actin filament to the transmembrane Protein Beta-Dystroglycan in the sarcolema. • Function: Adds strength to the muscle by providing a Scaffolding for the fibrils & connecting them to the Extra cellular Matrix
Dystrophin-Glycoprotein Complex Merosin
CLINICAL SIGNIFICANCE • Musculardystrophies. Diseases that cause progressive weakness of skeletal muscle 1) Duchenne muscular dystrophy. 2) Becker muscular dystrophy.