Skeletal and smooth muscle Flashcards
Individuals with prolonged immobilization experience atrophy of what type of muscle fibers?
Atrophy of type I fibers (slow twitch) and reduced diamter of type II fibers
At the same time capillary density in the immobilized muscles will decline
What detects the length of stretch or shortening in muscle?
Muscle spindles detect length of stretch (or shortening) as well as the velocity of contraction
Golgi tendon organs
Sense the force generated by a muscle gaugin the tension developed in muscle tension
X-linked muscular dystrophy
Duchenne’s muscular dystrophy
Skeletal muscle is replaced by adipose tissue
X-linked recessive - affects boys>girls
LOF mutation - deletion of gene that codes for dystrophin (largest gene in the human genome - highest risk of error)
Dystrophin- cell membrane protein that allows for interaction of the muscle fiber with extracellular matrix.
Disease is progressive, from proximal skeletal muscle outward toward the distal muscles
Presents around 1 y/o - affects quadriceps first (difficulty walking)
Total paryalysis by second decade of life - death occurs as result of myocardial infiltration
Gower’s sign
The unusual way in which a child w/ x-linked muscular dystrophy will use a series of hand pushes to rise from the ground - unable to rely on hip flexors or shoulder muscles
Becker’s muscular dystrophy
Same disease as Duchenne’s except there’s a mutation of the dystrophin gene rather than a deletion
Because there is some protein as opposed to none - disease is often milder
Myasthenia gravis
Autoimmune disorder caused by antibodies against postsynaptic nicotinic ACh receptors - binding of Ab’s competively inhibits ACh from binding
Decaying force of contraction on rapidly repeated contractions
Small muscles and muscles that are used a lot are affected most
Pt’s will c/o blurry bision, dysphagia, and difficulty writing
Pt’s with MG can sometimes have a hyperproliferation of mediastinal thymic tissue (thymom) - removal can reverse sxs of MG
Myasthenia gravis vs. Lamber-Eaton syndrome
Diagram
Lambert-Eaton Myasthenic Syndrome
More than 1/2 cases are associated w/ small cell lung cancer - Tx’ing the cancer helps resolve the sxs
Due to antibodies against voltage-gated calcium channels on presynaptic nerve terminals leading to a decrease in ateylcholine release
W/ successive presynaptic action potneitlas that temporally sum, ACh will be released and contraction will occur. Increasing frequency of contraction increases the strength of contraction - muscles not often used are first affected
myotonias
Are conditions characterized by the impairment of muscle relaxation following voluntary contraction or electrical stimkulation.
One sign is the inability to loosen a handgrip after lifting a heavy object or after a handshake
Myotonic dystrophy 1
Results from synthesis of a defective RNA that sequesters RNA binding proteins causing dysregulated RNA splicing. This leads to down regulation of a voltage-gated Cl channel (ClC1) involved in stabilizing muscle cell membrane potentials
Fx loss of this ClC1 leads to myotonia
Congenital myotonia
Results from a defect in the CLCN1 gene - encodes for ClC1 protein
TN fainting goats - result from a mutation in CLCN1
Malignant hyperthermia (MH)
Hereditary disorder of skeletal muscle - hypermetabolic response to halogenated anesthetic gasses and/or depolarizing muscle relaxant succinylcholine
Mutation in the ryanodine receptor results in uncontrolled release of calcium from skeletal muscle SR leading to sustained muscle contraction.
Dantrolene inhibits the ryanodine receptor calcium release channel in skeletal muscle without affecting nueromuscular transmission and is effective for both prophylaxis and tx of active MH
Myofilament composition
Myosin 2 and actin - make up the contractile unit (sarcomere)
Myosin vs actin on ESM
Myosin - appear darker than actin on an electron micrograph
Areas of overlap between myosin and actin appear darkest of all
Zones/bands of the sarcomere
During contraction which lines of the sarcomere get closer together?
The Z lines get closer together
During muscle contraction, what organelle propagates the action potential?
The T-tubule
Depolarization activates and opens the gate of T-tubule voltage-gated L-type calcium channels - do not allow for sufficient calcium movement to cause contraction (because they arent in the sarcoplasmic reticulum)
T-tubule L-type Ca2+ channels open what other channels?
They physically open the sarcoplasmic-reticulum-ryanodine-Ca2+ channels - this calcium influx is sufficient to induce contraction
Troponin I
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Physically in the way covering the myosin-binding site on actin
Troponin T (TnT)
Pushes tropomyosin aside
There is optimal overlap between the actin and myosin filaments