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PubMed1977

Experimental definition of independent myocutaneous vascular territories.

Experimental studies were undertaken in dogs to determine whether useful island myocutaneous flaps could be based on the gracilis, sartorius, biceps femoris, trapezius, or rectus abdominis muscles. Dissection and injection studies on these muscles were also undertaken in human cadavers to determine the contributions of these muscles to the blood supply of the overlying skin. In most instances it was considerable. The use of island myocutaneous flaps seems promising in many situations. Such transfers can be done in one operation, without delay procedures, and result usually in a better blood supply with the transfer of a thicker amount of tissue. Clinical research on such flaps in patients will be described in a subsequent paper.

Animals↗
PubMed1996

[Transplantation of the remedial free anteromedial thigh flap].

Over one hundred and twenty free anterolateral thigh flaps have been performed since 1988, of which ten were operated as free anteromedial flaps because the nutrient vessels emerged from the medial intermuscular space formed by the sartorius, the rectus femoris and vastus medialis muscles. Clinical study indicated that there were three types of nutrient cutaneous arteries in the space: type 1, the medial branch of the descending branch of the lateral circumflex femoral artery in 6 cases; type 2, the superficial femoris artery in 2 cases; and type 3, the branch of the femoris artery in 2 cases. The name of the flap, the operation technique and the compensative characteristics of the arteries are discussed.

Adolescent↗
PubMed2001

Myofiber injury and regeneration in a canine homologue of Duchenne muscular dystrophy.

OBJECTIVE: To test the hypothesis that differential skeletal muscle involvement, previously observed in dogs with a homologue of Duchenne muscular dystrophy, correlates with the histochemical markers of myofiber injury and regeneration. DESIGN: Evidence of injury (cellular penetration by Evans blue dye, immunoglobulin G expression, hematoxylin and eosin staining of necrotic figures), myofiber regeneration (fetal myosin heavy chain isoform expression), and morphologic indices in the cranial sartorius (CS), long digital extensor, and vastus lateralis muscles were examined in five dogs with dystrophy and five normal dogs. RESULTS: Only the CS muscle, at 1 mo, demonstrated significant differences in injury when compared with age-matched controls. By 6 mo, the long digital extensor and vastus lateralis also suffered greater than normal injury. Only the dystrophic CS tissue expressed a notable increase in mean myofiber diameter when compared with other muscles at 6 mo. Normal CS muscles revealed a distinct population of small myofibers at this age. CONCLUSION: The CS seems unique in its selective pathologic involvement. These differences may contribute to the marked regenerative response of this muscle in the dystrophic state. An improved understanding of mechanisms by which some dystrophin-deficient canine muscles remain spared from injury may provide clues to investigate and prevent the degenerative processes in humans.

Animals↗
PubMed1984

Heterogeneity of T-tubule geometry in vertebrate skeletal muscle fibres.

Average dimensions of transverse tubules were obtained from electron micrographs of thin sections of mammalian and amphibian skeletal muscle fibres and the effect of transverse tubule geometry on the electrical characteristics of the fibres has been considered. The preparations examined were toad sartorius, mouse soleus, rat extensor digitorum longus, soleus and sternomastoid muscles. The T-tubule dimensions varied considerably between the different preparations and the average volume to surface ratio of the transverse tubule in amphibian fibres (8.1 nm) was generally greater than that in mammalian fibres (3.0-6.2 nm). The small volume to surface ratio of the mammalian transverse tubule would tend to reduce the electrical space constant of the transverse tubule system and reduce the rate of cross-sectional activation of the fibres during a twitch contraction. The area of transverse tubule membrane in junctional contact with the sarcoplasmic reticulum was determined and was found to be greater in mammalian fibres than in amphibian fibres. The relative areas of junctional contact, along a unit length of transverse tubule, were the same in rat extensor digitorum longus and soleus fibres.

Animals↗
PubMed2004

Muscle MRI findings in patients with limb girdle muscular dystrophy with calpain 3 deficiency (LGMD2A) and early contractures.

Limb girdle muscular dystrophy 2A is a common variant secondary to mutations in the calpain 3 gene. A proportion of patients has early and severe contractures, which can cause diagnostic difficulties with other conditions. We report clinical and muscle magnetic resonance imaging findings in seven limb girdle muscular dystrophy 2A patients (four sporadic and three familial) who had prominent and early contractures. All patients showed a striking involvement of the posterior thigh muscles. The involvement of the other thigh muscles was variable and was related to clinical severity. Young patients with minimal functional motor impairment showed a predominant involvement of the adductors and semimembranosus muscles while patients with restricted ambulation had a more diffuse involvement of the posterolateral muscles of the thigh and of the vastus intermedius with relative sparing of the vastus lateralis, sartorius and gracilis. At calf level all patients showed involvement of the soleus muscle and of the medial head of the gastrocnemius with relative sparing of the lateral head. MRI findings were correlated to those found in two patients with the phenotype of limb girdle muscular dystrophy 2A without early contractures and the pattern observed was quite similar. However, the pattern observed in limb girdle muscular dystrophy 2A is different from that reported in other muscle diseases such as Emery-Dreifuss muscular dystrophy and Bethlem myopathy which have a significant clinical overlap with limb girdle muscular dystrophy 2A once early contractures are present. Our results suggest that muscle MRI may help in recognising patients with limb girdle muscular dystrophy 2A even when the clinical presentation overlaps with other conditions, and may therefore, be used as an additional investigation to target the appropriate biochemical and genetic tests.

Adolescent↗
PubMed2000

Afferent roles in hindlimb wipe-reflex trajectories: free-limb kinematics and motor patterns.

The hindlimb wiping reflex of the frog is an example of a targeted trajectory that is organized at the spinal level. In this paper, we examine this reflex in 45 spinal frogs to test the importance of proprioceptive afferents in trajectory formation at the spinal level. We tested hindlimb to hindlimb wiping, in which the wiping or effector limb and the target limb move together. Loss of afferent feedback from the wiping limb was produced by cutting dorsal roots 7-9. This caused altered initial trajectory direction, increased ankle path curvature, knee-joint velocity reversals, and overshooting misses of the target limb. We established that these kinematic and motor-pattern changes were due mainly to the loss of ipsilateral muscular and joint afferents. Loss of cutaneous afferents alone did not alter the initial trajectory up to target limb contact. However, there were cutaneous effects in later motor-pattern phases after the wiping and target limb had made contact: The knee extension or whisk phase of wiping was often lost. Finally, there was a minor and nonspecific excitatory effect of phasic contralateral feedback in the motor-pattern changes after deafferentation. Specific muscle groups were altered as a result of proprioceptive loss. These muscles also showed configuration-based regulation during wiping. Biceps, semitendinosus, and sartorius (all contributing knee flexor torques) all were regulated in amplitude based on the initial position of the limb. These muscles contributed to an initial electromyographic (EMG) burst in the motor pattern. Rectus internus and semimembranosus (contributing hip extensor torques) were regulated in onset but not in the time of peak EMG or in termination of EMG based on initial position. These two muscles contributed to a second EMG burst in the motor pattern. After deafferentation the initial burst was reduced and more synchronous with the second burst, and the second burst often was broadened in duration. Ankle path curvature and its degree of change after loss of proprioception depended on the degree of joint staggering used by the frog (i.e., the relative phasing between knee and hip motion) and on the degree of motor-pattern change. We examined these variations in 31 frogs. Twenty percent (6/31) of frogs showed largely synchronous joint coordination and little effect of deafferentation on joint coordination, end-point path, or the underlying synchronous motor pattern. Eighty percent of frogs (25/31) showed some degree of staggered joint coordination and also strong effects of loss of afferents. Loss of afferents caused two major joint level changes in these frogs: collapse of joint phasing into synchronous joint motion and increased hip velocity. Fifty percent of frogs (16/31) showed joint-coordination changes of type (1) without type (2). This change was associated with reduction, loss, or collapse of phasing of the sartorius, semitendinosus and biceps (iliofibularis) in the initial EMG burst in the motor pattern. The remaining 30% (9/31) of frogs showed both joint-coordination changes 1 and 2. These changes were associated with both the knee flexor EMG changes seen in the other frogs and with additional increased activity of rectus internus and semimembranosus muscles. Our data show that multiple ipsilateral modalities all play some role in regulating muscle activity patterns in the wiping limb. Our data support a strong role of ipsilateral proprioception in the process of trajectory formation and specifically in the control of limb segment interactions during wiping by way of the regulation and coordination of muscle groups based on initial limb configuration.

Algorithms↗
PubMed1992

Stimulation-enhanced 3-O-methylglucose efflux from the frog sartorius: kinetics and properties of the system.

The characteristics of the process by which contraction enhances glucose transport in the frog sartorius were studied. Electrical stimulation increased the permeability of muscles to 3-O-methylglucose (3-O-MeGlc), a nonmetabolizable glucose analogue, increasing efflux as well as uptake. Enhanced efflux was due to an increase in Vmax of the efflux process. A lactacidosis had no effect on basal 3-O-MeGlc efflux, and replacement of media Na+ with Li+ did not affect stimulation-induced uptake. Also, basal and stimulated uptake was not affected by 1 microM 12-O-tetradecanoylphorbol-13-acetate (TPA), a protein kinase C activator. Lastly, N-carbobenzoxy-glycyl-L-phenylalaninamide, which inhibits insulin-enhanced, but not basal, glucose uptake in adipocytes, inhibited both basal and stimulated 3-O-MeGlc fluxes in the frog sartorius. From these findings, we conclude: (1) contraction and exercise enhance glucose transport in muscle by increasing the number of transporters in the plasma membrane, or their turnover, by an unknown process; and (2) basal glucose transport of muscle, unlike that of adipocytes, can not be distinguished from stimulated transport on the basis of its insensitivity to N-carbobenzoxyglycyl-L-phenylalaninamide.

3-O-Methylglucose↗
PubMed1963

Spectrophotometric studies on intact muscle. I. Components of the respiratory chain.

The concentrations of the components of the respiratory chain were determined in a variety of intact skeletal muscles by a method of spectrophotometric observation of the transmitted light. In the case of the toad sartorius, these measurements were checked against isolated mitochondrial suspensions prepared from toad skeletal muscles. The relative concentrations of the respiratory components were found to be in reasonable agreement with those of various mitochondrial preparations of mammalian tissues and of the ones from toad skeletal muscle. The rather low cytochrome b and pyridine nucleotide levels in the anoxic minus oxygenated difference spectra were shown to be caused to a certain degree by a partial reduction during the resting steady state. Upon treatment with a strong, reducing agent or after long anoxia some absorption bands appeared with maxima at 591, 562 to 564, and 432 to 434 mmicro both in the intact and in the mitochondrial fractions of muscle tissue; they do not appear to be associated with the respiratory chain.

Animals↗
PubMed1982

Myocardial adaptation to stress from the viewpoint of evolution and development.

Myocardial hypertrophy, which results from the increase in demand placed on the heart, does not involve the simple enlargement of the myocardial cell. Instead, the cells are restructured in a manner that depends on the nature of the stress applied. The cellular reorganization involves the contractile, EC coupling, and recovery systems. In the remodeling of each of these systems, a repertoire of functional adaptation is used which bears a remarkable similarity to the changes seen in the differentiation of species and the development of muscle type. Thus, in pressure overload hypertrophy, where the maintenance of persistent high pressure in a necessity, the contractile system of the muscle is slower and more economical. This is similar to the specialization seen in the tortoise or in slow muscles like the rat soleus. In thyrotoxic hypertrophy, where the demand is to move the blood at high velocity, the muscle is fast and less economical. This is similar to the specialization seen in the frog sartorius or rat EDL. The restructuring involves specific molecular changes that uniquely adapt the muscles for the tasks at hand.

Adaptation, Physiological↗
PubMed1996

Respiratory distress and selective muscle involvement in central core disease: report of a case.

A 10-year-old girl who had suffered life-threatening respiratory distress in late infancy and mild muscle weakness in the extremities was found to have central core disease (CCD). Her developmental milestones were markedly delayed and she became ambulant at the age of 6 years. On physical examination, she had a high-arched palate, nasal tone vocalization, a positive Gowers' sign, waddling gait, and decreased deep tendon reflexes. On magnetic resonance imaging (MRI) examination, the thigh, calf, arm, and paravertebral muscles were selectively involved, especially in the paravertebral, biceps brachii, gluteus maximus and medius, vastus lateralis and medialis, sartorius, adductor magnus, tibialis anterior, soleus, lateral head of gastrocnemius, and peroneus longus muscles. Two biopsy specimens from the left biceps brachii and rectus femoris muscles showed the common histochemical findings of CCD, including type 1 fiber atrophy and predominance, and core structures. Proliferation of adipose tissue was only present in the biceps brachii muscle. The patient improved clinically in muscle strength after three year follow-up. Muscle MRI may provide a non-invasive evaluation of gross muscle involvement in CCD.

Arm↗
PubMed2026

INPP5K-related muscular dystrophy caused by a novel synonymous splicing variant in a Chinese patient: a case report.

Congenital muscular dystrophies (CMDs) are a genetically heterogeneous group of disorders. Variants in the INPP5K gene, which encodes a phosphoinositide phosphatase, are a rare cause of CMD. The condition is commonly associated with muscle weakness, early-onset cataracts, and intellectual disability, and prior reports have primarily identified missense, frameshift, or deletion variants. We describe the first Chinese case of INPP5K-related muscular dystrophy in a 28-year-old male with a mild phenotype, notably lacking intellectual disability. His presentation included bilateral cataracts at age 5 and adolescent onset limb girdle weakness. Muscle magnetic resonance imaging (MRI) revealed a characteristic pattern of selective fatty infiltration, with severe involvement of gluteal and thigh muscles and striking sparing of the rectus femoris, sartorius, and gracilis. Genetic analysis identified compound heterozygous novel INPP5K variants: a missense c.274C>T, p.(Arg92Cys) and a synonymous c.261G>A, p.(Lys87=) change. Functional studies confirmed the synonymous variant causes aberrant splicing (exon 3 skipping), leading to a frameshift and premature termination p.(Leu52SerfsTer49). According to American College of Medical Genetics and Genomics guidelines, the c.274C>T and c.261G>A variants were classified as likely pathogenic and pathogenic, respectively. This first report of a Chinese patient with INPP5K-related muscular dystrophy broadens both the genetic and clinical spectrum of the disorder. We identify the first disease-causing synonymous variant (via aberrant splicing) and a novel hypomorphic missense variant p.(Arg92Cys), the combination of which explains the attenuated phenotype lacking intellectual disability. Our case highlights the critical role of RNA analysis in diagnosing non-canonical variants and confirms the universal diagnostic relevance of the characteristic muscle MRI pattern.

Adult↗
PubMed1963

Stimulus frequency and neuromuscular block.

Muscle twitches of the rat isolated diaphragm and frog sartorius preparations were recorded. It was confirmed that, in the presence of tubocurarine, the degree of neuromuscular block was greater the higher the frequency of stimulation. The results suggest that the quantity of acetylcholine released by each nerve impulse was reduced by increasing the rate of stimulation so that, in a tubocurarine solution, muscle fibres which were only just being fired at a slow rate of stimulation failed to fire at a faster rate.

Acetylcholine↗
PubMed1984

A study of factors influencing muscle activity about the knee joint.

Several factors influencing the myoelectric activity of muscles surrounding the knee joint were studied using fine-wire monopolar electrodes. The muscles studied included the vastus lateralis, vastus intermedius, rectus femoris, vastus medialis, gracilis, sartorius, biceps femoris, semimembranosus, semitendinosus, tensor fasciae latae, medial head of the gastrocnemius, and lateral head of the gastrocnemius. Muscle activity was measured in response to unidirectional loads tending to flex and extend the knee, and to combined loads of flexion-adduction, flexion-abduction, extension-adduction, and extension-abduction. Results indicate that the individual muscle responses are dependent upon the direction, magnitude, and combination of external moments, as well as on the flexion angle of the knee joint. Muscle response appeared to be influenced by certain intrinsic mechanical characteristics of the knee joint that tend to change the moment arms of the muscles as the knee moves. For example, the substantial changes in quadriceps myoelectric activity with knee flexion, with constant load applied, can be related to the movement of the tibial-femoral contact changing the lever arm of the quadriceps mechanism. This study indicates that the mechanics of the knee joint must be taken into consideration while attempting to interpret or predict the load response of muscles crossing the knee joint.

Adult↗
PubMed1983

Indentations in the terminal cisternae of amphibian and mammalian skeletal muscle fibers.

Indentations (hillocks and dimples) in the terminal cisternae of mammalian and amphibian skeletal muscle fibers were studied using freeze-fracture and serial thin-section techniques. The structures were seen in all muscles and had a regular separation from each other and from the T-tubule. Indentations were smaller than fenestrations and formed concavities in, but not macromolecular pores through, the terminal cisternae. The average numbers of indentations in rat muscles (measured along the length of the terminal cisternae, within 150 nm of the triadic junction) varied from 0.9 per micrometer in soleus fibers to 9.6 per micrometer in posterior cricoarytenoid fibers. The average numbers in amphibian sartorius fibers varied from 1.6 to 3.6 per micrometer in muscles from different species. The regular alignment of the indentations along the triad, as well as a close correlation between their numbers and the contractile properties of the muscle, suggest that they function in contractile activation and may represent sites of calcium release from the terminal cisternae.

Animals↗
PubMed1997

Muscle activation at the human knee during isometric flexion-extension and varus-valgus loads.

We examined the role of muscles in counteracting static loads in the transverse plane at the knee to determine if (a) knee muscles are activated to counteract isometric varus or valgus loads, (b) muscle activity during varus and valgus loads changes with the angle of knee flexion, and (c) the direction of a muscle's activation can be predicted by its moment arm orientations. For seven subjects, muscle activity was recorded during isometric tasks using surface and intramuscular electrodes from 10 muscles that span the knee. A six-degree-of-freedom load cell was rigidly attached to each subject's lower leg just above the ankle, and the subjects were instructed to push against the load cell so as to produce moments in the flexion-extension-varus-valgus plane at the knee. Moments in this plane were all of equal magnitude and varied in direction the full 360 degrees in 20 degrees increments. Most muscles were not activated to stabilize the knee against varus-valgus loads, but the sartorius, gracilis, and tensor fasciae latae showed substantial electromyographic activation in these directions. The load directions where muscles were principally active were observed to be dependent on joint angle for some muscles. In particular, the principal directions of activation for these three muscles changed as the angle of knee flexion changed. Similarly, a muscle's moment arm orientation was a good predictor of direction of activation for some muscles and a poor one for others. These results suggest that different muscles may play different roles in providing joint stability and that these roles are complex functions of muscle moment arm orientations, joint angles, external load directions, and possibly other undetermined parameters.

Adult↗
PubMed1998

Mechanisms of spontaneous activity in the developing spinal cord and their relevance to locomotion.

The isolated lumbosacral cord of the chick embryo generates spontaneous episodes of rhythmic activity. Muscle nerve recordings show that the discharge of sartorius (flexor) and femorotibialis (extensor) motoneurons alternates even though the motoneurons are depolarized simultaneously during each cycle. The alternation occurs because sartorius motoneuron firing is shunted or voltage-clamped by its synaptic drive at the time of peak femorotibialis discharge. Ablation experiments have identified a region dorsomedial to the lateral motor column that may be required for the alternation of sartorius and femorotibialis motoneurons. This region overlaps the location of interneurons activated by ventral root stimulation. Wholecell recordings from interneurons receiving short latency ventral root input indicate that they fire at an appropriate time to contribute to the cyclical pause in firing of sartorius motoneurons. Spontaneous activity was modeled by the interaction of three variables: network activity and two activity-dependent forms of network depression. A "slow" depression which regulates the occurrence of episodes and a "fast" depression that controls cycling during an episode. The model successfully predicts several aspects of spinal network behavior including spontaneous rhythmic activity and the recovery of network activity following blockade of excitatory synaptic transmission.

Animals↗
PubMed1981

Cell death of motoneurons in the chick embryo spinal cord. V. Evidence on the role of cell death and neuromuscular function in the formation of specific peripheral connections.

Previous reports from this laboratory have shown that the chronic treatment of chick embryos with neuromuscular blocking agents (e.g., curare, alpha-bungarotoxin), during the period of naturally occurring cell death of spinal motoneurons (days 5 to 10), greatly reduces the amount of cell death in this system. The surviving motoneurons continue differentiation and innervate the peripheral musculature. Since cell death has been prevented in these preparations from the earliest stages of limb innervation on day 4 or 5, it was expected that any inappropriate synaptic connections present at that time, or formed later, would be retained as long as the cell and their axons were prevented from regressing. To test this possibility, small injections of horseradish peroxidase were made into specific leg and wing muscles on embryonic day 10 in order to label retrogradely motoneuron pools in the spinal cord. The location of labeled motoneurons was found to be the same in control and experimental embryos. The specific muscles examined included the gastrocnemius, peroneus, adductor, and sartorius in the leg and the biceps, triceps, extensor metacarpi radialis, and flexor carpi ulnaris in the wing. In virtually all cases, there was a greater number of labeled motoneurons in the experimental cases. Despite this difference, the location of motoneuron pools in the rostral-caudal and transverse planes were remarkably similar in control and experimental embryos. Thus, natural cell death in this system is not primarily designed to remove errors in synaptic connectivity. Since a normal pattern of neuromuscular connections was formed in the virtual absence of functional synaptic interactions between motoneurons and their targets, these data do not support the contention that function is involved in the developmental specificity of peripheral connectivity.

Animals↗
PubMed1979

The morphogenesis of the thigh of the mouse with special reference to tetrapod muscle homologies.

In order to provide an ontogenetic basis for the establishment of tetrapod muscle homologies and for the analysis of complex mammalian muscle states, a descriptive analysis of the morphogenesis of the thigh of Mus musculus has been made. The pattern and sequence of muscle cleavage and the migrations of individual muscle primordia are characterized from the eleventh day of gestation, when cleavage begins, through early neonatal stages. Observations on skeletal differentiation and lumbosacral plexus formation are also included. Thigh muscle morphogenesis is compared to that in the lizard, Lacerta, (Romer, '42) and the chick (Romer, '27) and homologies identified. An onogenetic basis for the definition of ancestral and derived muscle states is provided in muscles that are morphologically variable in mammals. These include the gluteus minimus, gracilis, adductor brevis and several hamstring muscles. Certain muscles that show variable innervation patterns in adult mammals, i.e., pectineus, quadratus and adductor magnus, typically develop from premuscle regions that separate muscle anlagen innervated by different nerves. Two muscle anlagen appear in the embryonic mouse thigh and then disappear late in prenatal or early postnatal development. Comparisons with other mammals, especially the marsupial, Marmosa, reveal that these muscles are phylogenetic vestiges that degenerate before maturity. A sartorius vestige is identifiable through the thirteenth day of gestation. A tenuissimus anlage is present until shortly after birth and is clearly innervated by a branch of the peroneal nerve.

Animals↗