Normal muscle pyruvate oxidation in spinocerebellar degenerations

1981 ◽  
Vol 9 (1) ◽  
pp. 93-94 ◽  
Author(s):  
Owen B. Evans
Keyword(s):  
Normal Muscle ◽  
Pyruvate Oxidation ◽  
Spinocerebellar Degenerations

scholarly journals POS0832 A NOVEL GREY SCALE AND POWER DOPPLER ULTRASONOGRAPHIC SCORE FOR IDIOPATHIC INFLAMMATORY MYOPATHIES: SIENA MYOSITIS ULTRASOUND GRADING SCALE

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 669.1-670
Author(s):  
E. Conticini ◽  
P. Falsetti ◽  
S. G. Al Khayyat ◽  
C. Baldi ◽  
F. Bellisai ◽  
...  
Keyword(s):  
Disease Duration ◽  
Muscle Fibers ◽  
Power Doppler ◽  
Muscle Thickness ◽  
Rectus Femoris ◽  
Normal Muscle ◽  
Small Vessels ◽  
Grey Scale ◽  
Vastus Intermedius ◽  
Grading Scale

Background:No clear-cut guidelines exist about the use of diagnostic procedures for idiopathic inflammatory myopathies (IIM) and only scanty and conflicting data report the use of ultrasound (US).Objectives:We aimed to assess if grey-scale (GS) and Power Doppler (PD) US, graded with a 0-3-points-scale, may be a reliable tool in a cohort of patients affected by IIM.Methods:We prospectively collected, since July to October 2020, all patients referred to Vasculitis and Myositis clinic, Rheumatology Unit, University of Siena, for suspected IIM, as well as patients with a previous, definite diagnosis of IIM and evaluated during follow-up or referred from other centers for a second opinion. All patients underwent US examination of both thighs in axial and longitudinal scans. Edema and atrophy, both assessed in GS, and PD, were graded with a 0-3-points-scale. Spearman test was used to identify the correlations between US and clinical and serological variables.Results:A total of 18 patients was included. Four of them were evaluated twice, at baseline and within 3 months of therapy. Muscle edema was found to be directly correlated with physician global assessment (PhGA), serum myoglobin and PD and negatively with disease duration. PD score was positively correlated to PhGA and negatively to disease duration. Muscle atrophy directly correlated with Myositis Damage Index and patients’ age. The single-thigh sub-analysis evidenced a direct correlation between PD score and Manual Muscle Test.Conclusion:In our cohort, we found that edema and PD are strictly related to early, active myositis, suggesting that an inflamed muscle should appear swollen, thickened and with Doppler signal. Conversely, muscle atrophy reflects the age of the patient and the overall severity of the disease. Such findings shed a new, promising, light in the role of US in diagnosis and monitoring of IIMs.Table 1.Siena Myositis Ultrasound Grading Scale (SMUGS).Grey-scale edemaGrey-scale atrophyPower Doppler0Normal muscle echotexture with hyperechoic septa and hypoechoic muscle fibers, conserved thickness.Normal muscle echotexture, with hyperechoic septa and hypoechoic muscle fibers, conserved thickness.No PD signal.1Focal hypoechoic areas, where septa are less evident. Conserved thickness.Focal heterogeneously hyperechoic areas, where septa are thicker and more evident, and muscle fibers are thinner. Conserved muscle thickness.One or two PD signals in at least one muscle (PD vascular spots, small vessels of homogenous diameters, vessel diameters approximately not superior to fibrous intramuscular septa)2Diffuse and heterogeneous hypo echogenicity (rectus femoris as hypoechoic or more than vastus intermedius), septa diffusely less evident. Conserved thickness.Diffuse and heterogeneously hyperechoic muscle, with thicker septa and thinner muscle fibers. Conserved muscle thickness.More than 2 PD signals for each muscle (as vascular spots, small vessels of homogenous diameters, vessel diameters approximately not superior to fibrous intramuscular septa).3Diffuse and heterogeneous hypo echogenicity (rectus femoris as hypoechoic or more than vastus intermedius), septa diffusely less evident. Increased thickness (rectus femoris became thicker than vastus intermedius).Diffuse and heterogeneously hyperechoic muscle, with thicker septa and thinner muscle fibers. Reduced muscle thickness.More than 2 PD signals for each muscle with larger diameter of the vessel (at least superior to fibrous intramuscular septa), or vessels with different diameters or branched vessels.Figure 1.Different PD findings (clockwise) in longitudinal anterior scans of the thigh: PD 3 in a patient with a recent diagnosis of anti-Mi2 DM; PD 2 in the same patient after one month of treatment with steroids and Methotrexate; PD 1 in a patient affected by anti-SAE DM, with a suspected disease flare; PD 0 in a patient affected by an advanced polymyositis diagnosed in 2000, currently not in treatment.Disclosure of Interests:None declared

Enzyme studies of skeletal muscle in mice with hereditary muscular dystrophy

1963 ◽  
Vol 205 (5) ◽  
pp. 897-901 ◽  
Author(s):  
Marilyn W. McCaman
Keyword(s):  
Skeletal Muscle ◽  
Muscular Dystrophy ◽  
Glutathione Reductase ◽  
Enzyme Activities ◽  
Lactic Dehydrogenase ◽  
Normal Muscle ◽  
Dystrophic Muscle ◽  
Nerve Section ◽  
Mouse Muscle ◽  
Dystrophic Mouse

The activities of 20 enzymes in normal, heterozygous, and dystrophic mouse muscle were studied by means of quantitative microchemical methods. Enzyme activities in normal and heterozygous muscle were essentially the same. In dystrophic muscle glucose-6-P dehydrogenase, 6-P-gluconic dehydrogenase, glutathione reductase, peptidase, ß-glucuronidase, and glucokinase activities were significantly higher than in normal muscle, while α-glycero-P dehydrogenase and lactic dehydrogenase activities were significantly lower. The pattern of enzyme activities found in normal gastrocnemius denervated by nerve section was strikingly similar to that in dystrophic muscle.

scholarly journals INHIBITORY ACTION OF meso-TARTRATE ON PYRUVATE OXIDATION

1955 ◽  
Vol 214 (1) ◽  
pp. 245-250
Author(s):  
J.H. Quastel ◽  
P.G. Scholefield
Keyword(s):  
Inhibitory Action ◽  
Pyruvate Oxidation

scholarly journals E4F1 controls a transcriptional program essential for pyruvate dehydrogenase activity

2016 ◽  
Vol 113 (39) ◽  
pp. 10998-11003 ◽  
Author(s):  
Matthieu Lacroix ◽  
Geneviève Rodier ◽  
Olivier Kirsh ◽  
Thibault Houles ◽  
Hélène Delpech ◽  
...  
Keyword(s):  
Pyruvate Dehydrogenase ◽  
Energy Demand ◽  
Clinical Symptoms ◽  
Tricarboxylic Acid Cycle ◽  
Striated Muscles ◽  
Pyruvate Oxidation ◽  
Mitochondrial Pyruvate Carrier ◽  
Lactic Acidemia ◽  
Transcription Factor 1 ◽  
Dihydrolipoyl Dehydrogenase

The mitochondrial pyruvate dehydrogenase (PDH) complex (PDC) acts as a central metabolic node that mediates pyruvate oxidation and fuels the tricarboxylic acid cycle to meet energy demand. Here, we reveal another level of regulation of the pyruvate oxidation pathway in mammals implicating the E4 transcription factor 1 (E4F1). E4F1 controls a set of four genes [dihydrolipoamide acetlytransferase (Dlat), dihydrolipoyl dehydrogenase (Dld), mitochondrial pyruvate carrier 1 (Mpc1), and solute carrier family 25 member 19 (Slc25a19)] involved in pyruvate oxidation and reported to be individually mutated in human metabolic syndromes. E4F1 dysfunction results in 80% decrease of PDH activity and alterations of pyruvate metabolism. Genetic inactivation of murine E4f1 in striated muscles results in viable animals that show low muscle PDH activity, severe endurance defects, and chronic lactic acidemia, recapitulating some clinical symptoms described in PDC-deficient patients. These phenotypes were attenuated by pharmacological stimulation of PDH or by a ketogenic diet, two treatments used for PDH deficiencies. Taken together, these data identify E4F1 as a master regulator of the PDC.

scholarly journals Normal muscle regeneration requires tight control of muscle cell fusion by tetraspanins CD9 and CD81

2013 ◽  
Vol 4 (1) ◽  
Author(s):  
Stéphanie Charrin ◽  
Mathilde Latil ◽  
Sabrina Soave ◽  
Anna Polesskaya ◽  
Fabrice Chrétien ◽  
...  
Keyword(s):  
Cell Fusion ◽  
Muscle Cell ◽  
Muscle Regeneration ◽  
Tight Control ◽  
Normal Muscle

Sensory Reinnervation of Muscles Following Nerve Section and Suture in Cats

1985 ◽  
Vol 10 (3) ◽  
pp. 340-344
Author(s):  
R. W. BANKS ◽  
D. BARKER ◽  
H. G. BROWN
Keyword(s):  
Common Peroneal Nerve ◽  
Muscle Spindles ◽  
Sensory Impairment ◽  
Normal Muscle ◽  
Axon Terminals ◽  
Reflex Action ◽  
Stretch Receptors ◽  
The Common ◽  
Sensory Reinnervation ◽  
Tendon Organ

The common peroneal nerve was transected and repaired by epineurial suture in nine cats. In a further nine the nerve was transected twice and similarly repaired so as to produce a short autograft. Recovery of stretch receptors in peroneus brevis was monitored histologically and physiologically from six to fifty weeks. In recovery after single neurotomy functionally identifiable muscle-spindle and tendon-organ afferents were reduced to 25% and 45% of normal, respectively; after double neurotomy (autograft) both were reduced to about 10% of normal. Muscle spindles were reinnervated with annulospiral terminals, or wholly abnormal fine axon terminals, or both. Recovery evidently entails not only a reduction in number of stretch afferents, but also the making of some incorrect reconnections that presumably result in abnormal proprioceptive feedback and reflex action. When a graft is used the sensory impairment is compounded.

Neuronotrophic effects of skeletal muscle fractions on spinal cord differentiation

Development ◽  
1982 ◽  
Vol 71 (1) ◽  
pp. 83-95
Author(s):  
L. Hsu ◽  
D. Natyzak ◽  
G. L. Trupin
Keyword(s):  
Skeletal Muscle ◽  
Spinal Cord ◽  
Neuronal Migration ◽  
Prolonged Treatment ◽  
Embryonic Chick ◽  
Neuronal Responses ◽  
Normal Muscle ◽  
Glial Development ◽  
Independent Effects ◽  
Chick Spinal Cord

Soluble fractions of homogenized skeletal muscle were found to promote neuronal migration and neuritic and glial outgrowth from embryonic chick spinal cord explants. Fractions obtained from skeletal muscle immobilized by prolonged treatment with curare were significantly more effective than normal muscle in accelerating neuronal and glial development. Fractions from other tissues such as brain and lung did not enhance neuronal differentiation, but were effective in stimulating outgrowth of glial cells. Separate measurements of glial and neuronal responses indicate that tissue fractions produce independent effects on the glial and neuronal components.

Do muscles function as adaptable locomotor springs?

2002 ◽  
Vol 205 (15) ◽  
pp. 2211-2216 ◽  
Author(s):  
Stan L. Lindstedt ◽  
Trude E. Reich ◽  
Paul Keim ◽  
Paul C. LaStayo
Keyword(s):  
Skeletal Muscle ◽  
Elastic Strain ◽  
Strain Energy ◽  
Elastic Strain Energy ◽  
Normal Animal ◽  
Eccentric Contractions ◽  
Normal Muscle ◽  
Energetic Costs ◽  
Locomotor Muscles ◽  
Power Outputs

SUMMARYDuring normal animal movements, the forces produced by the locomotor muscles may be greater than, equal to or less than the forces acting on those muscles, the consequences of which significantly affect both the maximum force produced and the energy consumed by the muscles. Lengthening (eccentric)contractions result in the greatest muscle forces at the lowest relative energetic costs. Eccentric contractions play a key role in storing elastic strain energy which, when recovered in subsequent contractions, has been shown to result in enhanced force, work or power outputs. We present data that support the concept that this ability of muscle to store and recover elastic strain energy is an adaptable property of skeletal muscle. Further, we speculate that a crucial element in that muscle spring may be the protein titin. It too seems to adapt to muscle use, and its stiffness seems to be`tuned' to the frequency of normal muscle use.

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