Clinical semiology of neuromuscular diseases (2).
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Hand↗
Biomedical subjects
G Serratrice
Publications and source records attributed to G Serratrice.
Clinical semiology of neuromuscular diseases (hand muscles).
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Distal Myopathies↗
Synthesis and iron-binding properties of quinolobactin, a siderophore from a pyoverdine-deficient Pseudomonas fluorescens.
Quinolobactin is a new siderophore produced by a pyoverdine deficient mutant of Pseudomonas fluorescens. A simple and efficient synthesis of quinolobactin is described, starting from xanthurenic acid. The protonation constants of quinolobactin were determined by potentiometric titrations as pKa2 = 5.50+/-0.07, pKa1 = 10.30+/-0.05. The equilibria of the metal complexes were studied by means of spectrophotometric and potentiometric titrations. The overall stability constants of the quinolobactin-FeIII complexes was found to be log beta111 = 18.60+/-0.10, log beta121 = 32.60+/-0.20, log beta120 = 28.20+/-0.25 resulting in a pFeIII value of 18.2 at pH 7.4. The UV-visible spectral parameters of the [FeL2] are in agreement with a complex containing two ligands coordinated to one Fe3+ cation through the oxygen and nitrogen quinoline atoms.
Gene Expression Regulation, Bacterial↗
[Attentional deficits].
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Attention Deficit Disorder with Hyperactivity↗
Neuromuscular diseases associated with antigliadin antibodies. A contentious concept.
Many neurological syndromes associated with antigliadin antibodies have been published. The most frequent are cerebellar ataxia and peripheral neuropathy. Such cases are reported under the title of gluten ataxia or of coeliac neuropathy. However, associations do not prove cause. Herein, a short review is made of the literature and the author's personal experience. Many unsolved questions remain. Gluten sensitivity is probably an immunological response in genetically susceptible individuals with or without enteropathy. Antigliadin antibodies are present in healthy adults, in some idiopathic ataxias and neuropathies. A gluten-free diet is effective on gastrointestinal but not on neurological symptoms. Whether antigliadin antibodies are elevated is more of a waiting classification than a definite diagnosis. Finally, the relationship between antigliadin antibodies and neurological diseases is very weak.
Antibodies↗
Hydroxyquinolines as iron chelators.
The interest in synthetic siderophore mimics includes therapeutic applications (iron chelation therapy), the design of more effective agents to deliver Fe to plants and the development of new chemical tools for studies of iron metabolism and for a better understanding of iron assimilation processes in living systems. The 8-hydroxyquinoline bidentate chelate moiety offers an alternative to the usual hydroxamic acid, catechol and/or alpha-hydroxycarboxylic acid metal-binding groups encountered in natural siderophores. The promising results obtained by the tris hydroxyquinoline-based ligand O-TRENSOX are summarized. O-TRENSOX exhibits a high and selective affinity for Fe(III) complexation. Its efficiency in delivering Fe to plants as well as its efficiency for iron mobilization, cellular protection and antiproliferative effects have been evidenced. Other chelators of the O-TRENSOX family (mixed catechol / 8-hydroxyquinoline ligands, lipophilic ligands) are also described. Some results question whether the use of partition coefficients is pertinent to foresee the activity of iron chelators. The development of probes (fluorescent, radioactive, spin labelled) based on the O-TRENSOX backbone is in progress. 8-hydroxyquinoline iron chelators seem to have a promising future.
Animals↗
[Exercise intolerance and muscular dystrophies].
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Energy Metabolism↗
New 8-hydroxyquinoline and catecholate iron chelators: influence of their partition coefficient on their biological activity.
Four new hexadendate chelators, three hydroxyquinoline-based, Csox, O-Trensox, Cox750, and one catecholate-based CacCam-which have comparable skeletal structures and pFe, but widely different partition coefficients, (Kpart), 0.01, 0.02, 1 and 3.2 respectively, have been tested for their iron chelating efficacy in vitro by two methods. First, by their ability to remove iron from ferritin in solution or second, to remove iron from iron-loaded hepatocytes in vitro. Our objective was to ascertain the importance of Kpart and pFe, on the biological efficiency of the molecule. Previous studies proposed that an ideal value of Kpart of 1 should give maximum biological activity. Mobilization of iron by Csox and CacCAM from ferritin was similar and furthermore more efficient than desferrioxamine B. In the iron-loaded hepatocyte cultures, the three hydroxyquinoline chelators, although showing diversity in terms of lipophilicity, appeared to be very similar in their capacity to chelate iron. CacCAM, the unique catecholate, was the most efficient of the molecules tested, as well as being the least toxic in the cellular model despite having the lowest value of pFe. In conclusion, the use of the partition coefficient and pFe, as tools for predicting biological activity of iron chelators should be not generalized. Further studies are required in order to understand the influence of the structure on the biological activity of the molecule.
Animals↗
[An unrecognized cause of camptocormia: proximal myotonic myopathy].
BACKGROUND: The underlying cause of camptocormia, a common condition in adults consisting of forward flexion of the trunk both in stance and gait, is often unrecognized, attributed by some to primary late-onset myopathy. CASE REPORTS: Two patients progressively developed camptocormia. The cause was identified as a relatively new disease, proximal myotonic myopathy. DISCUSSION: The diagnosis of proximal myotonic myopathy is based on clinical findings: myalgia and diffuse pain, gait disorders and progressive weakness of proximal muscles, electrical myotonia, cataracte in some cases, elevated gamma glutamyltranspeptidases. The disease is transmitted by autosomal dominant inheritance. This condition should be entertained as a possible cause in patients presenting an apparently primary camptocormic syndrome.
Aged↗
[Clinical and paraclinical investigations of chronic polyneuropathies].
Given the composition of the nerve fibre, the signs and symptoms of chronic polyneuropathy are not uniform. Clinically, they include sensory, motor and reflex signs and symptoms with, according to the case, association of sensory, either painful or not, motor and sensory-motor forms, with a reflex component. Electrophysiologic tests can usually distinguish initially axonal lesions from those due to initial myelin alteration. Nerve biopsy, although not a routine examination, brings precise information concerning etiology.
Chronic Disease↗
Partition coefficients (free ligands and their iron(III) complexes) and lipophilic behavior of new abiotic chelators. Correlation to biological activity.
Partition coefficients between n-octanol and water have been measured for ten tripodal ligands with catecholate or hydroxyquinolinate or pyridinophenolate chelating subunits and for their iron(III) complexes. The abilities of the ligands to cross an octanol phase and to extract ferric ion from its EDTA complex in an aqueous phase are studied. Correlation with biological properties are discussed.
Ferric Compounds↗
[Muscle contractures. Essay on a physiopathological approach to clarify the nomenclature].
There is a good deal of confusion in the international literature concerning the use of the term contracture. A pathophysiological approach would help in defining a more precise terminology. Antalgic contracture principally concerns a compensating phenomenon related to a polysynaptic reflex Painful contracture includes different varieties of cramps and metabolic-induced contractures. Painless contracture groups together myostatic and myostatic contractures. We suggest a simplified nomenclature.
Contracture↗
Calcein as a fluorescent probe for ferric iron. Application to iron nutrition in plant cells.
The recent use of calcein (CA) as a fluorescent probe for cellular iron has been shown to reflect the nutritional status of iron in mammalian cells (Breuer, W., Epsztejn, S., and Cabantchik, Z. I. (1995) J. Biol. Chem. 270, 24209-24215). CA was claimed to be a chemosensor for iron(II), to measure the labile iron pool and the concentration of cellular free iron(II). We first study here the thermodynamic and kinetic properties of iron binding by CA. Chelation of a first iron(III) involves one aminodiacetic arm and a phenol. The overall stability constant log beta111 of FeIIICAH is 33. 9. The free metal ion concentration is pFeIII = 20.3. A (FeIII)2 CA complex can be formed. A reversible iron(III) exchange from FeIIICAH to citrate and nitrilotriacetic acid is evidenced when these ligands are present in large excess. The kinetics of iron(III) exchange by CA is compatible with metabolic studies. The low reduction potential of FeIIICAH shows that the ferric form is highly stabilized. CA fluorescence is quenched by 85% after FeIII chelation but by only 20% using FeII. Real time iron nutrition by Arabidopsis thaliana cells has been measured by fluorimetry, and the iron buffer FeIIICAH + CA was used as source of iron. As a siderophore, FeIIICAH promotes cell growth and regreening of iron-deficient cells more rapidly than FeIIIEDTA. We conclude that CA is a good chemosensor for iron(III) in cells and biological fluids, but not for Fe(II). We discuss the interest of quantifying iron buffers in biochemical studies of iron, in vitro as well as in cells.
Cells, Cultured↗
MRI of the intracranial corticospinal tracts in amyotrophic and primary lateral sclerosis.
Our aim was to investigate the corticospinal tracts (CST) in motor neurone disease, using MRI, and to correlate findings with clinical data. We studied 31 patients with amyotrophic (ALS) and eight with primary lateral sclerosis (PLS). The signal from the CST was classified into four grades on T2-weighted images, and compared to T2-weighted images of 37 age-matched control subjects. No abnormalities were seen in the CST on T1-weighted images and were rarely evident on proton-density weighting. Variable high signal in the CST was found on T2-weighted images in 35 patients, and in 29 control subjects. Our grades 0 and 1 were more frequent in control subjects, grades 2 and 3 more frequent in patients. We found no correlation between the high signal and clinical data, including the duration of the illness. We therefore conclude that this technique is neither sensitive nor specific except in grade 3 which is quite specific for ALS. In half the patients we found atrophy of the superior parietal gyrus, which merits further study.
Adult↗
Pain in Parkinson's disease patients.
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Chronic Disease↗
[Muscular intolerance of exercise. Current data].
Our knowledge of the mechanisms leading to exercise intolerance is constantly expanding. Since the discovery of the glycolysis pathway block caused by phosphorylase deficiency as the cause of McArdle's disease, several other glycolysis blocks have been identified constituting a first group of exercise intolerance syndromes. A second group involves mitochondrial anomalies. More recently diverse exercise intolerance syndromes have been associated with insufficient regulation of calcium flow through the sarcoplasmic reticulum, particularly in sporadic cases of malignant hyperthermia with or without hyperthermia. A discrete form of dystrophinopathy is expressed by exercise-induced myalgia with myoglobinuria. Proximal myotonic myopathy also produces pain at exercise. The specificity of other syndromes such as AMP deaminase deficiency or myopathy with tubular aggregates remains debatable. Our understanding of these different syndromes, and their recognized or yet to be elucidated causes, is of practical significance for developing exploration protocols for patients with exercise intolerance with or without myoglobulinuria.
Exercise↗
[Neuromuscular diseases with eosinophilia].
Neuromuscular diseases with eosinophilia include a number of disorders associated with variable degrees of muscle, peripheral nerve, and connective tissue involvement. Eosinophilic infiltration in blood and/or tissue is a consistent finding. In addition to the neurologic manifestations of systemic vascularitis, in particular Churg and Strauss syndrome, there are three main forms of neuromuscular disease. Diffuse fasciitis or Shulman syndrome which can be limited to the fascia or associated with perimyositis is sensitive to corticosteroids. Eosinophilic myositis corresponds to focal muscle involvement and is also sensitive to corticosteroids. Eosinophilic polymyositis is a manifestation of essential hypereosinophilic syndrome and is life-threatening. Eosinophilia-myalgia syndrome and toxic oil syndrome are separate entities that occur in outbreaks and involve poisoning by ingestion of L-tryptophan and adulterated oil containing aniline respectively. The key to diagnosis of these neuromuscular diseases is muscle biopsy to detect the presence of polynuclear eosinophils.
Anti-Inflammatory Agents↗