[Mitochondriopathies].

Aims: The purpose of this study is to review different aspects of mitochondrial myopathies.

Development: Mitochondrial DNA is different to that found in the nucleus and is generally inherited through the mother. There are from 2 to 10 copies per mitochondrion and hundreds or thousands of mitochondria per cell.

Patterns of antimicrobial activities from soil actinomycetes isolated under different conditions of pH and salinity.

Aims: To evaluate the patterns of the production of antimicrobial compounds by diverse collection of actinomycetes isolated from different geographies under alternative conditions of pH and salinity in the media.

Methods And Results: Actinomycetes were grouped based on their method of isolation and their phenotype diversity was determined by total fatty acid analysis. A total of 335 representative isolates, including 235 Streptomyces species and 100 actinomycetes from other taxa, were screened for the production of antimicrobial activities against a panel of bacteria, filamentous fungi and yeasts, including some of clinical relevance.

Two pathogenic mutations in the mitochondrial DNA tRNA Leu(UUR) gene (T3258C and A3280G) resulting in variable clinical phenotypes.

We studied two patients with ragged-red fibers and combined defects of the mitochondrial respiratory chain in their muscle biopsy. One had mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes, and harbored a T3258C transition in the tRNA(Leu(UUR)) gene. The other showed myopathy plus cardiomyopathy and had an A3280G mutation in the same gene.

Kochen-Specker theorem for a single qubit using positive operator-valued measures.

A proof of the Kochen-Specker theorem for a single two-level system is presented. It employs five eight-element positive operator-valued measures and a simple algebraic reasoning based on the geometry of the dodecahedron.

Microbial natural products as a source of antifungals.

The vast number and variety of chemotherapeutic agents isolated from microbial natural products and used to treat bacterial infections have greatly contributed to the improvement of human health during the past century. However, only a limited number of antifungal agents (polyenes and azoles, plus the recently introduced caspofungin acetate) are currently available for the treatment of life-threatening fungal infections. Furthermore, the prevalence of systemic fungal infections has increased significantly during the past decade.

Intestinal pseudo-obstruction and urinary retention: cardinal features of a mitochondrial DNA-related disease.

The syndrome of mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) is a multisystemic disorder associated in most of the patients with an A to G transition at nucleotide position 3243 in the transfer RNA (tRNA)Leu(UUR) (A3243G) of the mitochondrial DNA. This syndrome is characterized by the preponderant involvement of skeletal muscle and central nervous system, but urinary or gastrointestinal symptoms are seldom documented. Here we report an unusual case of a 52-year-old woman with a clinical phenotype characterized by encephalopathy, left hemiparesis, urinary retention and gastrointestinal pseudo-obstruction.

[Impact of regulatory measures in the trends of community consumption of antibiotics in Chile].

Background: The indiscriminate use of antibiotics increases the appearance of bacterial resistance and forces the use of more expensive alternatives. The Chilean Ministry of Health is regulating the consumption of antibiotics since September 1999. These regulatory measures restricted the sale of antibiotics only when these were prescribed by an MD or an DS.

Myoglobinuria and COX deficiency in a patient taking cerivastatin and gemfibrozil.

The authors describe a patient who presented with myoglobinuria after starting cerivastatin-gemfibrozil therapy. Muscle histochemistry revealed ragged-red fibers and cytochrome c oxidase negative (COX) fibers, and biochemistry showed a defect of COX activity. Immunoblot analysis showed a 60% reduction of COX I and COX II polypeptides.

N-particle N-level singlet States: some properties and applications.

Three apparently unrelated problems which have no solution using classical tools are described: the "N-strangers," "secret sharing," and "liar detection" problems. A solution for each of them is proposed. Common to all three solutions is the use of quantum states of total spin zero of N spin-(N-1)/2 particles.

Cosegregation of the mitochondrial DNA A1555G and G4309A mutations results in deafness and mitochondrial myopathy.

We report a patient with progressive external ophthalmoplegia (PEO), exercise intolerance, and deafness after aminoglycoside exposure, harboring two pathogenic mutations in her mtDNA: an A1555G in the 12S rRNA gene and a G4309A in the tRNA(Ile) gene. Muscle histochemistry showed abundant ragged-red fibers, and biochemistry revealed normal respiratory chain function. The A1555G mutation was homoplasmic in blood from the proband and from all maternal relatives.

Violating Bell's inequality beyond Cirel'son's bound.

Cirel'son inequality states that the absolute value of the combination of quantum correlations appearing in the Clauser-Horne-Shimony-Holt (CHSH) inequality is bound by 2 square root of (2). It is shown that the correlations of two qubits belonging to a three-qubit system can violate the CHSH inequality beyond 2 square root of (2). Such a violation is not in conflict with Cirel'son's inequality because it is based on postselected systems.

[Eosinophilic myositis in a 9 year old boy].

Introduction: Eosinophil infiltration of skeletal muscle is rare, but often no etiological factor can be identified and these are isolated eosinophilic myositis. They may be associated with parasite infections or drugs, or be features of rare systemic disorders of hypereosinophilia, such as the myalgia eosinophilia syndrome and the idiopathic hypereosinophilic syndrome. The eosinophilic myopathies should be distinguished from the commoner inflammatory myopathies such as polymyositis and dermatomyositis.

Screening of antimicrobial activities in red, green and brown macroalgae from Gran Canaria (Canary Islands, Spain).

Extracts from 44 species of seaweed from Gran Canaria (Canary Islands, Spain) were screened for the production of antibacterial and antifungal compounds against a panel of gram-negative and gram-positive bacteria, mycobacteria, yeasts and fungi. A total of 28 species displayed antibacterial activity, of which six also showed antifungal activity. Asparagopsis taxiformis and Cymopolia barbata were the species with the strongest activities against the broadest spectrum of target microorganisms.

[Infantile neuroaxonal dystrophy. A report of two new cases and a review of the literature published over the past ten years].

Introduction: The neuroaxonal dystrophies make up a group of neurodegenerative disorders of unknown origin, which are characterized by all showing axonal lesions. The infantile form, or Seitelberger s disease, is one of the forms of earliest onset and rapid progression. The clinical, neurophysiological and pathological criteria described by Aicardi and Castelein in 1979 are still valid.

Antimicrobial activity of ergokonin A from Trichoderma longibrachiatum.

Aims: Natural fungal products were screened for antifungal compounds. The mode of action of one of the hits found and the taxonomy of the producing organism were analysed.

Methods And Results: An extract from a Trichoderma species showed a more potent activity in an agar-based assay against the null mutant fks1::HIS strain than against the wild-type strain, suggesting that it could contain a glucan synthesis inhibitor.

Molecular heterogeneity of myophosphorylase deficiency (McArdle's disease): a genotype-phenotype correlation study.

We report on 54 Spanish patients with McArdle's disease from 40 unrelated families. Molecular analysis revealed that the most common R49X mutation was present in 70% of patients and 55% of alleles. The G204S mutation was less frequent and found in 14.

Mitochondrial dysfunction associated with a mutation in the Notch3 gene in a CADASIL family.

Background: Cerebral autosomal arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterized by recurrent subcortical ischemic strokes and dementia caused by mutations in the Notch3 gene. In Drosophila melanogaster, Notch signaling has a pleiotropic effect, affecting most tissues of the organism during development.

Objective: To characterize a potential mitochondrial dysfunction associated with mutations in the Notch3 gene.

Early-onset multisystem mitochondrial disorder caused by a nonsense mutation in the mitochondrial DNA cytochrome C oxidase II gene.

We report the first nonsense mutation (G7896A) in the mtDNA gene for subunit II of cytochrome c oxidase (COX) in a patient with early-onset multisystem disease and COX deficiency in muscle. The mutation was heteroplasmic in muscle, blood, and fibroblasts from the patient and abundantly present in COX-deficient fibers, but less abundant in COX-positive fibers; it was not found in blood samples from the patient's asymptomatic maternal relatives. Immunoblot analysis showed a reduced concentration of both COX II and COX I polypeptides, suggesting impaired assembly of COX holoenzyme.

[Technology for the whole utilization of brewer's yeast in food industry].

A flexible scheme for the fractionation of brewer's yeast was developed. The procedure allows the production of different products such as: dry yeast flakes, dry yeast pills, yeast-extract based table sauce, yeast protein concentrates and soy-like sauce. The investment required for the processing of one ton per day is below 2 million dollars with an overall profitability higher than 53%.

"All versus nothing" inseparability for two observers.

A recent proof of Bell's theorem without inequalities [A. Cabello, Phys. Rev.

[Neonatal hypotonia of muscular origin: analysis of 50 cases].

Background: Neonatal hypotonia is a common request for neurological consultation. The aim of this study is to describe the main clinical features of muscular hypotonia in newborns at the light of the histopathological findings.

Methods: We reviewed 50 medical records of hypotonic neonates with abnormal muscular biopsy.

Bell's theorem without inequalities and without probabilities for two observers.

A proof of Bell's theorem using two maximally entangled states of two qubits is presented. It exhibits a similar logical structure to Hardy's argument of "nonlocality without inequalities." However, it works for 100% of the runs of a certain experiment.

[Early erythropoietin use for the prevention of anemia in infant premature].

Background: Anemia is common among very low birth weight newborns and requires frequent blood transfusions. Erythropoietin was been reported to be useful in the prevention of this anemia.

Aim: To assess the benefits of early (before the third week of life) Human recombinant Erythropoietin (r-EPO) administration to reduce the requirement of blood transfusions in very low birth weight newborns.

Quantum key distribution in the Holevo limit.

A theorem by Shannon and the Holevo theorem impose that the efficiency of any protocol for quantum key distribution, E, defined as the number of secret (i.e., allowing eavesdropping detection) bits per transmitted bit plus qubit, is E < or = 1.