The antifungal protein AFP secreted by Aspergillus giganteus does not cause detrimental effects on certain mammalian cells.

The antifungal protein AFP is a small, cystein-rich protein secreted by the imperfect ascomycete Aspergillus giganteus. The protein efficiently inhibits the growth of filamentous fungi, including a variety of serious human and plant pathogens mainly of the genera Aspergillus and Fusarium, whereas AFP does not affect the growth of yeast and bacteria. This restricted susceptibility range makes it very attractive for medical or biotechnological use to combat fungal infection and contamination.

Charcot-Marie-Tooth disease and related hereditary polyneuropathies: molecular diagnostics determine aspects of medical management.

Purpose: An evidence-based approach was used to determine the frequency distribution of genes contributing to the Charcot-Marie-Tooth (CMT) disease phenotype.

Methods: We performed a combined analysis of 11 population-based studies from various ethnic backgrounds to generate an evidence-based testing scheme. To estimate the relative frequencies of the responsible genes for which population-based studies are not available, we used our cohort of clinically classified patients with CMT and related neuropathies collected before the availability of genetic testing.

Differential expression of potassium currents in Deiters cells of the guinea pig cochlea.

Among the supporting cells, Deiters cells are in intimate contact with outer hair cells (OHCs) in the inner ear. The aim of this study was to characterize the outward rectifying K+ current of Deiters cells in conjunction with cellular morphological characteristics. In the majority of cells, the K+ current had a biphasic inactivation kinetics (tau1 and tau2 were 2,735+/-90 (n=77) and 160+/-14 ms (n=72), respectively).

T118M PMP22 mutation causes partial loss of function and HNPP-like neuropathy.

Objective: To determine the clinical consequences of the PMP22 point mutation, T118M, which has been previously considered to either cause an autosomal recessive form of Charcot-Marie-Tooth (CMT) disease or be a benign polymorphism.

Methods: We analyzed patients from five separate kindreds and characterized their peripheral nerve function by clinical and electrophysiological methods.

Results: All heterozygous patients had clinical and/or electrophysiological features of a neuropathy similar to hereditary neuropathy with liability to pressure palsies (HNPPs).

[Prolonged hiccups causing worsened quality of life--successful medical therapy].

Hiccups are intermittent, involuntary spasmodic contractions of the diaphragm and the intercostal muscles leading to the cessation of inspiration due to an abrupt closure of the glottis. Hiccups are usually innocuous phenomena, which could easily be terminated by simple manoeuvres but sometimes they become permanent and intractable causing significant decline in the patient's quality of life. Besides a number of provoking exogenous factors hiccups are caused by organic or psychical disorders.

Presence of 'PSI free' LHCI and monomeric LHCII and subsequent effects on fluorescence characteristics in lincomycin treated maize.

The cause of the strong non-photochemical fluorescence quenching was examined in maize (Zea mays L.) plants that were treated with lincomycin during the 72 h period of greening. They were deficient in core complexes but seemed to contain the full complement of antennae.

Potentiometric sensor for the measurement of Cd2+ transport in yeast and plants.

Research on heavy metals, and especially on transport of Cd(2+), has attracted much interest during the past decade. An optimized Cd(2+)-selective electrode for the continuous potentiometric monitoring of Cd(2+) fluxes in biological systems is presented. The selectivity of the electrode for Cd(2+) was further improved, and it now has very good long-term stability.

[The first genetically supported case of chronic benign pemphigus (Hailey-Hailey disease in Hungary].

Hailey-Hailey disease, or chronic benign pemphigus (MIM# 169600), is a genodermatosis arising in adult age with recurrent vesicles and erosions primarily in the flexural areas. It is an autosomal dominant skin disorder characterized by abnormal keratinocyte adhesion in the suprabasal layers of the epidermis. ATP2C1, encoding the human secretory pathway Ca(2+)-ATPase (hSPCA1), was recently identified as the defective gene in Hailey-Hailey disease.

Comparison of selective media for the isolation of Rhodococcus equi and description of a new selective plating medium.

In order to improve the isolation rate of Rhodococcus equi from animals and soil, the efficacy of four previously described selective media (CAZ-NB, M3T, NANAT and TINSDALE) and that of four other media (NC, PNP, TCP and TVP) composed by us was compared and evaluated. Two selective plating media proved to be the best for the isolation of R. equi from contaminated samples.

Extracellular matrix molecules and their possible roles in the regeneration of frog nervous system.

Recent biochemical and histochemical analyses explored different components of the extracellular matrix (ECM) in the nervous system, and either permissive or non-permissive roles in neuronal development and regeneration were suggested. The aim of this study was to detect the distribution pattern of a few of these molecules in the nervous system of intact frogs and during nerve regeneration. The hyaluronan (HA) and tenascin C reactions were negative in the peripheral nerves, but appeared in their entry zones.

Calcium and magnesium competitively influence the growth of a PMR1 deficient Saccharomyces cerevisiae strain.

PMR1, the Ca2+/Mn2+ ATPase of the secretory pathway in Saccharomyces cerevisiae was the first member of the secretory pathway Ca2+ ATPases (SPCA) to be characterized. In the past few years, pmr1Delta yeast have received more attention due to the recognition that the human homologue of this protein, hSPCA1 is defective in chronic benign pemphigus or Hailey-Hailey disease (HHD). Recent publications have described pmr1Delta S.

Age-dependence of the spontaneous activity of the rat urinary bladder.

Abnormal mechanical function of the bladder is manifested in a number of ways including higher frequency of involuntary detrusor contractions associated with reduced compliance of the bladder that is responsible for an increase in intraluminal pressure during filling. There are basically two ways to approach experimentally these problems: (1) by studying the neural control of the lower urinary tract function, and (2) by measuring the properties of smooth muscle cells in the bladder wall. Studies on smooth muscle function often do not take the origin of smooth muscle cells into account i.

Debris of "dark" (compacted) neurones are removed from an otherwise undamaged environment mainly by astrocytes via blood vessels.

By means of a condenser-discharge electric shock paradigm, "dark" granule neurones were momentarily produced in a sporadic distribution among normal ones in the otherwise undamaged (non-necrotic, non-excitotoxic, non-inflammatory or non-contused) hippocampal dentate gyri of the rat brain. In the electron microscope, the ultrastructural elements of the affected neurones remained undamaged but turned markedly electron-dense and the distances between them became strikingly reduced (compaction). A proportion of such neurones recovered in 1 day while others died.

Prenatal diagnosis, phenotypic and obstetric characteristics of holoprosencephaly.

The diagnosis of fetal malformations, especially those of the central nervous system, is strikingly important in the practice of genetic counseling. Early diagnosis is very significant, not only because of the prognosis, but also because of the emotional effects caused by the accompanying craniofacial malformations. The summary of the obstetrical and diagnostical characteristics should be useful in the management of holoprosencephaly.

SIMPLE mutations in Charcot-Marie-Tooth disease and the potential role of its protein product in protein degradation.

Charcot-Marie-Tooth (CMT) disease is a clinically and genetically heterogeneous group of inherited peripheral neuropathies characterized by progressive weakness and atrophy of distal limb muscles. Recently, SIMPLE/LITAF was shown to be responsible for an autosomal dominant demyelinating form of CMT linked to 16p (CMT1C). Although two transcripts encoding different proteins (SIMPLE and LITAF) have been reported from the same gene, we could not confirm the existence of LITAF.

Effect of induction chemotherapy on changes of laminin and syndecan expression in oral squamous cell carcinomas: a prospective, randomized, clinicopathologic and immunohistochemical study.

Sixty patients with tumors of the floor of the mouth or of the tongue (T2N0-1-2M0) were randomized into three treatment groups. The first two groups participated in low-dose inductive chemotherapy, surgery, and then radiotherapy, whereas the third control group underwent only surgery and radiotherapy. In all three groups, studies were made of the stage, grade, sex, localization, extents of expression of the pretreatment laminin and syndecan-1 and the cancer specific survival rate, and the correlations among these.

The Penicillium chrysogenum-derived antifungal peptide shows no toxic effects on mammalian cells in the intended therapeutic concentration.

Certain filamentous fungi, such as the penicillin-producing strain Penicillium chrysogenum, secrete small, highly basic and cysteine-rich proteins with antifungal effects. Affected fungi include a number of important zoopathogens, including those infecting humans. Recent studies, however, have pointed to a membrane-perturbing effect of these antifungal compounds, apparent as a potassium efflux from affected fungal cells.

Cloning, sequencing, structural and molecular biological characterization of placental protein 20 (PP20)/human thiamin pyrophosphokinase (hTPK).

Full-length cDNAs of placental protein 20 (PP20) were cloned by screening a human placental cDNA library, which encode a 243 amino acid protein, identical to human thiamin pyrophosphokinase (hTPK) as confirmed by protein sequence analysis. Genomic alignment showed that the PP20/hTPK gene contains 9 exons. It is abundantly expressed in placenta, as numerous EST clones were identified.

Extremely high maternal alkaline phosphatase serum concentration with syncytiotrophoblastic origin.

An extremely high alkaline phosphatase (AP) concentration (3609 IU/litre) was found in a 20 year old primigravida at 37 week's gestation, prompting an examination of its histological and cellular origin. Immunohistochemistry and western blots using antibodies against AP, Ki-67, phospho-protein kinase B (Akt), phospho-p44/42 mitogen activated protein kinase/extracellular signal regulated kinase 1/2 (MAPK/Erk1/2), phospho-glycogen synthase kinase-3beta (GSK-3beta), phospho-stress activated protein kinase/c-Jun N-terminal kinase, total-Akt, total-GSK-3beta, and phospho-p38-MAPK were carried out on index and control placental samples of the same gestational age. Compared with controls, staining of the index placenta showed minimal AP labelling of the brush border and remarkable positivity of the intervillous space.

Increased blood-brain barrier permeability with thymidine phosphorylase deficiency.

Mitochondrial neurogastrointestinal encephalomyopathy is an autosomal recessive multisystemic disorder caused by thymidine phosphorylase deficiency. Whereas the pathomechanism of the secondary mitochondrial dysfunction has been extensively studied, that of the leukoencephalopathy has not been elucidated. We hypothesized that the white matter hyperintensities on T2-weighted magnetic resonance images reflect disturbance of blood-brain barrier function.

Therapeutic efficiency of continuous renal replacement therapy--experience of a single Romanian center.

We have studied 24 patients with acute renal failure (ARF) or acutization of chronic renal failure (CRF) caused by a variety of different disorders. A rapid decline in renal excretory function, fluid, electrolyte, and divalent ion disturbances determined us to initiate continuous renal replacement: continuous venovenous hemofiltration (CVVHF)--28 procedures in 15 patients and continuous venovenous hemodiafiltration (CVVHDF)--11 procedures in 11 patients. Mean duration of CVVHF was 21.

[Alveolar bone regeneration stimulated by a combination of platelet-rich plasma and Cerasorb graft in Beagle dogs. Histological and histomorphometric studies].

Healing of extraction wounds was examined in an animal experiment model on Beagle dogs. After bilateral extraction of the premolars of 12 dogs the alveoli were filled up with a combination of beta-tricalcium phosphate (Cerasorb) graft and platelet rich plasma (PRP) on the experimental side, and with Cerasorb alone on the control side. Biopsy specimens were taken from the regenerating bone at 6, 12 and 24 weeks after surgery for histological study.