[A case of nerve angiomatosis associated with neurofibromatosis type I].

We present the case of a patient suffering from Neurofibromatosis type I (NF-1) with acute, very painful neuropathy of the right lower extremity. The preoperative electro neuro- physiological study showed an impaired function of the peroneal nerve. The MRI revealed an extended diffuse plexiform tumour of the sciatic nerve and at thigh level.

Spectroscopic evidence of cerebral axonopathy in patients with "pure" adrenomyeloneuropathy.

Background: Adrenomyeloneuropathy (AMN) is the adult variant of X-linked adrenoleukodystrophy. The disease pathology is usually limited to spinal cord and peripheral nerves, and when this is the case, it is referred to as "pure" AMN. Histopathology shows cerebral involvement even in pure AMN; however, not much is known about the nature, extent, and clinical relevance of these findings.

High dose vitamin E therapy in amyotrophic lateral sclerosis as add-on therapy to riluzole: results of a placebo-controlled double-blind study.

Unlabelled: Increasing evidence has suggested that oxidative stress may be involved in the pathogenesis of amyotrophic lateral sclerosis (ALS). The antioxidant vitamin E (alpha-tocopherol) has been shown to slow down the onset and progression of the paralysis in transgenic mice expressing a mutation in the superoxide dismutase gene found in certain forms of familial ALS. The current study, a double blind, placebo-controlled, randomised, stratified, parallel-group clinical trial, was designed to determine whether vitamin E (5000 mg per day) may be efficacious in slowing down disease progression when added to riluzole.

Mechanistic and genetic overlap of barley host and non-host resistance to Blumeria graminis.

SUMMARY Non-host resistance of barley to Blumeria graminis f.sp. tritici (Bgt), an inappropriate forma specialis of the grass powdery mildew fungus, is associated with formation of cell wall appositions (papillae) at sites of attempted fungal penetration and a hypersensitive cell death reaction (HR) of single attacked cells.

Amino acid neurotransmitters in the CNS: properties of diaminobutyric acid transport.

Uptake of L-2,4-diaminobutyric acid (DABA), a positively charged analogue of gamma-aminobutyric acid (GABA), by a synaptosomal fraction isolated from rat brain occurred with a Km of 54 +/- 12 microM and a Vmax of 1.3 +/- 0.2 nmol/min/mg protein.

Amino acid neurotransmitters in the CNS. Characteristics of the acidic amino acid exchange.

D-Aspartate exchange, defined as amino acid-stimulated D-[3H]aspartate efflux, was investigated in a preparation of rat brain synaptosomes. The efflux of radiolabelled D-aspartate was found to be enhanced by micromolar concentrations of externally added D- and L-aspartate, L-glutamate, L-cysteate and L-cysteinesulphinate. The stimulation of release by external amino acids followed Michaelis-Menten kinetics; the apparent Km values (in microM) were: 14.

The role of glial cells in regulation of neurotransmitter amino acids in the external environment. II. Mechanism of aspartate transport.

Active, high-affinity (Km = 4.4 microM) D-aspartate transport in C6 astrocytoma cells has been investigated. Uptake of radioactive D-aspartate was competitively inhibited by L-aspartate (Ki = 8.

The effect of thiol reagents on GABA transport in rat brain synaptosomes.

The nature of gamma-aminobutyric acid (GABA) transport has been investigated in preparations of rat brain synaptosomes using a number of thiol reagents with varying membrane permeabilities. N-Ethylmaleimide, p-chloromercuribenzoate and p-chloromercuriphenylsulfonate effectively inhibited GABA transport in both directions (i.e.

Effect of oleate on neurotransmitter transport and other plasma membrane functions in rat brain synaptosomes.

The effects of fatty acids, oleate and palmitate, on gamma-aminobutyric acid (GABA), aspartate, and 3,4- dihydroxyphenylethylamine (dopamine) transport and a variety of other membrane functions were studied in rat brain synaptosomes at a constant lipid-to-protein ratio. Under the conditions utilized oleate, but not palmitate, caused statistically significant changes in synaptosomal functions. Oleic acid inhibited the uptake of the amino acid neurotransmitters and dopamine in a tetrodotoxin-insensitive manner; it also induced the release of neurotransmitters from synaptosomes.

Kinetic evidence for heterogeneous responsiveness of mixed function oxidase isozymes to inhibition and induction by allylisopropylacetamide in chick embryo liver.

Changes in hepatic mixed function oxidase kinetics after administration of allylisopropylacetamide (AIA) to chick embryos indicate that the activities of different cytochrome P-450 isozymes, including those participating in the metabolism of the same substrates, can be simultaneously increased and inhibited by a single xenobiotic. Up to 4 h after administration in ovo, or in vitro, AIA exclusively inhibited mixed function oxidases. At 24 h after administration in ovo, AIA simultaneously decreased the Vmax of the isozymes active in 7-ethoxycoumarin deethylation and in biphenyl and antipyrine hydroxylations in control liver and caused new isozymes with higher Km and Vmax values to appear.