The Cystic Fibrosis Symptom Progression Survey (CF-SPS) in Arabic: A Tool for Monitoring Patient's Symptoms.

Objective: Our study aimed to develop a survey that could be used by nurses during regular cystic fibrosis (CF) clinic visits, providing clinicians with a standardized means of longitudinally assessing and monitoring symptom progression in their patients. In addition, the use of this survey would provide an opportunity for patient engagement and relationship building, thereby enhancing patient education and improving adherence to treatment. This is the first such survey designed specifically for use in Arab populations.

Defining a mutational panel and predicting the prevalence of cystic fibrosis in oman.

Objectives: Cystic fibrosis transmembrane conductance regulator (CFTR) mutations form distinct mutational panels in different populations and subgroups. The frequency of cystic fibrosis (CF) mutations and prevalence are unknown in Oman. This study aimed to elucidate the mutational panel and prevalence of CF for the North Al Batinah (NAB) region in Oman and to estimate the national prevalence of CF based on the carrier screening of unrelated volunteers.

Fatty acid cytotoxicity to human lens epithelial cells.

Data obtained with the neutral red cytotoxicity assay reveal that human lens epithelial cells in culture are highly sensitive to low micromolar concentrations of unsaturated, cis-configured fatty acids in the following order: arachidonic acid>linolenic acid=linoleic acid=oleic acid, whereas the saturated fatty acids are much less effective. Though the cytotoxic effects of the unsaturated fatty acids could not be discerned from effects of their oxidation products, the fact that oleic acid is equally cytotoxic as linoleic acid or linolenic acid as well as previously reported findings with bovine lens epithelial cells support the idea that the unsaturated fatty acid molecules directly account for the cytotoxicity and not their products of lipid peroxidation. Bleb formation and cell retraction are early morphological signs of fatty acid-induced lens cell damage.

The role of glutathione in nitric oxide donor toxicity to SN56 cholinergic neuron-like cells.

Our study was designed to determine if compounds used experimentally to generate nitric oxide excess differ in ability to elicit degenerative stress to cholinergic neurons and, if so, what mechanisms account for their differences. Nitric oxide donors are often used experimentally in attempts to emulate the bioactivities of endogenous NO, but the pharmacological actions of NO donors can vary dramatically according to the species of NO (NOx) and other agents (e.g.

Readthrough of dystrophin stop codon mutations induced by aminoglycosides.

We report the translational readthrough levels induced by the aminoglycosides gentamicin, amikacin, tobramycin, and paromomycin for eight premature stop codon mutations identified in Duchenne's and Becker's muscular dystrophy patients. In a transient transfection reporter assay, aminoglycoside treatment results show that one stop codon mutation is suppressed significantly better (up to 10% stop codon readthrough) than the others; five show lower but statistically significant suppression (< 2% stop codon readthrough); and two appear refractory to aminoglycoside treatment. Readthrough levels do not substantially vary between different sources of gentamicin, and, for this set of mutations, the efficiency of termination at the premature stop codon mutation does not appear to correlate with disease severity.

Analysis of expression patterns of protein phosphatase-1 and phosphatase-2A in rat and bovine lenses.

Purpose: The reversible phosphorylation and dephosphorylation at the serine and threonine residues on proteins play distinct roles in regulating multiple cellular activities. Whereas the protein serine-threonine kinases have been well studied in the lens system, very little is known about the expression and function of the serine-threonine phosphatases. The present article reports the expression patterns of protein phosphatase (PP)-1 and -2A in adult rat and bovine lenses.

Caspase-3 is actively involved in okadaic acid-induced lens epithelial cell apoptosis.

Phosphorylation and dephosphorylation are important cellular events regulating major metabolic activities such as signal transduction, gene expression, cell cycle progression, and apoptosis. It is well documented that okadaic acid, a potent inhibitor of protein phosphatase-1 (PP-1) and -2A (PP-2A), can induce apoptosis in a variety of cell lines. Our recent studies have revealed that in the immortal rabbit lens epithelial cell line, N/N1003A, inhibition of PP-1, but not PP-2A, leads to rapid apoptosis of the lens epithelial cells.

Retinoic acid-mediated enhancement of the cholinergic/neuronal nitric oxide synthase phenotype of the medial septal SN56 clone: establishment of a nitric oxide-sensitive proapoptotic state.

It is unclear what mechanisms lead to the degeneration of basal forebrain cholinergic neurons in Alzheimer's or other human brain diseases. Some brain cholinergic neurons express neuronal nitric oxide (NO) synthase (nNOS), which produces a free radical that has been implicated in some forms of neurodegeneration. We investigated nNOS expression and NO toxicity in SN56 cells, a clonal cholinergic model derived from the medial septum of the mouse basal forebrain.

Differential vulnerability of primary cultured cholinergic neurons to nitric oxide excess.

Many neuronal nitric oxide synthase (nNOS)-expressing brain neurons, including some cholinergic populations, are resistant to disease or to certain forms of excitotoxicity. Vulnerability to NO excess of forebrain (medial septal/diagonal band; MS-ACh) and brainstem (pedunculopontine/laterodorsal tegmental nuclei; BS-ACh) cholinergic neurons was compared in E16-E18 primary rat brain cultures. MS-ACh cells were approximately 300-fold more sensitive to the NO donor S-nitro-N-acetyl-D,L-penicillamine (SNAP) than were BS-ACh cells.

Okadaic acid-induced lens epithelial cell apoptosis requires inhibition of phosphatase-1 and is associated with induction of gene expression including p53 and bax.

It is well established that phosphorylation and dephosphorylation are key cellular events which regulate important metabolic activities such as gene expression, cell cycle progression, and apoptosis. The polyether fatty acid, okadaic acid has been shown previously to activate apoptosis in a variety of cell lines. Although this marine sponge toxin is known to inhibit protein phosphatase (PP)-2A and PP-1, it is not certain in most cases whether inhibition of PP-1 or PP-2A is necessary to activate apoptosis.

Low concentrations of cis-linoleic acid induce cell damage in epithelial cells from bovine lenses.

As low as 5 micromol/l of cis-linoleic acid proves to be cytotoxic for bovine lens epithelial cells in culture. Albumin eliminates the linoleic acid cytotoxicity completely, presumably by binding the fatty acid. However, the damaging effect appears again when the molar ratio of linoleic acid to albumin exceeds 1:1.