[Primary ciliary dyskinesia: a retrospective review of clinical and paraclinical data].

Introduction: Primary ciliary dyskinesia (PCD) is an inherited disease responsible for a disruption of normal ciliary function. Its clinical presentation is usually in early childhood with pulmonary and otorhinolaryngologic symptoms. Early diagnosis is essential to avoid the development of bronchiectasis.

Functional properties of muscle-derived cells related to morphological characteristics.

Satellite cells represent a specific lineage of myogenic progenitors that allow skeletal muscle postnatal growth and repair. They have been described as being heterogeneous in nature, a characteristic associated with functional disparities. Here, we aimed at determining whether the morphometric characteristics of freshly extracted turkey muscle-derived cells (MDC) could represent a distinctive criterion between them and could also be associated with their behavioural features.

Sarcolemma phospholipid structure investigated by immunogold electron microscopy and (31)P NMR spectroscopy with lanthanide ions.

The biological functions of plasma membranes depend greatly on the biophysical properties resulting from protein and phospholipid structure. We investigated the phospholipid structure of the normal sarcolemma membrane, which is known to be highly dysfunctional in myopathies. Combining electron microscopy and (31)P nuclear magnetic resonance (NMR) spectroscopy on isolated sarcolemma vesicles, we find that (i) the sarcolemma vesicles maintain the in-vivo cellular sidedness, (ii) the phospholipid mobility is close to that observed in model membranes (similar lateral diffusion coefficients and spin-lattice T(1) relaxation times).

[Adult bronchiectasis revealing familial ciliary anomaly].

We report a case of bronchiectasis in a 26-year-old man associated with the following congenital abnormalities: deafness, purulent bronchorrhea, nasal polyps, dysmorphic physical pattern and chronic sinusitis. Situs inversus was absent. A sampling was performed on the posterior nasal mucous membrane and displayed structural ciliary abnormality: a deficiency of the intern dynein-arm.

Static and magic angle spinning (31)P NMR spectroscopy of two natural plasma membranes.

Static and magic angle spinning (31)P NMR spectroscopy was used for the first time in natural plasma membranes from erythrocytes and skeletal muscle to study phospholipid arrangement and composition. Typical static powder-like spectra were obtained showing that phospholipids were in a bilayer arrangement. Magic angle spinning narrowed spectra into two components.