Search PubMed⌕ Search

Biomedical subjects

P Devaux

Publications and source records attributed to P Devaux.

29 records · Page 2Linked to original sources

Multiple congenital anomalies due to partial 2p13----2pter duplication resulting from an unbalanced X;2 translocation.

It has been suggested that partial distal 2p2----2pter duplication causes a relatively well defined clinical syndrome, mostly as regards craniofacial dysmorphism, musculoskeletal and genitalia anomalies. Duplications covering a larger portion of 2p i.e. 2p12 or 2p13----2pter are however less documented. The authors report a new case of partial 2p13----2pter duplication which supplies further evidence for short life expectancy due to the large number of malformations in these partial duplications.

Abnormalities, Multiple↗

Epidemiology of diaphragmatic hernia in Languedoc-Roussillon.

The frequency of diaphragmatic hernia (DH) varies, according to the studies, between 1/2000 and 1/7000. In the Languedoc-Roussillon (South of France), due to the presence of a Regional Foeto-Pathology Department and Medico Surgical Paediatric Department, it was possible to itemize all of the DH over a 24 month period (June 1989----May 1991). 20 children presenting DH (10 foetuses and 10 liveborns) were examined for a total population of 49.350 foetuses and liveborns (frequency of DH: 0.40/1000). 10 DH were associated with extra-pulmonary malformations (50%). 4 chromosome abnormalities were found (20%). Prenatal chromosome analysis in cases of ultrasound malformation detection has increased the number of karyotype abnormalities diagnosed.

Chromosome Aberrations↗

Interaction of crotoxin and its isolated subunits with spin-labeled fatty acids.

We have investigated the interaction of crotoxin (component A-component B complex) and of its isolated phospholipase subunit (component B) with hydrophobic compounds by ESR, using spin-labeled fatty acids as probes. The phospholipase subunit alone (component B) binds more than three labeled fatty acid molecules/molecule with different affinities, the highest corresponding to a Kd of 10 microM in the case of 5-doxyl palmitic acid. In contrast, the noncatalytic subunit (component A) and the crotoxin complex do not bind fatty acids. ESR studies of the component B-fatty acid complex reveal a strong immobilization of the whole length of the fatty acid chain, strong spin-spin interactions between bound fatty acids, and nonaccessibility of the bound paramagnetic probe to Ni2+ ions. This suggests that the phospholipase component B possesses a hydrophobic cleft which may contain one or two fatty acids. This hydrophobic cleft is not accessible to spin-labeled fatty acids in the crotoxin complex. An overall rotational correlation time of about 200 ns of the phospholipase component B was determined by saturation transfer ESR. This high value is incompatible with the diffusion of a polypeptide of 14,500 molecular weight. The hydrodynamic analysis of the fatty acid-component B complex led us to estimate an apparent molecular weight of 95,000 which reveals that fatty acids induce the formation of polymers (most probably octamers) of component B. We propose a model in which the phospholipase component B exists in two conformational states which differ by their hydrophobicity.

Crotalid Venoms↗

Rapid lateral diffusion of phospholipids in rabbit sarcoplasmic reticulum.

Phospholipid spin labels incorporated in the sarcoplasmic reticulum from rabbit-skeletal muscle undergo rapid lateral diffusion within the plane of the membrane. The diffusion constant, D, is 6x10(-8) cm(2)/sec at 37 degrees . With this diffusion constant, a phospholipid molecule can diffuse a distance of the order of 5000 nm in 1 sec.

Animals↗