Osteogenesis imperfecta

Osteogenesis imperfecta (OI) is the most frequently occurring congenital disorder with an increased fracture rate and systemic skeletal involvement. The vast majority of patients have an autosomal dominant form of OI resulting from a mutation in one of the two type I collagen genes COL1A1 or COL1A2. Since 2006, eight genes for autosomal recessive forms of the disorder have been identified, as well as one additional gene for autosomal dominant OI. Our knowledge concerning molecular pathophysiology has been substantially broadened, such that the paradigm of OI as a pure ??collagenopathy?? no longer applies and the clinical classification system will have to be revised. Standard therapy for the more severe forms of OI comprises intravenous administration of bisphosphonates. Additional elements of a multimodal therapeutic concept include surgical intervention for bone deformities or fractures and physiotherapy.     

Acanthodactyles of Tunisia: VIII--The southern acanthodactyles from south of Chott el Djerid

Applied to 135 Acanthodactylus, from eight clusters of collecting sites, a multidimensional analysis of 11 characters, mainly of colouring and scale patterns, providing 35 mathematical variables, reveals a hight intrapopulational variability. Samples dwelling on reg nebkhas and sandy knolls in the river beds are Acanthodactylus inornatus. Some specimens, in the sample from barkhanes and sand dunes areas, are morphologically close to A. longipes.     

On-line evaluation of fermentation broth colour

Summary A novel method for evaluating pigment production during fermentation ofMonascus ruber has been developed and consists of reflectance measurement with a 10° observer under an artificial illuminant. A spectrophotometer was adapted to measure pigmentation during culture in a bioreactor. The lightness parameter L was the most useful information delivered by this sensor. An absorbency over 30 units at 480nm on batch cultures was estimated on-line without dilution. member of the GR Automatique - SARTA - CNRS