نتایج جستجو برای: bullous ichthyosiform erythroderma
تعداد نتایج: 15264 فیلتر نتایج به سال:
The family of protein kinase C (PKC) isoenzymes plays a fundamental part in signal transduction, and thereby regulates important cellular functions, including growth, differentiation, cytokine production and adhesion molecule expression. In lesional psoriatic skin, Ca(2+)-dependent PKC activity, PKC-beta protein and epidermal Langerhans cell (LC) PKC-beta immunostaining are significantly decrea...
Sir, Bullous congenital ichthyosiform erythroderma (BCIE), or epidermolytic hyperkeratosis (MIM# 113.800), is a rare disorder of keratinization associated with blistering in its early phase. It was first described by Jean-Louis Brocq in 1902 as érythrodermie congénitale ichthyosiforme avec hyperépidermotrophie. Although most cases are sporadic, familial cases show an autosomal dominant pattern ...
We report an unusual phenotype of congenital hemidysplasia with ichthyosiform erythroderma and limb defects syndrome most likely resulting from a novel X-chromosomal microdeletion encompassing the promoter region and exon 1 of the nicotinamide adenine dinucleotide phosphate steroid dehydrogenase-like protein gene, the neighboring gene CETN2, and more than 10 kb of noncoding deoxyribonucleic acid.
High resolution while maintaining high peak reflectivities can be achieved for Lamellar Multilayer Amplitude Gratings (LMAG) in the soft-x-ray (SXR) region. Using the coupled waves approach (CWA), it is derived that for small lamellar widths only the zeroth diffraction order needs to be considered for LMAG performance calculations, referred to as the single-order regime. In this regime, LMAG pe...
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