Requirements for leukocyte transmigration via the transmembrane chemokine CX3CL1

The surface-expressed transmembrane CX3C chemokine ligand 1 (CX3CL1/fractalkine) induces firm adhesion of leukocytes expressing its receptor CX3CR1. After shedding by the disintegrins and metalloproteinases (ADAM) 10 and 17, CX3CL1 also acts as soluble leukocyte chemoattractant. Here, we demonstrate that transmembrane CX3CL1 expressed on both endothelial and epithelial cells induces leukocyte transmigration. To investigate the underlying mechanism, we generated CX3CR1 variants lacking the intracellular aspartate-arginine-tyrosine (DRY) motif or the intracellular C-terminus which led to a defect in intracellular calcium response and impaired ligand uptake, respectively. While both variants effectively mediated firm cell adhesion, they failed to induce transmigration and rather mediated retention of leukocytes on the CX3CL1-expressing cell layer. Targeting of ADAM10 led to increased adhesion but reduced transmigration in response to transmembrane CX3CL1, while transmigration towards soluble CX3CL1 was not affected. Thus, transmembrane CX3CL1 mediates leukocyte transmigration via the DRY motif and C-terminus of CX3CR1 and the activity of ADAM10.     

A transition zone complex regulates mammalian ciliogenesis and ciliary membrane composition

Mutations affecting ciliary components cause ciliopathies. As described here, we investigated Tectonic1 (Tctn1), a regulator of mouse Hedgehog signaling, and found that it is essential for ciliogenesis in some, but not all, tissues. Cell types that do not require Tctn1 for ciliogenesis require it to localize select membrane-associated proteins to the cilium, including Arl13b, AC3, Smoothened and Pkd2. Tctn1 forms a complex with multiple ciliopathy proteins associated with Meckel and Joubert syndromes, including Mks1, Tmem216, Tmem67, Cep290, B9d1, Tctn2 and Cc2d2a. Components of this complex co-localize at the transition zone, a region between the basal body and ciliary axoneme. Like Tctn1, loss of Tctn2, Tmem67 or Cc2d2a causes tissue-specific defects in ciliogenesis and ciliary membrane composition. Consistent with a shared function for complex components, we identified a mutation in TCTN1 that causes Joubert syndrome. Thus, a transition zone complex of Meckel and Joubert syndrome proteins regulates ciliary assembly and trafficking, suggesting that transition zone dysfunction is the cause of these ciliopathies.     

Predictability of Plastic Parts Behaviour Made from Rapid Manufacturing

One of the most important issues to resolve in parts manufactured from rapid manufacturing (RM) technologies is to know their behavior working under real conditions. Total quality manufacturing (TQM) is only possible if mechanical properties are well known in the design stage depending on the processing parameters. This work is mainly focused on testing of several samples made with different selective laser sintering (SLS) parameters and technologies. This procedure is the starting point to establish a basis for designing for RM and the standardization of RM testing. The experiments and the analysis of variance (ANOVA) analyzed the effects of several factors on mechanical properties. The SLS technologies were 3DSystem and EOS. The results show which factor has a large effect on the variables and the interaction between them. The conclusions are very useful for developing rules for designing (designing for RM) and creating new standard rules (ISO, AISI, and DIN) for RM materials and parts testing. The ANOVA gives a better knowledge of the effects of these factors and eliminates unimportant parameters.     

以龙舌兰蔗渣作为还原剂使磁铁矿精矿有效地金属化

The reduction behavior and metallization degree of magnetite concentrate with agave bagasse were investigated in an inert atmosphere. The effects of temperature, biomass content, and residence time on reduction experiments and metallization degree were investigated by X-ray diffraction and scanning electron microscopy. Compared with other types of biomass, agave bagasse had lower contents of nitrogen, sulfur, and ash. X-ray diffraction analysis showed that the metallization degree improved with increasing temperature and biomass content. Complete metallization was achieved at 1100°C for 30 min with 65:35 and 50:50 ratios of the magnetite concentrate to the agave bagasse. These results demonstrate that agave bagasse promotes the efficient metallization of magnetite concentrate without the external addition of a reducing agent. Therefore, this biomass is a technical suitable alternative to replace fossil fuels in steelmaking.     

X-linked Cornelia de Lange syndrome owing to SMC1L1 mutations

Cornelia de Lange syndrome is a multisystem developmental disorder characterized by facial dysmorphisms, upper limb abnormalities, growth delay and cognitive retardation. Mutations in the NIPBL gene, a component of the cohesin complex, account for approximately half of the affected individuals. We report here that mutations in SMC1L1 (also known as SMC1), which encodes a different subunit of the cohesin complex, are responsible for CdLS in three male members of an affected family and in one sporadic case.     

Loss of redundant gene expression after polyploidization in plants

Summary Based on chromosomal location data of genes encoding 28 biochemical systems in allohexaploid wheat,Triticum aestivum L. (genomes AABBDD), it is concluded that the proportions of systems controlled by triplicate, duplicate, and single loci are 57%, 25%, and 18% respectively.     

Identification of a new gene mutated in Fraser syndrome and mouse myelencephalic blebs

Fraser syndrome is a recessive, multisystem disorder presenting with cryptophthalmos, syndactyly and renal defects and associated with loss-of-function mutations of the extracellular matrix protein FRAS1. Fras1 mutant mice have a blebbed phenotype characterized by intrauterine epithelial fragility generating serous and, later, hemorrhagic blisters. The myelencephalic blebs (my) strain has a similar phenotype. We mapped my to Frem2, a gene related to Fras1 and Frem1, and showed that a Frem2 gene-trap mutation was allelic to my. Expression of Frem2 in adult kidneys correlated with cyst formation in my homozygotes, indicating that the gene is required for maintaining the differentiated state of renal epithelia. Two individuals with Fraser syndrome were homozygous with respect to the same missense mutation of FREM2, confirming genetic heterogeneity. This is the only missense mutation reported in any blebbing mutant or individual with Fraser syndrome, suggesting that calcium binding in the CALXbeta-cadherin motif is important for normal functioning of FREM2.     

Botany: floral fluorescence effect

The way flowers appear to insects is crucial for pollination. Here we describe an internal light-filtering effect in the flowers of Mirabilis jalapa, in which the visible fluorescence emitted by one pigment, a yellow betaxanthin, is absorbed by another, a violet betacyanin, to create a contrasting fluorescent pattern on the flower's petals. This finding opens up new possibilities for pollinator perception as fluorescence has not previously been considered as a potential signal in flowers.