Pleiotropic and diverse expression of ZFHX1B gene transcripts during mouse and human development supports the various clinical manifestations of the "Mowat-Wilson" syndrome.

ZFHX1B encodes Smad-interacting protein 1, a transcriptional corepressor involved in the transforming growth factors beta (TGFbeta) signaling pathway. ZFHX1B mutations cause a complex developmental phenotype characterized by severe mental retardation (MR) and multiple congenital defects. We compared the distribution of ZFHX1B transcripts during mouse and human embryogenesis as well as in adult mice and humans.

Proteomic analysis of lung biopsies: Differential protein expression profile between peritumoral and tumoral tissue.

The cellular proteome shows a dynamic profile and is subjected to changes in response to various stimuli and disease progression. Lung cancer remains the leading cause of cancer death in industrialized countries. In an attempt to find new disease markers, patients suffering from lung carcinoma have been selected to achieve differential protein expression patterns between normal and neoplasic tissue.

[Neurosurgical embryology. Part 7: Development of the spinal cord, the spine and the posterior fossa].

The spinal cord arises from the most caudal domain of the neural tube whereas the vertebrae develop from the para-axial mesoderm (namely the somites). The development of the spinal cord and the vertebrae is so intimately linked that it is more convenient to present it in the same chapter. The neural tube is formed from the neural plate during neurulation.

[Neurosurgical embryology. Part 6: The principal models that can be used in developmental biology].

To study the mechanisms controlling embryonic development, experimental analyses must be performed on animal models. There are different species that are currently used as models in developmental biology. In invertebrates, drosophila remains a very useful model thanks to its rapid development and to the extensive study of genes involved in the control of its development.

[Neurosurgical embryology. Part 5: Temporal landmarks of the development of the central nervous system in humans].

The establishment of temporal landmarks of the development of the central nervous system was the primary goal of early human embryologists. The description of these landmarks has lost much of its interest since it is now largely accepted that the temporal profile of development is at best a poor indicator of the origin of malformative syndromes. However, we propose here a rapid review describing the principal data available.

[Physical activity and cardiovascular and metabolic risk factors in general population].

Background And Objective: Our purpose was to evaluate in a cross-section, populational study the association between daily physical activity and various metabolic and cardiovascular disease risk factors.

Patients And Method: A total of 1226 randomly selected persons (aged 18-65 years) from a town in southeast Spain were evaluated for studying the association between the level of daily physical activity, both in and out of working hours, and the following variables: body mass index, plasma lipids, hypertension, obesity, altered baseline glycemia, and abnormal glucose tolerance.

Results: The intensity of the physical activity at work was related negatively with insulin resistance and levels of LDL cholesterol, and positively with HDL cholesterol.

[Embryology of the sphenoid bone].

The sphenoid bone represents a complex structure in terms of anatomy and embryology. Indeed, it is formed by the fusion of different primordia whose embryonic origins are different. In mammals, it is possible to distinguish two components of this bone: the orbitosphenoid and the basi-post-sphenoid derive from the cephalic mesoderm whereas the alisphenoid and the basi-pre-sphenoid are from neural crest cell origin.

[Neurosurgical embryology. Part 4: What are stem-cells?].

Stem-cells have been identified in the adult human brain in two zones which are the subventricular zone and the gyrus dentatus of the hippocampus. Improvement of techniques aimed to identify, to localize and to follow the lineage of these cells have been crucial to the understanding of the following processes: a) identification of cellular proliferation, b) specific immunostaining of differentiated glial and neuronal cells, c) transplantations to decipher between intrinsic stem-cell properties and influence of the environment on the fate of the cell. Furthermore, it seems that stem-cells from other sources than the brain can differentiate into neurons both in vitro and in vivo.

[Neurosurgical Embryology. Part 3: Molecular control of corpus callosum development].

The corpus callosum is the most important cerebral commissure allowing axonal fibres to cross the midline. Corpus callosum agenesis is an important condition in man that can reveal numerous genetic syndromes. The corpus callosum develops from the commissural plate, a dorsal region of the telencephalon.

[Neurosurgical Embryology. Part 2: Recent data on normal and pathological development of the cortex].

Understanding how the cortex develops has gained very important new data thanks to both experimental and clinical studies. Experimental studies have shown that: --neurons are generated in the ventricular zone by asymmetric mitoses; --the first cortical region to differentiate is the so-called pre-plate that plays a major role in the control of neuroblasts migration; --neuroblasts arise according to an inside-outside gradient; they migrate either along the processes of radial cells or according to a new type of non radial migration; --all the cortical neurons are not generated by the dorsal telencephalon; some of these neurons derive from the basal telencephalon; --neuroblasts acquire their specificity during their way to the cortical plate. There are several genetic syndromes leading to a malformation of the cortex.

[Neurosurgical embryology. Part 1: Cell differentiation].

In pluricellular organisms, cell differentiation helps to decrease the total amount of energy needed for life. These differentiations can be evidenced at the tissular, the cellular or the molecular levels. Cell differentiation is a progressive process achieved during embryogenesis; different steps in the program can be described.

Establishment of embryonic neuroepithelial cell lines exhibiting an epiplastic expression pattern of region specific markers.

Neuroepithelial b2T cells were derived from the hindbrain and the spinal cord of mouse transgenic embryos, which expressed SV40 T antigen under the control of a Hoxb2 enhancer. Strikingly, b2T cell lines of either origin exhibit a very similar gene expression pattern, including markers of the hindbrain and the spinal cord, such as Hox genes, but not of more anterior cephalic regions. In addition, the broad expression pattern of b2T cells, probably linked to culture conditions, appeared to be appropriately modulated when the cells were reimplanted at different longitudinal levels into chick host embryos, suggesting that these cells are responsive to exogenous signalling mechanisms.

[The formation of neurons in the bone marrow, the dream of alchemy in the new millenium].

In rodents, bone marrow contains stem cells that have the potentiality to differentiate into mesodermal and non-mesodermal cells, both in vitro and in vivo. These cells can populate a wide panel of organs such as the liver, the brain, the lungs, the heart..

Immune responses in breast cancer patients immunized with an anti-idiotype antibody mimicking NeuGc-containing gangliosides.

A phase I clinical trial was conducted in patients with stage III/IV breast cancer who were treated with the anti-idiotype mAb 1E10 specific to an Ab1 mAb able to react specifically with N-glycolyl-containing gangliosides and with antigens expressed on human melanoma and breast carcinoma cells. Patients were treated with 1 or 2 mg of aluminum hydroxide-precipitated 1E10 mAb every other week for six injections. Two patients at each dose were reimmunized 7-9 months after completing the induction phase.

[Development and maturation of the pyramidal tract].

The pyramidal tract contains axons that originate from neurons located in layer 5 of the neocortex of the frontal areas 4 and 6 and of the parietal lobe. These neurons are generated during the first half of gestation in humans. The growth of these axons is highly regulated and the mechanisms that control this growth begin to be unravelled.

Preoperative lanreotide treatment for GH-secreting pituitary adenomas: effect on tumour volume and predictive factors of significant tumour shrinkage.

Objective: The objective of this open study of 104 patients was to determine whether the somatostatin analogue lanreotide shrinks GH-secreting adenomas and to identify the predictive factors of a significant tumour volume reduction (> 20%).

Patients: A total of 104 previously untreated and newly diagnosed acromegalic patients received the prolonged release (PR) formulation of lanreotide (lanreotide 30 mg, one intramuscular injection every 10 days) for either 1 (n = 84), 2 (n = 13), or 3 or more (n = 7) months before transsphenoidal surgery.

Measurements: Pituitary tumour volumes, tumour extension grade and possible cavernous sinus invasion were assessed in blinded conditions by a centralized team of radiologists.

Pharmacological evaluation of humanized anti-epidermal growth factor receptor, monoclonal antibody h-R3, in patients with advanced epithelial-derived cancer.

Epidermal growth factor receptor (EGFR) overexpression has been detected in many tumors of epithelial origin, and it is often associated with tumor growth advantages and poor prognosis. h-R3 is a genetically engineered humanized antibody (mAb) that recognizes an epitope located in the extracellular domain of human EGFR. The antibody exhibited potent in vitro and in vivo antitumor effect on EGFR overexpressing cell lines.

Rapid detection of 17p11.2 rearrangements by FISH without cell culture (direct FISH, DFISH): a prospective study of 130 patients with inherited peripheral neuropathies.

Charcot-Marie-Tooth (CMT) disease and hereditary neuropathy with pressure palsies (HNPP) are two frequent hereditary motor and sensory neuropathies. CMT is characterized by slowly progressive weakness and atrophy, primarily in peroneal and distal leg muscles. The most frequent form, CMT1A, is due, in most cases, to the duplication of a 1.

Epidermal growth factor-based cancer vaccine for non-small-cell lung cancer therapy.

Background: The role that growth factors and their receptors play in human cancer growth and progression makes them interesting targets for novel treatment modalities. Our approach consisted of active immunotherapy with the epidermal growth factor (EGF). Two pilot clinical trials were conducted to examine the safety and immunogenicity of a five-dose immunization protocol and to compare different adjuvants and treatment designs.

[Recent contributions to the establishment of the axes of the mammalian embryo].

The study of the establishment of embryonic axes during early development has shown that this process is a very early event (occurRing either during ovogenesis or during fertilization) for invertebrates and for lower vertebrates. In mammals, it was considered that this establishment appears late during development because of the great plasticity of blastomeres. Recent data in the mouse embryon show that the mammalian ovocyte is a polarized cell, the polar body corresponding to the animal pole of this cell.

Acromegaly Quality of Life Questionnaire (ACROQOL) a new health-related quality of life questionnaire for patients with acromegaly: development and psychometric properties.

Objective: To develop a disease-specific questionnaire suitable to measure health-related quality of life (HRQOL) in acromegaly (ACROQOL).

Design, Patients And Measurements: For the development of the ACROQOL questionnaire different sources of information were used: First, a literature search was performed to identify relevant papers describing the impact of acromegaly in HRQOL. Second, 10 endocrinologists identified the main domains of impact on HRQOL in patients with acromegaly.

[Control of the development of Onuf's spinal nucleus].

Onuf's nucleus is located at the sacral level of the spinal cord and is formed by the motoneurons innervating the perineal muscles. Both the number and the size of these neurons vary between males and females creating a so-called sexual dimorphism. This dimosphism is mediated by androgens.

A physical interaction between Gar1p and Rnt1pi is required for the nuclear import of H/ACA small nucleolar RNA-associated proteins.

During rRNA biogenesis, multiple RNA and protein substrates are modified and assembled through the coordinated activity of many factors. In Saccharomyces cerevisiae, the double-stranded RNA nuclease Rnt1p and the H/ACA snoRNA pseudouridylase complex participate in the transformation of the nascent pre-rRNA transcript into 35S pre-rRNA. Here we demonstrate the binding of a component of the H/ACA complex (Gar1p) to Rnt1p in vivo and in vitro in the absence of other factors.

Action of E. coli endotoxin, IL-1beta and TNF-alpha on antioxidant status of cultured hepatocytes.

We have previously reported that endotoxin induces in vivo oxidative stress in liver and a significant increase in hepatic and plasma glutathione concentrations during the acute phase of reversible endotoxic shock in rats. In the present study we examined the in vitro effects of E. coli 0111:B4 endotoxin (lipopolysaccharide, LPS), IL-1beta and TNF-alpha on antioxidant status of cultured hepatocytes in order to differentiate between the direct and mediated endotoxin action.