Laryngeal Schwannoma: A Case Presentation and Review of the Mayo Clinic Experience.

Objectives: The aim of this study was to clarify the nature of laryngeal schwannomas through review of the experience of a single institution during a 104-year period.

Study Design: This is a retrospective case series.

Methods: The Mayo Clinic, Rochester, Minnesota clinical and surgical pathology database was reviewed for the years 1985-2011.

Genome-wide identification of mRNAs associated with the protein SMN whose depletion decreases their axonal localization.

Spinal muscular atrophy is a neuromuscular disease resulting from mutations in the SMN1 gene, which encodes the survival motor neuron (SMN) protein. SMN is part of a large complex that is essential for the biogenesis of spliceosomal small nuclear RNPs. SMN also colocalizes with mRNAs in granules that are actively transported in neuronal processes, supporting the hypothesis that SMN is involved in axonal trafficking of mRNPs.

Impaired minor tri-snRNP assembly generates differential splicing defects of U12-type introns in lymphoblasts derived from a type I SMA patient.

The survival of motor neuron (SMN) protein is essential for cytoplasmic assembly of spliceosomal snRNPs. Although the normal proportion of endogenous snRNAs is unevenly altered in spinal muscular atrophy (SMA) tissues, the biogenesis of individual snRNPs is not dramatically affected in SMN-deficient cells. The SMN protein is also required for normal Cajal body (CB) formation, but the functional consequences of CB disruption upon SMN deficiency have not yet been analyzed at the level of macromolecular snRNPs assembly.

HSP90 and its R2TP/Prefoldin-like cochaperone are involved in the cytoplasmic assembly of RNA polymerase II.

RNA polymerases are key multisubunit cellular enzymes. Microscopy studies indicated that RNA polymerase I assembles near its promoter. However, the mechanism by which RNA polymerase II is assembled from its 12 subunits remains unclear.

Specific splicing defects in S. pombe carrying a degron allele of the Survival of Motor Neuron gene.

Spinal muscular atrophy results from deletions or mutations in the survival of motor neuron (SMN1) gene. The SMN protein has an essential role in the biogenesis of spliceosomal snRNPs, but the link between a defect in this process and specific splicing inhibition of pre-mRNAs has not been established. In this study, we report the construction of a temperature-degron (td) allele of the Schizosaccharomyces pombe SMN protein and show that its depletion at 37 degrees C affects splicing and formation of U1, U2, U4 and U5 snRNPs, but not of U6 and U3 ribonucleoproteins.

Characterization of a short isoform of human Tgs1 hypermethylase associating with small nucleolar ribonucleoprotein core proteins and produced by limited proteolytic processing.

Tgs1 is the hypermethylase responsible for m(3)G cap formation of U small nuclear RNAs (U snRNAs) and small nucleolar RNAs (snoRNAs). In vertebrates, hypermethylation of snRNAs occurs in the cytoplasm, whereas this process takes place in the nucleus for snoRNAs. Accordingly, the hypermethylase is found in both compartments with a diffuse localization in the cytoplasm and a concentration in Cajal bodies in the nucleoplasm.

Differential regulation of estrogen receptor alpha turnover and transactivation by Mdm2 and stress-inducing agents.

In mammalian cells, the level of estrogen receptor alpha (ERalpha) is rapidly decreased upon estrogen treatment, and this regulation involves proteasome degradation. Using different approaches, we showed that the Mdm2 oncogenic ubiquitin-ligase directly interacts with ERalpha in a ternary complex with p53 and is involved in the regulation of ERalpha turnover (both in the absence or presence of estrogens). Several lines of evidence indicated that this effect of Mdm2 required its ubiquitin-ligase activity and involved the ubiquitin/proteasome pathway.

Depletion of SMN by RNA interference in HeLa cells induces defects in Cajal body formation.

Neuronal degeneration in spinal muscular atrophy (SMA) is caused by reduced expression of the survival of motor neuron (SMN) protein. The SMN protein is ubiquitously expressed and is present both in the cytoplasm and in the nucleus where it localizes in Cajal bodies. The SMN complex plays an essential role for the biogenesis of spliceosomal U-snRNPs.

[District program to improve the cardiovascular risk of resistant hypertensive patients in general medicine].

Purpose: To try to improve the cardiovascular risk of resistant hypertensive patients in general medicine in Brittany after using french hypertension recommendations.

Methods: 581 hypertensive patients under 3 antihypertensive drugs have asked for an exoneration of the patients' social contribution: 297 (51%) were uncontrolled at their general practitioner (GP) among whom 106 (36%) have refused to take part in the program. 191 resistant hypertensive patients followed by 170 different GP have been pre-included.

Nuclear localization properties of a conserved protuberance in the Sm core complex.

The nuclear import signal of snRNPs is composed of two essential components, the m(3)G cap structure of the snRNA and the Sm core NLS carried by the Sm protein core complex. We have previously proposed that, in yeast, this last determinant is represented by a basic-rich protuberance formed by the C-terminal extensions of Sm proteins. In mammals, as well as in other organisms, this component has not yet been precisely defined.

Identification of NY-ESO-1, MAGE-1, and MAGE-3 in head and neck squamous cell carcinoma.

Background: Certain tumor antigens have been identified that stimulate an immune response, thus making them targets for immunotherapy. NY-ESO-1, MAGE-1, and MAGE-3 are such antigens. This study was undertaken to determine their presence or absence in head and neck squamous cell cancers and to correlate this with patient characteristics.

MDM2: life without p53.

The MDM2 protein suppresses the ability of p53 to inhibit cellular proliferation or to induce cell death. This property underlies the oncogenic potential of MDM2, which is overexpressed in various human tumours. However, MDM2 also has p53-independent activities, which we focus on here.

Preferential expression of Mdm2 oncogene during the development of neural crest and its derivatives in mouse early embryogenesis.

The Mdm2 oncoprotein acts as the principal negative regulator of p53 activities and is essential for its control during mouse early development, at least before implantation. We analyzed Mdm2 expression between 7.5 and 9 days post-coitum (dpc) by whole-mount in situ hybridization and report here a novel expression pattern during neural crest development.

The Mdm2 gene of zebrafish (Danio rerio): preferential expression during development of neural and muscular tissues, and absence of tumor formation after overexpression of its cDNA during early embryogenesis.

The Mdm2 protein is most probably the main negative cellular regulator of the p53 tumor-suppressor protein. It was found to be overexpressed in a great number of human tumors and is considered as a potential target for anti-tumor therapies. Mdm2 is an essential gene in mice, yet its role in normal development and tissue differentiation is unknown.

Mdm2: keeping p53 under control.

The Mdm2 gene is overexpressed in several human tumors. The oncogenic potential of Mdm2 is partially explained by the inhibition of the activity of the tumor suppressor protein p53. Determination of the three-dimensional structure of complexes between Mdm2 and the N-terminal p53 peptide provided a molecular basis for the inhibition of the transcriptional function of p53 by Mdm2.

MDM-2 protein is expressed in different layers of normal human skin.

MDM-2 is one of the target genes of the p53 tumor suppressor protein. Its best characterized function is found in the inhibition of p53's ability to modulate transcription. Deregulated expression of MDM-2 could thus at least partially substitute for p53 mutation in the process of tumorigenesis.

Regulation of pre-mRNA processing by src.

Background: Changes in gene expression in response to external signals provide a key mechanisms for the regulation of higher eukaryotic cell functions. The importance of transcriptional control in the response of cells to growth factors and cytokines has been extensively documented, but gene expression has also been shown to be controlled at other levels, such as the stability of mRNA in the cytoplasm, its localization and translation. By contrast to transcriptional control, little is known of the contribution of pre-mRNA nuclear processing to the regulation of gene expression, as most of our knowledge of pre-mRNA processing in vivo is indirect, being inferred from comparisons of transcription rates and levels of mRNA accumulation.

[The sampan dwellers of the Vi Da district (Hue, 1993). Results of a survey preliminary to humanitarian aid intervention].

This survey is a preliminary step in a cooperative health-care programme, run by the association Le Pélican. It was conducted in July 1993 among the sampan dwellers in the district of Vi Da (Hué), Vietnam. The People's Committee of the town of Hué hopes to improve the living conditions of the inhabitants of the district.

Recurrences after endoscopic management of early (T1) glottic carcinoma.

From 1976 to 1986, 106 patients with early glottic carcinoma were managed endoscopically at our institution. Twenty-four (23%) patients required retreatment of the larynx for local recurrences or new primary lesions after initial endoscopic management. The probability of remaining free of local recurrence 3 years after primary surgery was estimated to be 0.

Allergic fungal sinusitis: the Mayo Clinic experience.

The diagnosis of allergic fungal sinusitis (AFS) is difficult to establish. The clinical presentation is not diagnostic. We define current criteria for diagnosis and the role of total and specific immunoglobulin E (IgE) and immunoglobulin G (IgG) levels in the disease process and review the clinical features of this disease.