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PubMedDate not supplied

Parallel auditory vestibular evoked neurogenic and myogenic potential results in a case of peripheral vestibular dysfunction, showing that the former originates from the vestibular system.

PURPOSE: Vestibular evoked myogenic potentials (VEMPs) uses high intensity clicks with recording from the tonically active sternocleidomastoid muscle, taking advantage of the close proximity of the saccule to the oval window. Our group has used the same stimulus to record Vestibular Evoked Neurogenic Potentials (VENPs) directly from the brain. VEMPs are now regarded the electrophysiological gold standard in peripheral vestibular system examination. We present a case of peripheral vestibular dysfunction to show that both VEMPs and VENPs provide similar results during recovery. METHODS: A case of Meniere's Disease in recovery is examined. VEMPs were recorded using a 105 dB nHL click stimulus from the ipsilateral sternocleidomastoid muscle. VENPs were recorded using an ipsilateral parietal to Fpz montage and a 1 kHz tone-pip stimulus. Standard BAEPs and threshold latency series (TLS) were performed. RESULTS: VEMP and VENP were unobtainable from the left side at initial presentation in a patient with Meniere's Disease, with normal BAEP and TLS bilaterally. After one month of therapy both the VEMP and VENP normalized. CONCLUSIONS: As VEMPs are known to originate from the vestibular system, the parallel VENP result suggests the same for the latter VENP may prove to be useful and complement VEMP in determining vestibular dysfunction.

Evoked Potentials, Auditory↗

PubMed2006

[Analysis of the characters of organic matter in water using spectral fluorescence signature and fitting Gaussian].

A sort of analytical method of fast diagnosis of organic matter in water is discussed. The total luminescence spectra (TLS) of water samples in combination with laser induced fluorescence (LIF) measurements using 532 nm wavelength excitation source were measured in the laboratory. The spectra of dissolved organic matter (DOM) and chlorophyll a (Chl a) were pointed out and obtained with spectral fluorescence signature (SFS) technique. The spectrum of water Raman scattering and fluorescence of DOM and Chl a were separated from TLS with fiting Gaussian of the least squares method, and a high correlation coefficient excelling 0.996 4 was obtained. The results indicated the possibilities of water quality monitoring in real time and on line based upon SFS technique and fitting Gaussian of the least squares method.

Environmental Monitoring↗

PubMed1991

Radiation induced acute tumor lysis syndrome in the bone marrow transplant setting.

Patients with highly chemo-responsive malignancies have for some time been known to be at risk for life threatening acute tumor lysis syndrome (TLS). This report describes two cases involving acute TLS in patients with malignant diseases which had become refractory to therapy and received initial total body irradiation as a part of the preparative regimen for allogeneic bone marrow transplantation.

Acute Disease↗

PubMed1984

[Primary and secondary (reactive) thymus hyperplasia in cases of sudden death].

Two post-mortem cases of thymic hyperplasia are studied. The death of patients occurred suddenly, from an acute hypocorticoidism developing in connection with a surgical operation. In the 1st case (a woman of 27) the thymicolymphatic state (TLS) with anomalies of sex organs was observed. A sudden death occurred 2 hours after the operation of colpopoiesis from the pelvic peritoneum. In the 2nd case (a woman of 30) a sudden death occurred at the end of cesarean operation and extraction of two fetuses (the pregnancy was of a normal duration). The patient had hyperprolactinemia and infertility for 8 years and for this reason was treated with parlodel (total dose 780 mg within 4 months). Acute hypocorticoidism in the 1st case was connected with the adrenal hypoplasia characteristic of TLS in which thymic hyperplasia is a manifestation of a congenital immune-endocrine insufficiency. In the 2nd case hypocorticoidism was due to the adrenal atrophy developing apparently as a result of focal sclerosis of the adenohypophysis. Its development may be explained by the disintegration of prolactin-secreting cells resulting from parlodel treatment. Hyperplasia of the thymus in this case is secondary and reflects the degree of adrenal insufficiency.

Adrenal Cortex↗

PubMed1995

Translocation t(12;16)(q13;p11) in myxoid liposarcoma and round cell liposarcoma: molecular and cytogenetic analysis.

Translocation t(12;16)(q13;p11) is regarded as a diagnostic marker for myxoid liposarcoma. Cytogenetic data on round cell liposarcomas and combined myxoid and round cell tumors is scarce, and the genetic basis of progression of myxoid tumors to high grade, round cell lesions is unknown. We have accumulated six round cell, four combined myxoid and round cell, and three myxoid liposarcomas for analysis. t(12;16)(q13;p11) was present in three round cell lesions and was detectable in all of the tumors by DNA analysis. In each tumor type, the CHOP gene in 12q13 was rearranged and fused to the TLS gene in 16p11. A variant TLS-CHOP RNA transcript was detected by polymerase chain reaction but did not correlate with clinicopathological data. No distinguishing cytogenetic or molecular markers for round cell or mixed lesions were found. The histogenic and genetic relatedness of myxoid and round cell liposarcomas is apparent from these data.

Adult↗

PubMed1994

Fusion of the FUS gene with ERG in acute myeloid leukemia with t(16;21)(p11;q22).

It has been shown that the gene ERG in 21q22 is rearranged in the t(16;21)(p11;q22) associated with acute myeloid leukemia (AML). ERG is a member of the ETS gene family and is fused with EWS in a subset of Ewing's sarcomas. EWS in 22q12 has a very high homology with FUS (also called TLS) in 16p11; the latter gene is rearranged in the t(12;16)(q13;p11) that characterizes myxoid liposarcoma. To investigate whether FUS is involved in the t(16;21) of AML, we used the Southern blot technique and polymerase chain reaction (PCR) to examine the bone marrow of a 3-year-old boy with a t(16;21)(p11;q22)-positive AML. Hybridization of Southern blot filters containing digested DNA with probes for FUS and ERG showed both germline and aberrant fragments. Using specific primers for the 5' part of FUS and the 3' part of ERG, we amplified a 4.4 kb genomic FUS/ERG DNA fragment from the leukemic sample. In a second PCR experiment, in which we used primers upstream of the 5' part of ERG and downstream of the 3' part of FUS, a 5.6 kb fragment was amplified. Blotting and hybridization with specific probes for FUS and ERG revealed that the amplified fragments consisted of FUS/ERG and ERG/FUS hybrid DNA. Both PCR fragments, when used as probes, detected germline ERG and FUS as well as aberrant fragments on Southern blot filters. The results suggest that the t(16;21) in AML leads to rearrangement and fusion of the FUS and ERG genes.(ABSTRACT TRUNCATED AT 250 WORDS)

Base Sequence↗

PubMed2006

EEG-fMRI using z-shimming in patients with temporal lobe epilepsy.

PURPOSE: To use z-shimming, a technique that reduces signal loss due to susceptibility artifacts that can result in reduced or absent activation in electroencephalography (EEG) functional MRI (fMRI) sessions in patients with temporal lobe epilepsy (TLE), to determine whether it would result in an increased ability to detect significant regions of blood oxygenation level-dependent (BOLD) signal change. MATERIALS AND METHODS: Eight patients with TL EEG spikes underwent an EEG-fMRI scanning session using z-shimming. The signal intensities in the z-shimmed images were compared with those in the standard images. BOLD activation maps were created from the two sets of images using the timings of the spikes observed on the EEG. RESULTS: The mean signal increase in the TLs as a result of z-shimming was 45.9%+/-4.5%. The percentage of TL voxels above a brain intensity threshold rose from 66.1%+/-7.6% to 77.6%+/-5.7%. This appreciable increase in signal did not lead to any significant differences in the statistical maps created with the two sets of functional images. CONCLUSION: The results suggest that loss of signal is not the limiting factor for the detection of spike-related BOLD signal changes in patients with TLE activity.

Algorithms↗

PubMed2004

Theoretical analysis of the effects of viscous losses and abdominal straining on urinary outlet function.

AIMS: The aim of this study was to theoretically explore the relationship between the tube law (TL) of the urethra and the pressure-flow (p-Q) relationship during micturition. The understanding of this relation is important for the evaluation of outlet obstruction by urodynamic interpretation of p-Q plots. METHODS: We simulated the outlet function by a lumped theoretical model, and suggested how the TL can be used to quantitatively predict the p-Q relationship of the system. Our analysis considered the relation between the TL and the steady state p-Q plot for various TLs including the hysteresis of the pressure-area relationship. RESULTS AND CONCLUSIONS: The inclusion of pressure losses distal to the flow controlling zone and experimentally measured p-A relations of the urethra in the model lead to the predictions that flow in the flow controlling zone is not always critical but is often in the sub-critical range, that an increase in abdominal pressure can increase the flow under certain conditions, and that hysteresis in the pressure-area relation is correlated to the hysteresis in the p-Q plot.

Abdomen↗

PubMed2004

Outcome of children with B cell lymphoma in Venezuela with the LMB-89 protocol.

BACKGROUND: We analyzed the results of the LMB-89 protocol performed in seven centers in Venezuela in 96 children having B-cell non-Hodgkin lymphoma treated from 1995 to 2002. PROCEDURE: Mean age was 7.1 years with 71 (74%) been male. Eighty-two patients (85%) had diffuse small cell lymphoma Burkitt and Burkitt-like, and 14 (15%) had diffuse large B-cell lymphoma. Initial disease sites included the abdomen in 67%, peripheral nodes in 8%, and mediastinal in 4%. Treatment was directed to risk groups as described for LMB-89 protocol. Group A: seven patients (7%), group B: 80 patients (83%), and group C: nine patients (9%). RESULTS: Mean follow-up was 35 +/- 31 months. Complete remission (CR) occurred in 70 patients (73%); four patients (6%) had relapse during the first year and ten patients (10%) had progressive disease. Overall survival (OS) and event free survival (EFS) were 85 and 80% at 1 year, and 82 and 75% at 2 years, respectively. The EFS by therapeutic groups at 3 years was A: 100%; B: 76%, and C: 56%. TOXICITY: neutropenia in 75%, thrombocytopenia in 63%, febrile neutropenia in 39%. Viral infections: hepatitis B in 20%, hepatitis C in 2%, and Herpes zoster in 3%. Tumor lysis syndrome (TLS) occurred in 9% during induction phase with a high mortality of 44% (urate-oxidase was available only at the end of the study). CONCLUSIONS: The high mortality rate during induction phase prohibited a better EFS. Prophylactic use of xantine-oxidase may improve future results. The high incidence of hepatitis B requires a vaccination program.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed2006

An in vitro enzymatic assay coupled to proteomics analysis reveals a new DNA processing activity for Ewing sarcoma and TAF(II)68 proteins.

Based on structural and functional similarities, translocated in liposarcoma/fusion (TLS/FUS) protein, Ewing sarcoma (EWS) protein and human TATA binding protein-associated factor (hTAF(II)68) have been grouped in the TLS-EWS-TAF(II)68 (TET) protein family. Translocations involving their genes lead to sarcomas. Polypyrimidine tract-binding protein-associated splicing factor (PSF), although not grouped in this family, presents structural and functional similarities with TET proteins and is involved in translocation leading to carcinoma. Beside their role in RNA metabolism, the precise cellular functions of these multifunctional proteins are not yet fully elucidated. We previously showed that both TLS/FUS and PSF display activities able to pair homologous DNA on membrane in an in vitro assay. In the present study, we address the question whether EWS and hTAF(II)68 also display pairing on membrane activities, and to a larger extent whether other proteins also exhibit such activity. We applied the pairing on membrane assay to 2-DE coupled to MS analysis for a global screening of DNA pairing on membrane activities. In addition to TLS/FUS and PSF, this test allowed us to identify EWS and hTAF(II)68, but no other proteins, indicating a feature specific to a protein family whose members share extensive structural similarities. This common activity suggests a role for TET proteins and PSF in genome plasticity control.

Cell Line, Transformed↗

PubMed1999

CHOP/GADD153 and methionyl-tRNA synthetase (MetRS) genes overlap in a conserved region that controls mRNA stability.

The transcription factor CHOP is involved in the regulation of the cell division cycle and the control of programmed cell death in response to cellular stress. CHOP expression has been linked with several forms of cancer. A reciprocal translocation between the CHOP and TLS RNA-binding protein gene results in myxoid liposarcoma and amplifications of the CHOP gene are associated with solid tumors including several types of sarcomas. Here we report the mapping of the methionyl tRNA synthetase (MetRS) gene to the identical 12q13 locus where the CHOP gene had previously been mapped. PCR analysis demonstrates a tail-to-tail overlap of both genes over a 55-bp region. As a result the two mRNAs share a 3' UTR complementary sequence allowing an in vivo interaction between the two mRNAs. An AU-rich regulatory element (ARE) known to control mRNA stability resides in the overlapping sequence. To test for functional significance of the ARE a luciferase reporter plasmid containing the 3'UTR of CHOP was constructed. Transfection experiments in NIH-3T3 cells show that CHOP 3'UTR confers a significantly lower activity than a control reporter or a reporter in which the region overlapping the MetRS mRNA is deleted. The conservation of this overlapping of the CHOP and MetRS genes and the role of their complementary sequence in the control of mRNA stability suggest the existence of a functional link between the expression of these two genes.

3' Untranslated Regions↗

PubMed1996

A nuclear protein regulated during the transition from active to quiescent phenotype in cultured endothelial cells.

Pigpen is a 67-kDa Sepharose-binding molecule isolated from mammalian endothelial and retinal pigmented epithelial cells. The protein is distributed nonhomogeneously in the nucleus, exhibiting diffuse staining throughout (excluding nucleoli), together with a small number of intensely stained focal points, or granules, and punctate staining along the nuclear envelope. Pigpen was absent or greatly attenuated in the nonepithelial cell types we examined, including fibroblasts, myeloma, and astroglia. cDNA sequence analysis revealed a positively charged molecule with an RNP-CS RNA-binding domain, 19 RGG repeats, and a consensus tyrosine phosphorylation site in the C-terminus. The amino terminal portion of the molecule is characterized by 7 glutamine-rich hexapeptide repeats similar to those found in the transactivation domain of known transcription activators. Pigpen has a high level of identity with the FUS gene product, TLS (Translocated in Liposarcoma; Crozat et al, 1993; Rabbits et al., 1993), a new member of the EWS family of proteins. Expression of pigpen is regulated during the transition between active and quiescent endothelial cell phenotypes. Both mRNA and overall protein levels are maintained at a steady level in actively growing cells. The number of nuclear granules increases as cultures approach confluency. When cells reach confluency, overall expression is sharply reduced and the number of nuclear focal points declines gradually. We observed that reactivation of endothelial cells locally by wounding of confluent cultures resulted in a spatially restricted reactivation of pigpen expression. This pattern of expression, taken together with structural data, suggests that pigpen may function in the growth and differentiation of endothelial cells during angiogenesis.

Amino Acid Sequence↗

PubMed2000

Nonconvulsive Kainic Acid-Induced Seizures Elicit Age-Dependent Impairment of Memory for the Elevated Plus-Maze.

The goal of this study was to evaluate changes in spatial learning in adult and immature rats during and after nonconvulsive seizures. An elevated plus-maze was used in 18- and 25-day-old and adult rats. Kainic acid (KA 6 mg/kg) was administered 60 minutes before the first exposure (Experiment 1) or after a 3-day pretraining (Experiment 2, only adult rats). Animals were retested three times with 24-hour intervals. EEG activity was monitored in 18-day-old rats. KA prolonged the transfer latency (TL) in all age groups. In the youngest group the TL was prolonged 24 hours after KA when epileptic EEG graphoelements were still registered. In both older groups, prolonged TL was measured only 60 minutes after KA. In the pretrained adults, significantly prolonged TLs persisted for 24 hours after KA. KA changed the performance of adult and immature rats in the elevated plus maze not only during nonconvulsive seizures but also 24 hours later.

Journal Article↗

PubMed2002

The localization of nuclear DNA helicase II in different nuclear compartments is linked to transcription.

Nuclear DNA helicase II (NDH II) is a member of the DEAH superfamily of helicases and functions as a pre-mRNA- and mRNA-binding protein in human cells. Here we report for the first time that human NDH II is associated with the nucleolus of transformed and nontransformed cells as shown by immunofluorescence and by ultrastructural studies. When RNA polymerase II (POL II) transcription is inhibited, NDH II highly accumulates in the nucleolus and shows predominant association with subdomains in DFC and in a portion of GC attached to DFC. Furthermore, these subdomains completely co-localize with mRNA-binding protein TLS. In addition, we show that nucleolar accumulation of NDH II is closely related to G(0)-phase growth arrest in human fibroblasts. Thus, the nucleolar localization of NDH II depends upon the metabolic state of the cell. Based on the data we propose that NDH II operates in both nucleoplasmic and nucleolar mode, and that its redistribution reflects accumulations indicating a possible cycling of NDH II between nucleoplasm and the nucleolus. The nucleolus can serve as a temporary storage or recycling center for NDH II. Possible functions of NDH II in pre-rRNA biogenesis, or in nucleolar mRNA metabolism, are also discussed.

Adenosine Triphosphatases↗

PubMed1996

Expression patterns of the human sarcoma-associated genes FUS and EWS and the genomic structure of FUS.

FUS (TLS) was first identified as the 5'-part of a fusion gene with CHOP (GADD153, DDIT3) in myxoid liposarcomas with t(12; 16)(q13; p11). Homologies were found with the EWS oncogene, which is rearranged in Ewing sarcomas and other neoplasias. The genomic structure of FUS shows extensive similarities with that of EWS, but the exon/intron structures differ in the 5' parts, and overall FUS is smaller than EWS. Exon 3 of FUS corresponds to exons 3 and 4 in EWS. FUS exons 4-6 correspond to EWS exons 5-8. Exons 7 to 15 of FUS are very similar to those in EWS, although the EWS exons are larger than the corresponding FUS exons. FUS and EWS were expressed in all tissues investigated. The transcripts were stable within the 160-min half-life experiments. No or little variation in FUS or EWS expression was seen when resting lymphocytes were activated. These observations indicate that FUS and EWS belong to the housekeeping type of genes. This view is supported by the presence of the housekeeping gene type of promoter region in both genes.

Amino Acid Sequence↗

PubMed2000

The 1.0 A crystal structure of Ca(2+)-bound calmodulin: an analysis of disorder and implications for functionally relevant plasticity.

Calmodulin (CaM) is a highly conserved 17 kDa eukaryotic protein that can bind specifically to over 100 protein targets in response to a Ca(2+) signal. Ca(2+)-CaM requires a considerable degree of structural plasticity to accomplish this physiological role; however, the nature and extent of this plasticity remain poorly characterized. Here, we present the 1.0 A crystal structure of Paramecium tetraurelia Ca(2+)-CaM, including 36 discretely disordered residues and a fifth Ca(2+) that mediates a crystal contact. The 36 discretely disordered residues are located primarily in the central helix and the two hydrophobic binding pockets, and reveal correlated side-chain disorder that may assist target-specific deformation of the binding pockets. Evidence of domain displacements and discrete backbone disorder is provided by translation-libration-screw (TLS) analysis and multiconformer models of protein disorder, respectively. In total, the evidence for disorder at every accessible length-scale in Ca(2+)-CaM suggests that the protein occupies a large number of hierarchically arranged conformational substates in the crystalline environment and may sample a quasi-continuous spectrum of conformations in solution. Therefore, we propose that the functionally distinct forms of CaM are less structurally distinct than previously believed, and that the different activities of CaM in response to Ca(2+) may result primarily from Ca(2+)-mediated alterations in the dynamics of the protein.

Adenosine Diphosphate↗

PubMed2004

Forehead is as sensitive as finger pulse oximetry during general anesthesia.

PURPOSE: To compare the performance of a forehead probe to a conventional finger pulse oximetry probe in anesthetized patients. METHODS: Eighteen patients participated in the study. Each probe was connected to a Nellcor N-550 pulse oximeter. Anesthesia was induced and maintained with propofol. After intubation, the patients received air to achieve a steady-state of peripheral arterial oxygen saturation (SpO(2)). Ventilation was interrupted to induce a hypoxic state. As soon as one of the two SpO(2)'s decreased to 90%, the patients' lungs were ventilated with 100% oxygen. To evaluate the performance of the two pulse oximeters, time to the lowest (TL), time of recovery (TR) and lag times to beginning of SpO(2) decrease (Lag) were measured. RESULTS: There were no significant differences in TL and TR between forehead and finger pulse oximetry under normal perfusion conditions during general anesthesia. When the axillary artery was compressed to mimic reduced peripheral perfusion, SpO(2) in the forehead decreased sooner than in the finger during hypoxia. The forehead and finger TLs were similar, however, TR was significantly longer in the finger. CONCLUSION: The forehead SpO(2) sensor can be used as an alternative to the conventional finger sensor during general anesthesia.

Anesthesia, General↗

PubMed2001

Acute tumor lysis syndrome induced by high-dose corticosteroids in a patient with chronic lymphatic leukemia.

Acute tumor lysis syndrome (TLS) has been reported in hematological malignancies, such as aggressive non-Hodgkin's lymphoma, acute lymphoblastic leukemia, and rarely, in other malignancies (solid tumors) in association with the administration of cytotoxic therapy. We report a case of a patient with chronic lymphatic leukemia (CLL) who developed autoimmune hemolytic anemia treated by high dose corticosteroids and, following this treatment, developed acute tumor lysis syndrome. Only one similar case has been reported recently. Clinicians should be aware that corticosteroids alone may produce this potentially life-threatening complication.

Acute Disease↗