Vacuolated PAS-Positive Lymphocytes on Blood Smear: An Easy Screening Tool and a Possible Biomarker for Monitoring Therapeutic Responses in Late Onset Pompe Disease (LOPD).

Primary aim was to investigate the diagnostic value of PAS-positive vacuolated lymphocytes on blood smear in Late Onset Pompe Disease (LOPD) patients and, secondly, to evaluate its potential utility in monitoring treatment effects. We examined blood smear of 26 LOPD patients. We evaluated 10 treated and 16 untreated LOPD patients.

Myasthenia Gravis: Unusual Presentations and Diagnostic Pitfalls.

Background: Myasthenia gravis (MG) is an autoimmune disorder presenting with fluctuating, fatigable muscle weakness. Initial symptoms classically involve ocular and proximal limb muscles. Rarely, MG may onset with unusual features, so it can be misdiagnosed with other neuromuscular diseases.

Impaired Muscle Mitochondrial Biogenesis and Myogenesis in Spinal Muscular Atrophy.

Importance: The important depletion of mitochondrial DNA (mtDNA) and the general depression of mitochondrial respiratory chain complex levels (including complex II) have been confirmed, implying an increasing paucity of mitochondria in the muscle from patients with types I, II, and III spinal muscular atrophy (SMA-I, -II, and -III, respectively).

Objective: To investigate mitochondrial dysfunction in a large series of muscle biopsy samples from patients with SMA.

Design, Setting, And Participants: We studied quadriceps muscle samples from 24 patients with genetically documented SMA and paraspinal muscle samples from 3 patients with SMA-II undergoing surgery for scoliosis correction.

Yellow laser performance of Dy³⁺ in co-doped Dy,Tb:LiLuF₄.

We present laser results obtained from a Dy³⁺-Tb³⁺ co-doped LiLuF₄ crystal, pumped by a blue emitting InGaN laser diode, aiming for generation of a compact 578 nm source. We exploit the yellow Dy³⁺ transition ⁴F(9/2)⇒⁶H(13/2) to generate yellow laser emission. The lifetime of the lower laser level is quenched, via energy transfer, to co-doped Tb³⁺ ions in the fluoride crystal.

Multiple polarization orange and red laser emissions with Pr:BaY₂F₈.

We investigated the polarization of continuous-wave laser emission in the orange region, at 607 nm, and in the red region, at 639 nm and 643 nm, from a Pr:BaY2F8 (Pr:BYF) crystal, pumped by a 445 nm laser diode. We achieved linearly polarized emission along two optic axes of the crystal by changing its orientation with respect to the pump. Simultaneous emission of two orthogonal linear polarizations was observed in the orange region, at the same wavelength, and in the red region, with concurrent emission from the two separate lines.

Continuous-wave and Q-switched operation of a resonantly pumped Ho³⁺:KY₃F₁₀ laser.

We report continuous-wave and repetitively Q-switched operation of a resonantly pumped Ho3+:KY3F10 laser at room temperature. End pumped by a Tm3+-doped silica fiber laser operating at 1938 nm, a maximum laser power of 7.8 W was obtained at a wavelength of ∼2041  nm for 21 W of absorbed pump power, corresponding to a slope efficiency of 60.

In-band pumped Ho3+:KY3F10 2 μm laser.

We report the first observation to our knowledge of room-temperature continuous-wave laser operation on the (5)I(7)→(5)I(8) transition of Ho(3+) ions in a KY(3)F(10) single crystal. Using a Tm-doped silica fiber laser operating at 1938 nm as a pump source, a maximum laser power of 1.8 W was obtained at a wavelength of ~2040 nm for 27 W of absorbed pump power with a slope efficiency of 19.

Compact passively Q-switched diode-pumped Tm:LiLuF4 laser with 1.26 mJ output energy.

We demonstrate efficient continuous-wave (CW) and passively Q-switched Tm:LiLuF(4) laser operation near 1.9 μm. The CW slope efficiency reached 54.

Passively Q-switched Tm:YLF laser.

Stable passive Q-switching of a Tm:  LiYF4 laser is obtained using polycrystalline Cr2+:  ZnS as a saturable absorber. The achieved maximum pulse energy of 0.9 mJ and peak power of 65 kW for a pulse duration of ∼14  ns represent substantial improvement and highest values for a passively Q-switched diode-pumped Tm laser operating at ∼1.

High-efficiency 1.9 µm Tm3+:LiLuF4 thin-disk laser.

We present the first demonstration of a 792 nm diode-pumped Tm3+:LiLuF4 thin-disk laser operation around 1.9 μm. In multimode configuration, up to 21 W of output power and a maximal slope efficiency of 49% with an optical-to-optical efficiency of 40% was demonstrated.

Diode-pumped Pr:BaY2F8 continuous-wave orange laser.

We report the realization of the continuous wave laser emission in the orange at 607 nm from a Pr:BaY(2)F(8) (Pr:BYF) crystal pumped by a blue GaN laser diode. A maximal output power of 78 mW is obtained in a quasi-single transverse mode beam. The effect of reabsorption losses at the laser wavelength is also evidenced.

Anti-Stokes luminescence cooling of Tm3+ doped BaY2F8.

We report laser-induced cooling with thulium-doped BaY2F8 single crystals grown using the Czochralski technique. The spectroscopic characterization of the crystals has been used to evaluate the laser cooling performance of the samples. Cooling by 3 degrees below ambient temperature is obtained in a single-pass geometry with 4.

Analysis and classification of bacteria by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry and a chemometric approach.

Matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) is a useful technique for the identification of bacteria on the basis of their characteristic protein mass spectrum fingerprint. Highly standardized instrumental analytical performance and bacterial culture conditions are required to achieve useful information. A chemometric approach based on multivariate analysis techniques was developed for the analysis of MALDI data of different bacteria to allow their identification from their fingerprint.

Hollow-fiber flow field-flow fractionation of whole blood serum.

Hollow-fiber flow field-flow fractionation is here applied to untreated, whole human blood serum. Matrix-assisted, laser desorption/ionization time-of-flight-mass spectrometry (MALDI-TOF-MS) of serum fractions shows mass signals in the <30,000 M(r) range where low-abundance, serum protein components are known to be present, though a membrane of nominal 30,000 Da cutoff was employed for the fractionation device. Using diluted sera spiked with low amounts (0.

Serum protein profiling in patients with inflammatory bowel diseases using selective solid-phase bulk extraction, matrix-assisted laser desorption/ionization time-of-flight mass spectrometry and chemometric data analysis.

The identification of new biomarkers or a disease-related protein fingerprint for inflammatory bowel diseases (IBDs) represents a major task in the diagnosis, prognosis and pharmacological therapy. To address these issues, a simple and rapid analytical proteomic method for serum protein profiling based on selective beads-based solid-phase bulk extraction, matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS) and chemometric data analysis was developed. Serum proteins from healthy subjects (22) and patients with Crohn's disease (15) and ulcerative colitis (26) were selectively extracted according to reversed-phase (C18), strong anion-exchange (SAX) and metal ion affinity (IDA-Cu(II)) principles.

Tm-doped LiLuF4 crystal for efficient laser action in the wavelength range from 1.82 to 2.06 microm.

Room-temperature continuous-wave laser action in a novel Tm-doped LiLuF(4) crystal emitting at around 1.9 microm is demonstrated. The crystal growth process, spectroscopic measurements, and laser performance are presented for different Tm concentrations.

High efficiency room temperature laser emission in heavily doped Yb:YLF.

We report the tunable, CW and quasi CW laser operation at room temperature of an highly doped (30% at.) Yb:YLF crystal longitudinally pumped by a fiber coupled laser diode array. The CW output power is 1.

Visible laser emission of solid state pumped LiLuF(4):Pr(3+).

In the present work we report on the growth, spectroscopy and laser results of LiLuF(4):Pr(3+) 1.25% in the melt. Room temperature polarized absorption and emission spectra have been recorded and the decay time of the (3)P0 manifold has been measured.

Proteomic-based analysis of nuclear signaling: PLCbeta1 affects the expression of the splicing factor SRp20 in Friend erythroleukemia cells.

An extensive body of evidence links inositide-specific phospholipase C (PLC) to the nucleus and the main isoform located in the nucleus is PLCbeta(1). Constitutive overexpression of nuclear PLCbeta(1) has been previously shown to inhibit Friend erythroleukemia cells differentiation and to induce cell cycle progression targeting cyclin D3. The aim of this study was to identify new proteins regulated by PLCbeta(1) overexpression, given the role exerted by its signaling in the nucleus during cell growth and differentiation.

Hollow-fiber flow field-flow fractionation: a gentle separation method for mass spectrometry of native proteins.

Low-impact ionization sources like electrospray ionization (ESI) and matrix-assisted, laser desorption/ionization (MALDI) equipped with time-of-flight (TOF) mass analyzers provide intact protein analysis over a very wide molar mass range. ESI/TOFMS provides also indications on the higher-order structure of intact proteins and non-covalent protein complexes. However, direct analysis of intact proteins mixtures in real samples shows limited success, mainly because spectra become very complex to interpret.

Room-temperature Q-switched Tm:BaY(2)F(8) laser pumped by CW diode laser.

We report on the realization of CW diode-pumped Tm:BaY(2)F(8) Q-switched laser at 1.93 microm. Active Q-switching was obtained by means of an intracavity Pockels cell.

Combined approach to the analysis of recombinant protein drugs using hollow-fiber flow field-flow fractionation, mass spectrometry, and chemiluminescence detection.

The impurities present in recombinant protein drugs produced by large-scale refolding processes can not only affect the product safety but also interact with the expressed protein. To relate the impurity profile to conformation and functionality of the protein drug, analytical methods able not to degrade the sample components should be preferred. In this work, an urate oxidase (uricase) drug from Aspergillus flavus expressed in Saccharomyces cerevisiae, and a reagent-grade uricase from Candida sphaerica expressed in Escherichia coli, are analyzed by combining hollow-fiber flow field-flow fractionation with matrix-assisted laser desorption-ionization time-of-flight mass spectrometry (MALDI/TOFMS) and with chemiluminescence enzyme activity assay.

Widely tunable 1.94-microm Tm:BaY2F8 laser.

A novel BaY2F8 crystal doped with thulium ions is grown and extensively investigated. Owing to the large number of vibronic levels and to a favorable electron-phonon coupling, extremely wide absorption and emission bands around 1.9 microm are observed.

Ultrasensitive quantitative determination of paraquat: application to river, ground, and drinking water analysis in an agricultural area.

The water specimens were collected from wells and irrigation ditches in the agricultural area to the south of Milan and from Olona River and Mantua Lake and analyzed for paraquat detection. The assay was performed using a specific polyclonal antibody raised in sheep and rabbit anti-sheep IgG conjugated with a chelating molecule 4,7-bis(chlorosulfophenyl)-1,10-phenanthroline-2,9-dicarboxylic acid complexed with Eu3+ as a fluorescent marker. Bovine serum albumin conjugated with 5-(1'-methyl[4,4']bipyridinyl-1-yl)pentanoic acid was used in solid phase preparation.

On-line hollow-fiber flow field-flow fractionation-electrospray ionization/time-of-flight mass spectrometry of intact proteins.

Capabilities of mass spectrometry for the analysis of intact proteins can be increased through separation methods. Flow field-flow fractionation (FlFFF) is characterized by the particularly "soft" separation mechanism, which is ideally suited to maintain the native structure of intact proteins. This work describes the original on-line coupling between hollow-fiber FlFFF (HF FlFFF), the microcolumn variant of FlFFF, and electrospray ionization/time-of-flight mass spectrometry (ESI/TOFMS) for the analysis and characterization of intact proteins.