Bone marrow transplantation improves outcome in a mouse model of congenital muscular dystrophy

AbstractWe examined whether pathogenesis in dystrophin-deficient (mdx) mice and laminin-α2-deficient (dy) mice is ameliorated by bone marrow transplantation (BMT). Green fluorescent protein (GFP) mice were used as donors. In mdx mice, BMT failed to produce any significant differences in muscle pathology, although some GFP-positive fibers with restored dystrophin expression were observed. In contrast, in the dy mice, BMT led to a significant increase in lifespan and an increase in growth rate, muscle strength, and respiratory function. We conclude that BMT improved outcome in dy mice but not mdx mice

Bendamustine Plus Rituximab as Salvage Treatment for Patients with Relapsed or Refractory Low-grade B-cell Lymphoma and Mantle Cell Lymphoma: A Single-Center Retrospective Study

Bendamustine plus rituximab (B-R) is an effective therapy for relapsed or refractory (r/r) low-grade B-cell lymphoma (LGBCL) and mantle cell lymphoma (MCL); however, clinical data from Japanese patients treated with B-R therapy are limited. We retrospectively evaluated the efficacy and safety of B-R therapy in 42 patients who received B-R therapy at our hospital for r/r LGBCL and MCL. All patients received intravenous (IV) ritux-imab 375 mg/m2 on day 1 and IV bendamustine 90 mg/m2 on days 2 and 3 every 28 days for up to 6 cycles. The common histologic subtypes were follicular lymphoma (n = 29, 70%), marginal zone lymphoma (n = 6, 14%), and MCL (n = 5, 12%). The overall response rate was 93%, with 62% complete response and complete response unconfirmed. The median progression-free survival (PFS) was 38 months (95% confidence interval [CI], 24.6 to not reached [NR]), and the median overall survival (OS) was 80 months (95% CI, 60.7 to NR). Patients receiving a cumulative dose of bendamustine ≥ 720 mg/m2 showed a significantly longer PFS and OS. Grade 3/4 adverse events (≥ 10%) included neutropenia (55%), lymphopenia (69%), and nausea (24%). B-R therapy was effective and well tolerated, and the cumulative dose of bendamustine was associated with a favorable outcome

Suzaku Discovery of a Hard X-Ray Tail in the Persistent Spectra from the Magnetar 1E 1547.0-5408 during its 2009 Activity

The fastest-rotating magnetar 1E 1547.0-5408 was observed in broad-band X-rays with Suzaku for 33 ks on 2009 January 28-29, 7 days after the onset of its latest bursting activity. After removing burst events, the absorption-uncorrected 2-10 keV flux of the persistent emission was measured with the XIS as 5.7e-11 ergs cm-2 s-1, which is 1-2 orders of magnitude higher than was measured in 2006 and 2007 when the source was less active. The persistent emission was also detected significantly with the HXD in >10 keV up to at least ~110 keV, with an even higher flux of 1.3e-10 ergs cm-2 s-1 in 20-100 keV. The pulsation was detected at least up to 70 keV at a period of 2.072135+/-0.00005 s, with a deeper modulation than was measured in a fainter state. The phase-averaged 0.7-114 keV spectrum was reproduced by an absorbed blackbody emission with a temperature of 0.65+/-0.02 keV, plus a hard power-law with a photon index of ~1.5. At a distance of 9 kpc, the bolometric luminosity of the blackbody and the 2-100 keV luminosity of the hard power-law are estimated as (6.2+/-1.2)e+35 ergs s-1 and 1.9e+36 ergs s-1, respectively, while the blackbody radius becomes ~5 km. Although the source had not been detected significantly in hard X-rays during the past fainter states, a comparison of the present and past spectra in energies below 10 keV suggests that the hard component is more enhanced than the soft X-ray component during the persistent activity.Comment: 12 pages, 7 figures, PASJ Vol.62 No.2 accepte

Pilocarpine-induced salivary fluid secretion in the perfused submandibular gland of the rat

Xerostomia is the symptom of dry mouth often seen in patients who receive head and neck radiation therapy or in patients who have Sjögren’s syndrome. The primary treatment to relieve xerostomia symptom is oral administration of pilocarpine, a parasympathomimetic agent with muscarinic action. Increase in salivary secretion induced by systemic administration of pilocarpine is considered to be mediated by actions on muscarinic cholinergic receptors in the central nervous system and salivary glands. In this study, we investigated the direct effect of pilocarpine on salivary fluid secretion in the isolated, perfused rat submandibular gland. Pilocarpine provoked salivary fluid secretion in a dose-dependent manner. The Na+-channel blocker tetrodotoxin had almost no effect on the pilocarpine-induced salivary fluid secretion, indicating that pilocarpine directly stimulates submandibular gland. Pilocarpine induced an increase in intracellular Ca2+ concentration in dispersed submandibular gland cells at 37℃, but not 25℃. The salivary fluid secretion induced by pilocarpine was consisted of a rapid and transient phase and a subsequent sustained phase, which profile was different from that evoked by carbachol, another typical muscarinic agonist. Pilocarpine also induced Lucifer yellow secretion via paracellular route

Case Reports of TFE3-Rearranged Renal Cell Carcinoma: FDG-PET Uptake Might Help Diagnosis

Translocation and transcription factor E3 (TFE3)-rearranged renal cell carcinoma (RCC) is a rare subtype of RCCs characterised by the fusion of the TFE3 transcription factor genes on chromosome Xp11.2 with one of the multiple genes. TFE3-rearranged RCC occurs mainly in children and adolescents, although middle-aged cases are also observed. As computed tomography (CT)/magnetic resonance imaging (MRI) findings of TFE3-rearranged RCC overlap with those of other RCCs, differential diagnosis is often challenging. In the present case reports, we highlighted the features of the fluorine-18-labelled fluorodeoxyglucose positron emission tomography with CT (FDG PET-CT) in TFE3-rearranged RCCs. Due to the rarity of the disease, FDG PET-CT features of TFE3-rearranged RCC have not yet been reported. In our cases, FDG PET-CT showed high standardised uptake values (SUVmax) of 7.14 and 6.25 for primary tumours. This might imply that TFE3-rearranged RCC has high malignant potential. This is conceivable when the molecular background of the disease is considered in terms of glucose metabolism. Our cases suggest that a high SUVmax of the primary tumour is a clinical characteristic of TFE3-rearranged RCCs

Hot debris dust around HD 106797

Photometry of the A0 V main-sequence star HD 106797 with AKARI and Gemini/T-ReCS is used to detect excess emission over the expected stellar photospheric emission between 10 and 20 micron, which is best attributed to hot circumstellar debris dust surrounding the star. The temperature of the debris dust is derived as Td ~ 190 K by assuming that the excess emission is approximated by a single temperature blackbody. The derived temperature suggests that the inner radius of the debris disk is ~ 14 AU. The fractional luminosity of the debris disk is 1000 times brighter than that of our own zodiacal cloud. The existence of such a large amount of hot dust around HD 106797 cannot be accounted for by a simple model of the steady state evolution of a debris disk due to collisions, and it is likely that transient events play a significant role. Our data also show a narrow spectral feature between 11 and 12 micron attributable to crystalline silicates, suggesting that dust heating has occurred during the formation and evolution of the debris disk of HD 106797.Comment: Accepted to ApJ Letters, 8 pages, 2 figure

In Vitro Assessment of Factors Affecting the Apparent Diffusion Coefficient of Ramos Cells Using Bio-phantoms

The roles of cell density, extracellular space, intracellular factors, and apoptosis induced by the molecularly targeted drug rituximab on the apparent diffusion coefficient (ADC) values were investigated using bio-phantoms. In these bio-phantoms, Ramos cells (a human Burkittｾs lymphoma cell line) were encapsulated in gellan gum. The ADC values decreased linearly with the increase in cell density, and declined steeply when the extracellular space became less than 4 μm. The analysis of ADC values after destruction of the cellular membrane by sonication indicated that approximately 65% of the ADC values of normal cells originate from the cell structures made of membranes and that the remaining 35% originate from intracellular components. Microparticles, defined as particles smaller than the normal cells, increased in number after rituximab treatments, migrated to the extracellular space and significantly decreased the ADC values of bio-phantoms during apoptosis. An in vitro study using bio-phantoms was conducted to quantitatively clarify the roles of cellular factors and of extracellular space in determining the ADC values yielded by tumor cells and the mechanism by which apoptosis changes those values

Kohn anomalies in graphene nanoribbons

The quantum corrections to the energies of the $\Gamma$ point optical phonon modes (Kohn anomalies) in graphene nanoribbons are investigated. We show theoretically that the longitudinal optical modes undergo a Kohn anomaly effect, while the transverse optical modes do not. In relation to Raman spectroscopy, we show that the longitudinal modes are not Raman active near the zigzag edge, while the transverse optical modes are not Raman active near the armchair edge. These results are useful for identifying the orientation of the edge of graphene nanoribbons by G band Raman spectroscopy, as is demonstrated experimentally. The differences in the Kohn anomalies for nanoribbons and for metallic single wall nanotubes are pointed out, and our results are explained in terms of pseudospin effects.Comment: 11 pages, 6 figure