283 research outputs found

    A Characterization of right coideals of quotient type and its application to classification of Poisson boundaries

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    Let GG be a co-amenable compact quantum group. We show that a right coideal of GG is of quotient type if and only if it is the range of a conditional expectation preserving the Haar state and is globally invariant under the left action of the dual discrete quantum group. We apply this result to theory of Poisson boundaries introduced by Izumi for discrete quantum groups and generalize a work of Izumi-Neshveyev-Tuset on SUq(N)SU_q(N) for co-amenable compact quantum groups with the commutative fusion rules. More precisely, we prove that the Poisson integral is an isomorphism between the Poisson boundary and the right coideal of quotient type by maximal quantum subgroup of Kac type. In particular, the Poisson boundary and the quantum flag manifold are isomorphic for any q-deformed classical compact Lie group.Comment: 28 pages, Remark 4.9 adde

    Releasing dentate nucleus cells from Purkinje cell inhibition generates output from the cerebrocerebellum

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    The cerebellum generates its vast amount of output to the cerebral cortex through the dentate nucleus (DN) that is essential for precise limb movements in primates. Nuclear cells in DN generate burst activity prior to limb movement, and inactivation of DN results in cerebellar ataxia. The question is how DN cells become active under intensive inhibitory drive from Purkinje cells (PCs). There are two excitatory inputs to DN, mossy fiber and climbing fiber collaterals, but neither of them appears to have sufficient strength for generation of burst activity in DN. Therefore, we can assume two possible mechanisms: post-inhibitory rebound excitation and disinhibition. If rebound excitation works, phasic excitation of PCs and a concomitant inhibition of DN cells should precede the excitation of DN cells. On the other hand, if disinhibition plays a primary role, phasic suppression of PCs and activation of DN cells should be observed at the same timing. To examine these two hypotheses, we compared the activity patterns of PCs in the cerebrocerebellum and DN cells during step-tracking wrist movements in three Japanese monkeys. As a result, we found that the majority of wrist-movement-related PCs were suppressed prior to movement onset and the majority of wrist-movement-related DN cells showed concurrent burst activity without prior suppression. In a minority of PCs and DN cells, movement-related increases and decreases in activity, respectively, developed later. These activity patterns suggest that the initial burst activity in DN cells is generated by reduced inhibition from PCs, i.e., by disinhibition. Our results indicate that suppression of PCs, which has been considered secondary to facilitation, plays the primary role in generating outputs from DN. Our findings provide a new perspective on the mechanisms used by PCs to influence limb motor control and on the plastic changes that underlie motor learning in the cerebrocerebellum

    Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I

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    Umbilical cord blood (UCB) is a promising source of stem cells to use in early haematopoietic stem cell transplantation (HSCT) approaches for several genetic diseases that can be diagnosed at birth. Mucopolysaccharidosis type I (MPS-I) is a progressive multi-system disorder caused by deficiency of lysosomal enzyme α-L-iduronidase, and patients treated with allogeneic HSCT at the onset have improved outcome, suggesting to administer such therapy as early as possible. Given that the best characterized MPS-I murine model is an immunocompetent mouse, we here developed a transplantation system based on murine UCB. With the final aim of testing the therapeutic efficacy of UCB in MPS-I mice transplanted at birth, we first defined the features of murine UCB cells and demonstrated that they are capable of multi-lineage haematopoietic repopulation of myeloablated adult mice similarly to bone marrow cells. We then assessed the effectiveness of murine UCB cells transplantation in busulfan-conditioned newborn MPS-I mice. Twenty weeks after treatment, iduronidase activity was increased in visceral organs of MPS-I animals, glycosaminoglycans storage was reduced, and skeletal phenotype was ameliorated. This study explores a potential therapy for MPS-I at a very early stage in life and represents a novel model to test UCB-based transplantation approaches for various diseases

    On infinite-volume mixing

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    In the context of the long-standing issue of mixing in infinite ergodic theory, we introduce the idea of mixing for observables possessing an infinite-volume average. The idea is borrowed from statistical mechanics and appears to be relevant, at least for extended systems with a direct physical interpretation. We discuss the pros and cons of a few mathematical definitions that can be devised, testing them on a prototypical class of infinite measure-preserving dynamical systems, namely, the random walks.Comment: 34 pages, final version accepted by Communications in Mathematical Physics (some changes in Sect. 3 -- Prop. 3.1 in previous version was partially incorrect

    Magnified Examination of Small Colorectal Polyps Using a Prototype Electronic Endoscope

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    Magnifying electronic endoscopes are frequently used to evaluate the pit patterns of the colorectal mucosa, but such endoscopes suffer from a number of problems. For example, they tend to have long, hard tips and heavy controller sections. In addition, the magnified endoscopic images obtained are often quite coarse due to the small number of pixels in the charge-coupled device (CCD). As a result, at higher magnification ratios, the orientation of the field of view is easily lost. A newly developed prototype colorectal electronic endoscope (Toshiba Corporation, Tokyo) overcomes these problems. The length of the hard tip of the scope and the weight of the controller section are comparable to those of the TCE-3680MH (Toshiba Corporation). High-resolution magnified images can be obtained, because a 410,000-pixel CCD is employed. Two magnification methods are available, optical magnification and electronic zooming, permitting images to be magnified by a factor of up to 90–120 without losing the orientation of the field of view. This newly developed magnifying electronic endoscope was found to be very useful, allowing us to observe the pit patterns of the colorectal mucosa in 82 small colorectal polyps measuring 7 mm or less in diameter

    Classification of minimal actions of a compact Kac algebra with amenable dual

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    We show the uniqueness of minimal actions of a compact Kac algebra with amenable dual on the AFD factor of type II1_1. This particularly implies the uniqueness of minimal actions of a compact group. Our main tools are a Rohlin type theorem, the 2-cohomology vanishing theorem, and the Evans-Kishimoto type intertwining argument.Comment: 68 pages, Introduction rewritten; minor correction

    Promoter considerations in the design of lentiviral vectors for use in treating lysosomal storage diseases

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    More than 50 lysosomal storage diseases (LSDs) are associated with lysosomal dysfunctions with the frequency of 1:5,000 live births. As a result of missing enzyme activity, the lysosome dysfunction accumulates undegraded or partially degraded molecules, affecting the entire body. Most of them are life-threatening diseases where patients could die within the first or second decade of life. Approximately 20 LSDs have the approved treatments, which do not provide the cure for the disorder. Therefore, the delivery of missing genes through gene therapy is a promising approach for LSDs. Over the years, ex vivo lentiviral-mediated gene therapy for LSDs has been approached using different strategies. Several clinical trials for LSDs are under investigation.Ex vivo lentiviral-mediated gene therapy needs optimization in dose, time of delivery, and promoter-driven expression. Choosing suitable promoters seems to be one of the important factors for the effective expression of the dysfunctional enzyme. This review summarizes the research on therapy for LSDs that has used different lentiviral vectors, emphasizing gene promoters

    Spinal involvement in mucopolysaccharidosis IVA (Morquio-Brailsford or Morquio A syndrome): presentation, diagnosis and management.

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    Mucopolysaccharidosis IVA (MPS IVA), also known as Morquio-Brailsford or Morquio A syndrome, is a lysosomal storage disorder caused by a deficiency of the enzyme N-acetyl-galactosamine-6-sulphate sulphatase (GALNS). MPS IVA is multisystemic but manifests primarily as a progressive skeletal dysplasia. Spinal involvement is a major cause of morbidity and mortality in MPS IVA. Early diagnosis and timely treatment of problems involving the spine are critical in preventing or arresting neurological deterioration and loss of function. This review details the spinal manifestations of MPS IVA and describes the tools used to diagnose and monitor spinal involvement. The relative utility of radiography, computed tomography (CT) and magnetic resonance imaging (MRI) for the evaluation of cervical spine instability, stenosis, and cord compression is discussed. Surgical interventions, anaesthetic considerations, and the use of neurophysiological monitoring during procedures performed under general anaesthesia are reviewed. Recommendations for regular radiological imaging and neurologic assessments are presented, and the need for a more standardized approach for evaluating and managing spinal involvement in MPS IVA is addressed

    Diagnosing mucopolysaccharidosis IVA

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    Mucopolysaccharidosis IVA (MPS IVA; Morquio A syndrome) is an autosomal recessive lysosomal storage disorder resulting from a deficiency of N-acetylgalactosamine-6-sulfate sulfatase (GALNS) activity. Diagnosis can be challenging and requires agreement of clinical, radiographic, and laboratory findings. A group of biochemical genetics laboratory directors and clinicians involved in the diagnosis of MPS IVA, convened by BioMarin Pharmaceutical Inc., met to develop recommendations for diagnosis. The following conclusions were reached. Due to the wide variation and subtleties of radiographic findings, imaging of multiple body regions is recommended. Urinary glycosaminoglycan analysis is particularly problematic for MPS IVA and it is strongly recommended to proceed to enzyme activity testing even if urine appears normal when there is clinical suspicion of MPS IVA. Enzyme activity testing of GALNS is essential in diagnosing MPS IVA. Additional analyses to confirm sample integrity and rule out MPS IVB, multiple sulfatase deficiency, and mucolipidoses types II/III are critical as part of enzyme activity testing. Leukocytes or cultured dermal fibroblasts are strongly recommended for enzyme activity testing to confirm screening results. Molecular testing may also be used to confirm the diagnosis in many patients. However, two known or probable causative mutations may not be identified in all cases of MPS IVA. A diagnostic testing algorithm is presented which attempts to streamline this complex testing process
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