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Hematopoietic stem cell targeted neonatal gene therapy reverses lethally progressive osteopetrosis in oc/oc mice.

Infantile malignant osteopetrosis (IMO) is a fatal disease caused by lack of functional osteoclasts, and the only available treatment is hematopoietic stem cell (HSC) transplantation. In the majority of patients, the TCIRG1 gene, coding for a subunit of a proton pump essential for bone resorption, is mutated. Oc/oc mice have a deletion in the homologue gene (tcirg 1) and die at 3 to 4 weeks, but c

Ursolic acid inhibits the formation of aberrant crypt foci and affects colonic sphingomyelin hydrolyzing enzymes in azoxymethane-treated rats

Ursolic acid (UA) is a pentacyclic triterpenoid, with anti-cancer and anti-inflammatory properties. Sphingomyelin (SM) hydrolysis generates lipid messengers regulating cell survival. Earlier studies showed that UA has anti-proliferative and apoptotic effects on HT29 cells, accompanied by a rapid increase in alkaline sphingomyelinase (Alk-SMase) activity. This study examines the effect of orally ad

Language development in hearing-impaired children - Establishment of a reference material for a 'Language test for hearing-impaired children', LATHIC

Objective: In Sweden, there has previously been no normalised test material for the evaluation of language development in individual hearing-impaired children, and for the assessment of various methods of auditory habilitation. The purpose of the present study was to compose, apply and evaluate a test for language development in hearing-impaired children, and to establish the first set of referenc

Exacerbations of COPD: quantifying the patient's perspective using discrete choice modelling

Patient-centred care is the current vogue in chronic obstructive pulmonary disease (COPD), but it is only recently that robust techniques have become available to determine patients' values and preferences. In this international cross-sectional study, patients' concerns and expectations regarding COPD exacerbations were explored using discrete choice modelling. A fractional factorial design was us

The genetic control of sialadenitis versus arthritis in a NOD.QxB10.Q F2 cross.

The non-obese diabetic (NOD) mouse spontaneously develops diabetes and sialadenitis. The sialadenitis is characterized by histopathological changes in salivary glands and functional deficit similar to Sjögren's syndrome. In humans, Sjögren's syndrome could be associated with other connective tissue disorders, such as rheumatoid arthritis. In the present study the genetic control of sialadenitis in

MHC diversity in two Acrocephalus species: the outbred Great reed warbler and the inbred Seychelles warbler

The Great reed warbler (GRW) and the Seychelles warbler (SW) are congeners with markedly different demographic histories. The GRW is a normal outbred bird species while the SW population remains isolated and inbred after undergoing a severe population bottleneck. We examined variation at Major Histocompatibility Complex (MHC) class I exon 3 using restriction fragment length polymorphism, denaturin

Intensive care unit syndrome/delirium is associated with anemia, drug therapy and duration of ventilation treatment.

BACKGROUND: We have performed a prospective qualitative investigation of the ICU syndrome/delirium; the main parts of which have recently been published. The aim of the present study was to explore the relationship between the ICU syndrome/delirium and age, gender, length of ventilator treatment, length of stay and severity of disease, as well as factors related to arterial oxygenation and the amo

Lymphocyte subpopulations and lymphokine production in children with constitutional aplastic anemia

The expression of lymphocyte surface markers as well as the production of interleukin-2 (IL-2) and interferon-gamma (IFN) by mitogen-stimulated peripheral blood mononuclear cells (MNC) have been studied in five children with constitutional aplastic anemia. A significantly reduced T4/T8 ratio was found and two of five patients also had a reduced percentage of B cells. One patient had a high percent

The C4b-binding protein-protein S complex inhibits the phagocytosis of apoptotic cells.

The phagocytosis of apoptotic cells is a complex process involving numerous interactions between the target cell and the macrophage. We have examined a role of the major soluble inhibitor of the classic and lectin complement pathways, C4b-binding protein (C4BP), in the clearance of apoptotic cells. The major form of C4BP present in blood is composed of seven alpha-chains and one beta-chain, which

A Fast, Bandlimited Solver for Scattering Problems in Inhomogeneous Media

The numerical treatment of two-dimensional scattering in inhomogeneous media is considered. A novel approach in treating convolution operators with low regularity is used to construct an iterative solver for the Lippmann-Schwinger integral equation. In this way, accurate approximations within a choice of bandwidth can be obtained in a rapid manner. The performance of the method is tested on a disc

Genetic dissection of lymphopenia from autoimmunity by introgression of mutated Ian5 gene onto the F344 rat.

Peripheral T cell lymphopenia (lyp) in the BioBreeding (BB) rat is linked to a frameshift mutation in Ian5, a member of the Immune Associated Nucleotide (Ian) gene family on rat chromosome 4. This lymphopenia leads to type 1 (insulin-dependent) diabetes mellitus (T1DM) at rates up to 100% when combined with the BB rat MHC RT1 u/u genotype. In order, to better study the lymphopenia phenotype withou

Short-term administration of ACTH improves plasma lipid profile and renal function in kidney transplant patients

The present study investigated effects of short-term administration of adrenocorticotrophic hormone (ACTH) on blood lipid profile and renal function in kidney transplant patients. Six patients who had kidney transplantations 2 to 10 years earlier received ACTH intramuscularly (1 mg/d) for 4 days. We analyzed serum levels of lipids, lipoproteins, apolipoproteins, blood creatinine, and other paramet

Gated tunnel diode in oscillator applications with high frequency tuning

A gated tunnel diode has been introduced into a wave-guide oscillator circuit and the gate is used to tune the oscillation frequency and to turn the oscillator on and off. Oscillators with oscillation frequencies in the range of 9-22 GHz with a typical oscillator output power about -20 dBm have been fabricated. We found that the output power remains essentially constant over a gate bias range (abo

The method of administration of cholecystokinin determines the effects evoked in the pancreas

Earlier studies have shown different effects on cell proliferation and weight characteristics by sulfated cholecystokinin-8 (CCK-8S) in the rat pancreas when the peptide has been administered continuously rather than intermittently. The aim of this study was as follows: (i) to compare the effect of continuous infusion and of intermittent injections of CCK-8S on cell proliferation, weight gain, and

Maximum likelihood estimates for object detection using multiple detectors

Object detection in real images has attracted much attention during the last decade. Using machine learning and large databases it is possible to develop detectors for visual categories that have a very high hit-rate, with low false positive rates. In this paper we investigate a general probabilistic framework for context based scene interpretation using multiple detectors. Methods for finding max