Bone marrow transplantation in canine mucopolysaccharidosis I. Effects within the central nervous system.
نویسندگان
چکیده
منابع مشابه
Bone marrow transplantation for feline mucopolysaccharidosis I.
Severe mucopolysaccharidosis type I (MPS I) is a fatal neuropathic lysosomal storage disorder with significant skeletal involvement. Treatment involves bone marrow transplantation (BMT), and although effective, is suboptimal, due to treatment sequelae and residual disease. Improved approaches will need to be tested in animal models and compared to BMT. Herein we report on bone marrow transplant...
متن کاملNeonatal bone marrow transplantation prevents bone pathology in a mouse model of mucopolysaccharidosis type I.
Neonatal bone marrow transplantation (BMT) could offer a novel therapeutic opportunity for genetic disorders by providing sustainable levels of the missing protein at birth, thus preventing tissue damage. We tested this concept in mucopolysaccharidosis type I (MPS IH; Hurler syndrome), a lysosomal storage disorder caused by deficiency of α-l-iduronidase. MPS IH is characterized by a broad spect...
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Besides being a metabolite of nucleotides like ATP, adenosine is a mediator of neuronal function in the central nervous system. Its actions are mediated by at least three extracellular receptors. In this review different aspects of adenosine such as biosynthesis, release, inactivation and its receptors are discussed. It also covers pre- and postsynaptic effects as well as postreceptor mecha...
متن کاملAngiitis of the central nervous system after allogeneic bone marrow transplantation?
BACKGROUND AND PURPOSE There is only limited information about late neurological complications after bone marrow transplantation (BMT). The purpose of this study is to describe a cerebral angiitis-like syndrome after allogeneic BMT. METHODS Clinical and diagnostic findings of 5 BMT patients with chronic graft versus host disease and neuropathological data of 1 patient were reported. RESULTS...
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Eighteen patients, twelve men and six women, with aplastic anemia underwent allogenic bone marrow transplantation (BMT) from HLA-matched siblings during the period of 1990 to 1996. The conditioning regimen was cyclophosphamide with or without busulfan, depending on the cause of aplasia. Antilymphocyte globulin (ALG) and cyclosporine were used for rejection and acute GVHD prophylaxis, respe...
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ژورنال
عنوان ژورنال: Journal of Clinical Investigation
سال: 1987
ISSN: 0021-9738
DOI: 10.1172/jci112830