Substrate-Reduction Therapy Enhances the Benefits of Bone Marrow Transplantation in Young Mice with Globoid Cell Leukodystrophy
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چکیده
منابع مشابه
Bone Marrow Transplantation Alters the Tremor Phenotype in the Murine Model of Globoid-Cell Leukodystrophy
Tremor is a prominent phenotype of the twitcher mouse, an authentic genetic model of Globoid-Cell Leukodystrophy (GLD, Krabbe's disease). In the current study, the tremor was quantified using a force-plate actometer designed to accommodate low-weight mice. The actometer records the force oscillations caused by a mouse's movements, and the rhythmic structure of the force variations can be reveal...
متن کاملHematopoietic stem-cell transplantation in globoid-cell leukodystrophy.
BACKGROUND Globoid-cell leukodystrophy is caused by a deficiency of galactocerebrosidase, which results in progressive central nervous system deterioration. We investigated whether allogeneic hematopoietic stem-cell transplantation can provide a source of leukocyte galactocerebrosidase and thereby prevent the decline of central nervous system function in patients with the disease. METHODS Fiv...
متن کامل[Globoid cell leukodystrophy].
A 29 year old male with onset of globoid cell leukodystrophy at age 14 is described. This is the first case of enzymatically confirmed globoid cell leukodystrophy with onset of symptoms after the age of ten. This patient is unique because of the late onset and slow progression and extends the clinical spectrum of globoid cell leukodystrophy. Globoid cell leukodystrophy is caused by the deficien...
متن کاملEvaluation of the Repair of Bone Defect after Bone Marrow Stromal Cell Transplantation in Rat
Background & Objective: Bone marrow stromal cells (BMSCs) are multipotent stem cells that can proliferate and differentiate into osteogenic cells . T his study aimed to investigate the effects of BMSC transplantation for the repair of bone defects in the rat. Materials & Methods : Twenty-eight male adult rats were divided into 4 groups. Group 1 ( control group) received no treatment after the...
متن کاملgloboid cell leukodystrophy (krabbe disease)
how to cite this article: tavasoli a. globoid cell leukodystrophy (krabbe disease). iran j child neurol. autumn 2014;8;4(suppl.1):14-15. pls see pdf.
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 2002
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-200201000-00009