Natural History and Inherited Disorders of a Lysosomal Enzyme, β-Hexosaminidase
نویسندگان
چکیده
منابع مشابه
strategies in enzyme replacement therapy of lysosomal storage disorders
lysosomal storage diseases (lsds) result from a genetic defect in synthesis and cellular transport of lysosomal enzyme to the lysosomes. lsds are progressive and may present at any age affecting multiple tissues and organ systems. they comprise a diverse group of over 40 clinically distinct inherited disorders. as a group they occur in approximately 1 in 5000 to 8000 births in the western socie...
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The pathogenic mycobacteria that cause tuberculosis (TB) and TB-like diseases in humans and animals elude sterilizing immunity by residing within an intracellular niche in host macrophages, where they are protected from microbicidal attack. Recent studies have emphasized microbial mechanisms for evasion of host defense; less is known about mycobactericidal mechanisms that remain intact during i...
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The synthesis and dimerization of beta-chains during the formation of catalytically active beta-hexosaminidase B were studied in a cell-free system. beta-chain mRNA, transcribed from the cloned cDNA with SP6 polymerase, was translated in a rabbit reticulocyte protein-synthesizing system in the presence of dog pancreas microsomal membranes and oxidized glutathione. Under these conditions, the pr...
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BACKGROUND For most arrhythmia syndromes, the risk of sudden cardiac death for asymptomatic mutation carriers is ill defined. Data on the natural history of these diseases, therefore, are essential. The family tree mortality ratio method offers the unique possibility to study the natural history at a time when the disease was not known and patients received no treatment. METHODS AND RESULTS I...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 1989
ISSN: 0021-9258
DOI: 10.1016/s0021-9258(18)60406-5