منابع مشابه
Characterization of a murine model of mucopolysaccharidosis type IIID: A knockout model
The Mucopolysaccharidoses (MPS) are a class of lysosomal storage disorders, characterized by the primary lysosomal storage of either single or multiple species of glycosaminoglycan (GAG) which leads to cell, tissue, and organ dysfunction. This is manifested clinically by either bone/connective tissue disease, central nervous system (CNS) disease, or both, and often results in premature death. M...
متن کاملExpression, purification and characterization of recombinant caprine N-acetylglucosamine-6-sulphatase.
Mucopolysaccharidosis type IIID or Sanfilippo D syndrome is a lysosomal storage disorder caused by the deficiency of N-acetylglucosamine-6-sulphatase (Glc6S). In addition to human patients, a Nubian goat with this disorder has been described and the caprine Glc6S (cGlc6S) cDNA cloned. In this study, the full-length cGlc6S cDNA was inserted into the expression vector, pEFNeo, which placed the cG...
متن کاملMucopolysaccharidosis VI
Mucopolysaccharidosis VI (MPS VI) is a lysosomal storage disease with progressive multisystem involvement, associated with a deficiency of arylsulfatase B leading to the accumulation of dermatan sulfate. Birth prevalence is between 1 in 43,261 and 1 in 1,505,160 live births. The disorder shows a wide spectrum of symptoms from slowly to rapidly progressing forms. The characteristic skeletal dysp...
متن کاملCaprine Arthritis and Encephalitis
Caprine arthritis and encephalitis (CAE) is an economically important viral disease of goats. The caprine arthritis encephalitis virus (CAEV), a lentivirus; infects its hosts for life. Although most infections are subclinical, a minority of animals develops progressive, untreatable disease syndromes including polyarthritis in adults and encephalomyelitis in kids. This virus also causes indurati...
متن کاملCaprine Arthritis and Encephalitis
Caprine arthritis and encephalitis (CAE) is an economically important viral disease of goats. The caprine arthritis encephalitis virus (CAEV), a lentivirus; infects its hosts for life. Although most infections are subclinical, a minority of animals develops progressive, untreatable disease syndromes including polyarthritis in adults and encephalomyelitis in kids. This virus also causes indurati...
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ژورنال
عنوان ژورنال: Journal of Neuropathology and Experimental Neurology
سال: 1998
ISSN: 0022-3069
DOI: 10.1097/00005072-199802000-00006