Hunter disease

F-1) The mucopolysaccharidoses are proteoglycan disorders that generally result from a hereditary lysosomal defect in enzymes that normally degrade mucopolysaccharides (in most cases heparan sulfate and dermatan sulfate). This leads to the accumulation of different mucopolysaccharides, which may be associated with a variety of different findings, commonly including mental retardation and various skeletal abnormalities. These diseases include Hunter disease, Hurler and Scheie disease, I-cell disease , Maroteaux-Laury disease, Morquio syndrome, Mucolipidoses VH disease, multiple sulfatase deficiency, and Sanfilippo A and B diseases, which will not be elaborated on further here. Often these conditions can be detected in advance on amniocentesis. 

Kint, J. A., In vitro restoration of deficient p-galactosidase activity in liver of patients with Hurler and Hunter disease. Nature (London) 250, 424-425 (1974). 

D Hunter, Diseases of Occupations, 4th Edition, Little Brown, Boston, 1969. 

Hunter PR (2003) Climate change and waterborne and vector-home diseases. J Appl Microbiol 94 37S 6S... 

Schneider RR, Hunter DB. 1993. Nursing disease in mink Clinical and postmortem findings. Vet Pathol 30(6) 512-521. 

Foster J, Hunter N. Transmissible spongiform encephalopathies transmission, mechanism of disease, and persistence. Curr Opin Microbiol 1998 1 442-447. 

Hunter, T., The Croonian Lecture 1997. The phosphorylation of proteins on tyrosine its role in cell growth and disease, Philos. Trans. Roy. Soc. London B (Biol. Sci.), 353, 583-605, 1998. 

Primary lysosomal hydrolase defects. Two-thirds of the lysosomal storage diseases involve defects in genes that code for acid hydrolases. Table 41-2 lists 29 defects that have been defined so far. They have an autosomal recessive mode of inheritance, except for Hunter s syndrome and Fabry s disease, where the mode is X-linked recessive. The defective genes have been identified and mutations have been defined for nearly all. The nervous system is involved in most. Many of the disorders show a wide range of clinical severity, which may range from death in early childhood to a moderate disability in adulthood. 

Hunter, D. "The Diseases of Occupations" Hodder and Stoughton London, 1975 p. 1225. 

Hunter, D.J. (2005) Gene-environment interactions in human diseases. Nat. Rev. Genet., 6, 287-298. 

Coeliac disease probably arose around 10000 bc when humans switched from the hunter-gatherer way of life to cultivation of cereals (barley, wheat and oats). An illness... 

Hunter D. 1978. The diseases of occupations. 6th ed. London, England Hodder and Stoughton, 518-522. 

Idursulfase (Elaprase) is a drug used to treat mucopolysaccharidosis II or Hunter syndrome. It is a lysosomal storage disease caused by iduronate-2-sulfatase deficiency. Idursulfase is a purified form of iduronate-2-sulfatase produced by recombinant DNA technology in a human cell line. The drug provides clinically important benefits to Hunter syndrome patients. After intravenous infusion Idursulfase is eliminated by peptide hydrolysis with an elimination half-life of 45 minutes. The most common adverse events are hypersensitivity reactions, pyrexia, headache and arthralgia. 

Strickland GT. Hunter s tropical medicine and emerging infectious diseases (8th ed.). Philadelphia Saunders, 2000. 

Hunter, J. E., Zhang, J., and Kris-Etherton, P. M. (2009). Cardiovascular disease risk of dietary stearic acid compared with trans, other saturated, and unsaturated fatty acids A systematic review. Am.. Clin. Nutr. 91(1), 46-63. 

Lysosomal storage abnormalities, such as glycogenosis (Pompe s disease). Tay-Sachs, Krabbe s. Gaucher s, and Fabry s diseases, as well as melachromatic leukodystrophy, aspariylgiycosaminuria, and Niemann-Pick disease. Also included in this category are mucopolysaccharidoses. Hunter s, Scheie s, and Hurler s syndromes. 

Hunter, M.M. and McKay, D.M. (2004) Review article helminths as therapeutic agents for inflammatory bowel disease. Alimentary Pharmacology and Therapeutics 19, 1 67-1 77. 

Porter JB, Hunter JR, Jick H, Stergachis A. Oral contraceptives and nonfatal vascular disease. Obstet Gynecol 1985 66(l) l-4. 

Hung HC, Joshipura KJ, Jiang R, Hu FB, Hunter D, Smith-Warner SA, Colditz GA, Rosner B, Spiegelman D, Willett WC. 2004. Fruit and vegetable intake and risk of major chronic disease. J Natl Cancer Inst 96 1577-1584. 

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