Galactose Toxicity in Animals

被引:98
作者
Lai, Kent [1 ]
Elsas, Louis J. [2 ]
Wierenga, Klaas J. [3 ]
机构
[1] Univ Utah, Sch Med, Dept Pediat, Div Med Genet, Salt Lake City, UT 84132 USA
[2] Univ Miami, Miller Sch Med, Dept Biochem & Mol Biol, Miami, FL 33136 USA
[3] Univ Oklahoma, Hlth Sci Ctr, Dept Pediat, Genet Sect, Oklahoma City, OK 73104 USA
关键词
disease models; drug discovery; genetics; glycobiology; URIDINE-DIPHOSPHATE GALACTOSE; PREMATURE OVARIAN FAILURE; HUMAN GALACTOSE-1-PHOSPHATE URIDYLYLTRANSFERASE; EPIMERASE-DEFICIENCY GALACTOSEMIA; ALDOSE REDUCTASE INHIBITOR; YEAST EXPRESSION SYSTEM; LELOIR PATHWAY ENZYMES; PRIMORDIAL GERM-CELLS; BONE-MINERAL TURNOVER; GALACTOKINASE DEFICIENCY;
D O I
10.1002/iub.262
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
In most organisms, productive utilization of galactose requires the highly conserved Leloir pathway of galactose metabolism. Yet, if this metabolic pathway is perturbed due to congenital deficiencies of the three associated enzymes, or an overwhelming presence of galactose, this monosaccharide which is abundantly present in milk and many non-dairy foodstuffs, will become highly toxic to humans and animals. Despite more than four decades of intense research, little is known about the molecular mechanisms of galactose toxicity in human patients and animal models. In this contemporary review, we take a unique approach to present an overview of galactose toxicity resulting from the three known congenital disorders of galactose metabolism and from experimental hypergalactosemia. Additionally, we update the reader about research progress on animal models, as well as advances in clinical management and therapies of these disorders. (C) 2009 IUBMB IUBMB Life, 61(11): 1063-10741, 2009
引用
收藏
页码:1063 / 1074
页数:12
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