Galactose toxicity in animals.
Galactose toxicity in animals.
复制标题
DOI：
10.1002/iub.262
复制
发表时间：
2009-11
期刊：
影响因子：
4.6
通讯作者：
Wierenga, Klaas J.
中科院分区：
文献类型：
--
作者：
Lai, Kent;Elsas, Louis J.;Wierenga, Klaas J.
在大多数生物体中，半乳糖的有效利用需要高度保守的半乳糖代谢的Leloir途径。然而，如果这一代谢途径由于三种相关酶的先天缺乏或半乳糖的大量存在而受到干扰，这种大量存在于牛奶和许多非乳制品食品中的单糖将对人类和动物产生剧毒。尽管经过40多年的深入研究，人们对半乳糖在人类患者和动物模型中毒性的分子机制知之甚少。在这篇当代综述中，我们采用一种独特的方法来概述由三种已知的先天性半乳糖代谢障碍和实验性高半乳糖血症引起的半乳糖毒性。此外，我们更新读者关于动物模型的研究进展，以及在这些疾病的临床管理和治疗方面的进展。©2009 IUBMB IUBMB生活，61(11):1063-1074,2009
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. © 2009 IUBMB IUBMB Life, 61(11): 1063–1074, 2009
登录
查看更多内容
影响因子：
8.8
作者：
Coffee, Bradford;Hjelm, Lawrence N.;Muralidharan, Kasinathan
通讯作者：
Muralidharan, Kasinathan
影响因子：
6.1
作者：
Bandyopadhyay, S;Chakrabarti, J;Kabir, SN
通讯作者：
Kabir, SN
影响因子：
9.8
作者：
Berry, GT;Wehrli, S;Segal, S
通讯作者：
Segal, S
影响因子：
4.2
作者：
Bosch, Annet M.
通讯作者：
Bosch, Annet M.
影响因子：
3.6
作者：
ACOSTA, PB;GROSS, KC
通讯作者：
GROSS, KC