Galactose metabolism and health

被引:180
作者
Coelho, Ana I. [1 ,2 ]
Berry, Gerard T. [3 ]
Rubio-Gozalbo, M. Estela [1 ,2 ]
机构
[1] Maastricht Univ, Med Ctr, Dept Pediat, NL-6200 MD Maastricht, Netherlands
[2] Maastricht Univ, Med Ctr, Lab Genet Metab Dis, NL-6200 MD Maastricht, Netherlands
[3] Harvard Univ, Sch Med, Boston Childrens Hosp, Manton Ctr Orphan Dis Res,Div Genet & Genom, 3 Blackfan Circle,Ctr Life Sci Bldg,Suite 14070, Boston, MA 02115 USA
关键词
energy; galactose; glycosylation; metabolism; 1-PHOSPHATE URIDYLYLTRANSFERASE; CLASSICAL GALACTOSEMIA; ELIMINATION CAPACITY; I GALACTOSEMIA; HUMAN-MILK; OLIGOSACCHARIDES; INHIBITORS; CHILDREN; GLUCOSE; GLYCANS;
D O I
10.1097/MCO.0000000000000189
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Purpose of review Galactose - a key source of energy and a crucial structural element in complex molecules - is particularly important for early human development. However, galactose metabolism might be important not only for fetal and neonatal development but also for adulthood, as evidenced by the inherited disorders of galactose metabolism. The purpose of this review is to summarize the current evidence of galactose metabolism in health and disease. Recent findings The biological importance of galactose goes beyond its importance as a nutrient and a metabolite. Galactose has been selected by evolutionary pressure to exert also a crucial structural role in macromolecules. Additionally, galactose has recently been reported as beneficial in a number of diseases, particularly in those affecting the brain. Summary Galactose is crucial for human metabolism, with an established role in energy delivery and galactosylation of complex molecules, and evidence for other roles is emerging.
引用
收藏
页码:422 / 427
页数:6
相关论文
共 34 条
[1]  
[Anonymous], 2014, CHEM BIOL
[2]  
[Anonymous], ONLINE METABOLIC MOL
[3]  
[Anonymous], 2012, INBORN METABOLIC DIS
[4]   The Protein Family of Glucose Transport Facilitators: It's Not Only About Glucose After All [J].
Augustin, Robert .
IUBMB LIFE, 2010, 62 (05) :315-333
[5]  
Bell D, 2012, COMP BIOCH COMPREHEN, V3, P287
[6]   Is prenatal myo-inositol deficiency a mechanism of CNS injury in galactosemia? [J].
Berry, Gerard T. .
JOURNAL OF INHERITED METABOLIC DISEASE, 2011, 34 (02) :345-355
[7]   Introduction to the Maastricht workshop: lessons from the past and new directions in galactosemia [J].
Berry, Gerard T. ;
Elsas, Louis J. .
JOURNAL OF INHERITED METABOLIC DISEASE, 2011, 34 (02) :249-255
[8]   Metadynamics Simulations Reveal a Na+ Independent Exiting Path of Galactose for the Inward-Facing Conformation of vSGLT [J].
Bisha, Ina ;
Rodriguez, Alex ;
Laio, Alessandro ;
Magistrato, Alessandra .
PLOS COMPUTATIONAL BIOLOGY, 2014, 10 (12)
[9]   Static and dynamic interactions between GALK enzyme and known inhibitors: Guidelines to design new drugs for galactosemic patients [J].
Chiappori, Federica ;
Merelli, Ivan ;
Milanesi, Luciano ;
Marabotti, Anna .
EUROPEAN JOURNAL OF MEDICINAL CHEMISTRY, 2013, 63 :423-434
[10]   The Influence of Milk Oligosaccharides on Microbiota of Infants: Opportunities for Formulas [J].
Chichlowski, Maciej ;
German, J. Bruce ;
Lebrilla, Carlito B. ;
Mills, David A. .
ANNUAL REVIEW OF FOOD SCIENCE AND TECHNOLOGY, VOL 2, 2011, 2 :331-351