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UDPglucose 4-Epimerase, Gale, UDP-galactose-4-epimerase
This gene encodes UDP-galactose-4-epimerase which catalyzes two distinct but analogous reactions: the epimerization of UDP-glucose to UDP-galactose, and the epimerization of UDP-N-acetylglucosamine to UDP-N-acetylgalactosamine. The bifunctional nature of the enzyme has the important metabolic consequence that mutant cells (or individuals) are dependent not only on exogenous galactose, but also on exogenous N-acetylgalactosamine as a necessary precursor for the synthesis of glycoproteins and glycolipids. Mutations in this gene result in epimerase-deficiency galactosemia, also referred to as galactosemia type 3, a disease characterized by liver damage, early-onset cataracts, deafness and mental retardation, with symptoms ranging from mild ('peripheral' form) to severe ('generalized' form). Multiple alternatively spliced transcripts encoding the same protein have been identified. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: ACID, CAN, HAD, GalT, galactokinase
Papers using UDPglucose 4-Epimerase antibodies
Sequencing of 50 human exomes reveals adaptation to high altitude.
Shendure Jay et al., In Genome Biology, 1989
... lacX74 phoAΔ20 galE galK thi rpsE rpoB argEam recA1)) [19], genomic DNA, and CRW10 and PA1 phage genomic DNA were extracted using Qiagen (Valencia, CA, USA) MidiPrep ...
Papers on UDPglucose 4-Epimerase
Structural insights on mouse l-threonine dehydrogenase: A regulatory role of Arg180 in catalysis.
Shi et al., Hefei, China. In J Struct Biol, Dec 2015
In sum, our results suggest that activity of GalE-like TDH can be regulated by remote interaction, such as hydrogen bonding and hydrophobic interaction around the Arg180 of mTDH.
A first case report of UDP-galactose-4'-epimerase deficiency in China: genotype and phenotype.
Gao et al., In J Pediatr Endocrinol Metab, Dec 2015
BACKGROUND: The aim of the study was to investigate the incidence and genotype-phenotype characteristics of UDP-galactose-4'-epimerase (GALE) deficiency in newborn screening of Chinese population.
The structural basis of substrate promiscuity in UDP-hexose 4-epimerase from the hyperthermophilic Eubacterium Thermotoga maritima.
Lee et al., Taegu, South Korea. In Arch Biochem Biophys, Dec 2015
UDP-galactose 4-epimerase (GalE) catalyzes the interconversion of UDP-glucose (UDP-Glc) and UDP-galactose (UDP-Gal), which is a pivotal step in the Leloir pathway for d-galactose metabolism.
UDP-hexose 4-epimerases: a view on structure, mechanism and substrate specificity.
Desmet et al., Gent, Belgium. In Carbohydr Res, Oct 2015
UDP-sugar 4-epimerase (GalE) belongs to the short-chain dehydrogenase/reductase (SDR) superfamily of proteins and is one of enzymes in the Leloir pathway.
Phylogenetic study of clonal complex (CC)198 capsule null locus (cnl) genomes: A distinctive group within the species Neisseria meningitidis.
Shao et al., Beijing, China. In Infect Genet Evol, Aug 2015
A number of possible recombination events were identified in the galE and tex genes between different serogroups of encapsulated N. meningitidis and CC53 strains, especially in tex.
The molecular basis of galactosemia - Past, present and future.
Timson, Belfast, United Kingdom. In Gene, Aug 2015
However, over the next three decades it became clear that there were two other forms of galactosemia: type II resulting from deficiencies in galactokinase (GALK1) and type III where the affected enzyme is UDP-galactose 4'-epimerase (GALE).
Effect of estrogen on recovering the injured nervous system.
Wang et al., Jinan, China. In Pak J Pharm Sci, Jul 2015
In the 14th d after the injury of spinal cord nervous system, Gale grading and inclined plate maintenance tests were carried out.
Incretin-based drugs and adverse pancreatic events: almost a decade later and uncertainty remains.
Azoulay, Montréal, Canada. In Diabetes Care, Jun 2015
In the narrative preceding Dr. Azoulay's contribution, Prof. Edwin A.M. Gale provides a commentary on the report that focuses on clinical trials of liraglutide in the treatment of diabetes.
Smoke or fire? Acute pancreatitis and the liraglutide trials.
Gale, Bristol, United Kingdom. In Diabetes Care, Jun 2015
In the narrative outlined below, Prof. Edwin A.M. Gale provides a commentary on the report that focuses on clinical trials of liraglutide in the treatment of diabetes.
PTS 50: Past, Present and Future, or Diauxie Revisited.
Lengeler, Osnabrück, Germany. In J Mol Microbiol Biotechnol, 2014
They are primarily linked to names like Epps and Gale, J. Monod, Cohn and Horibata, and B. Magasanik, and to phenomena like 'glucose effects', 'diauxie', 'catabolite repression' and carbohydrate transport.
Capsular profiling of the Cronobacter genus and the association of specific Cronobacter sakazakii and C. malonaticus capsule types with neonatal meningitis and necrotizing enterocolitis.
Forsythe et al., Nottingham, United Kingdom. In Bmc Genomics, 2014
Two variants of the colanic acid synthesis gene cluster (CA1 and CA2) were found which differed with the absence of galE in CA2.
mHealth: a strategic field without a solid scientific soul. a systematic review of pain-related apps.
Miró et al., Tarragona, Spain. In Plos One, 2013
METHODS AND FINDINGS: To exemplify the issues, we made a systematic review of the pain-related apps available in scientific databases (Medline, Web of Science, Gale, Psycinfo, etc.) and the main application shops (App Store, Blackberry App World, Google Play, Nokia Store and Windows Phone Store).
Chemical and stereochemical actions of UDP-galactose 4-epimerase.
Hegeman et al., Madison, United States. In Acc Chem Res, 2013
All healthy cells produce UDP-gal from uridine(5')diphospho(1)α-D-glucose (UDP-glc) by the action of UDP-galactose 4-epimerase (GalE).
Functional analysis of mutations in UDP-galactose-4-epimerase (GALE) associated with galactosemia in Korean patients using mammalian GALE-null cells.
Song et al., Anyang, South Korea. In Febs J, 2009
Our observations show that altered protein stability is due to misfolding and that loss or reduction of enzyme activity is responsible for the molecular defects underlying GALE-deficiency galactosemia.
Regulatory activity revealed by dynamic correlations in gene expression noise.
Elowitz et al., Pasadena, United States. In Nat Genet, 2008
We find that the CRPGalS-GalE feed-forward loop is inactive in standard conditions but can become active in a GalR mutant.
Analysis of UDP-galactose 4'-epimerase mutations associated with the intermediate form of type III galactosaemia.
Timson et al., Atlanta, United States. In J Inherit Metab Dis, 2008
Subtle biochemical and metabolic abnormalities detected in patients expressing these GALE alleles likely reflect, at least in part, the reduced enzymatic activity of the encoded GALE proteins.
Functional analysis of disease-causing mutations in human UDP-galactose 4-epimerase.
Timson, Belfast, United Kingdom. In Febs J, 2005
Disease-causing mutations result in a variety of changes to the steady-state parameters. Mostly these are changes in turnover number, kcat. The ability to dimerize is not affected, but some mutants have increased sensitivity to protease digestion.
Mediators of galactose sensitivity in UDP-galactose 4'-epimerase-impaired mammalian cells.
Fridovich-Keil et al., Atlanta, United States. In J Biol Chem, 2005
Resulst describe the relationship among UDP-galactose 4'-epimerase activity, substrate specificity, metabolic balance, and galactose sensitivity in mammalian cells.
Determinants of function and substrate specificity in human UDP-galactose 4'-epimerase.
Fridovich-Keil et al., Atlanta, United States. In J Biol Chem, 2004
study of hGALE crystal structure and demonstration that residue 307 acts as a gatekeeper mediating substrate access to the hGALE active site
A control element within a structural gene: the gal operon of Escherichia coli.
Adhya et al., In Cell, 1983
From these studies, we propose the existence of a second functional gal operator element at an extraordinary site--within galE, the first structural gene.
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