The topic of this review is hepatorenal tyrosinemia (hereditary tyrosinemia type 1 [HT1], or
fumarylacetoacetate hydrolase deficiency; OMIM# 276700). HT1 is the most serious and …

Life as we know it would not exist without water. However, water molecules not only serve as
a solvent and reactant but can also promote hydrolysis, which counteracts the formation of …

Inborn errors of metabolism (IEMs) are a group of diseases involving a genetic defect that
alters a metabolic pathway and that presents usually during infancy. The tyrosine …

The degradation of synthetic compounds requires bacteria to recruit and adapt enzymes
from pathways for naturally occurring compounds. Previous work defined the steps in 2, 4 …

The pentaketide 1, 3, 6, 8-tetrahydroxynaphthalene (T4HN) is a key precursor of 1, 8-
dihydroxynaphthalene-melanin, an important virulence factor in pathogenic fungi, where …

Interactions between plants and soil microbial communities that benefit plant growth and
enhance nutrient acquisition are driven by the selective release of metabolites from plant …

A prevalent side-reaction of succinate dehydrogenase oxidizes malate to enol-oxaloacetate
(OAA), a metabolically inactive form of OAA that is a strong inhibitor of succinate …

Prokaryotic and eukaryotic fumarylacetoacetate hydrolase (FAH) superfamily members,
sharing conserved regions that form the so-called FAH-domain, catalyze a remarkable …

The classical approach to building the amino-acid residues into the initial electron-density
map requires days to weeks of a skilled investigator's time. Automating this procedure …

The enzymatic cleavage of C–C bonds in β-diketones is, comparatively, a little studied
biochemical process, but one that has important relevance to human metabolism …