The biochemical basis of hereditary fructose intolerance

N Bouteldja, DJ Timson - Journal of inherited metabolic disease, 2010 - Springer
Hereditary fructose intolerance is a rare, but potentially lethal, inherited disorder of fructose
metabolism, caused by mutation of the aldolase B gene. Treatment currently relies solely on …

Fructose metabolism in the cerebellum

VA Funari, JE Crandall, DR Tolan - The Cerebellum, 2007 - Springer
Under normal physiological conditions, the brain utilizes only a small number of carbon
sources for energy. Recently, there is growing molecular and biochemical evidence that …

Thermal ring-based heat switches in hyperthermophilic class II bacterial fructose aldolase

G Wang - ACS omega, 2023 - ACS Publications
Both thermophilic and hyperthermophilic enzymes in bacterial and archaeal species are
activated above a specific temperature threshold but inactivated at another higher …

The network basis for the structural thermostability and the functional thermoactivity of aldolase B

G Wang - Molecules, 2023 - mdpi.com
Thermostability is important for the thermoactivity of proteins including enzymes. However, it
is still challenging to pinpoint the specific structural factors for different temperature …

Epidemiological aspects of hereditary fructose intolerance: A database study

FC Pinheiro, F Sperb‐Ludwig, IVD Schwartz - Human Mutation, 2021 - Wiley Online Library
Hereditary fructose intolerance (HFI) is an inborn error of fructose metabolism of autosomal
recessive inheritance caused by pathogenic variants in the ALDOB gene that lead to …

Evolution of protein quaternary structure in response to selective pressure for increased thermostability

NJ Fraser, JW Liu, PD Mabbitt, GJ Correy… - Journal of Molecular …, 2016 - Elsevier
Oligomerization has been suggested to be an important mechanism for increasing or
maintaining the thermostability of proteins. Although it is evident that protein–protein …

Effects of pH, temperature, and sucrose on benzyl alcohol-induced aggregation of recombinant human granulocyte colony stimulating factor

R Thirumangalathu, S Krishnan, DN Brems… - Journal of …, 2006 - Elsevier
Antimicrobial preservatives (eg, benzyl alcohol), which are required in multidose
formulations, can induce protein aggregation. In this study, the mechanism of benzyl alcohol …

Increased prevalence of mutant null alleles that cause hereditary fructose intolerance in the American population

EM Coffee, L Yerkes, EP Ewen, T Zee… - Journal of inherited …, 2010 - Springer
Mutations in the aldolase B gene (ALDOB) impairing enzyme activity toward fructose-1-
phosphate cleavage cause hereditary fructose intolerance (HFI). Diagnosis of the disease is …

Mutations in the promoter region of the aldolase B gene that cause hereditary fructose intolerance

EM Coffee, DR Tolan - Journal of inherited metabolic disease, 2010 - Springer
Hereditary fructose intolerance (HFI) is a potentially fatal inherited metabolic disease caused
by a deficiency of aldolase B activity in the liver and kidney. Over 40 disease-causing …

Structure of the thermolabile mutant aldolase B, A149P: molecular basis of hereditary fructose intolerance

AD Malay, KN Allen, DR Tolan - Journal of molecular biology, 2005 - Elsevier
Hereditary fructose intolerance (HFI) is a potentially lethal inborn error in metabolism caused
by mutations in the aldolase B gene, which is critical for gluconeogenesis and fructose …