Escherichia coli GH352, which was originally described as a temperature-sensitive strain containing a thermolabile acyl coenzyme A:monoacylglycerol 3-phosphate acyltransferase, does not now contain a thermolabile form of this enzyme. It has a defect in fructose-1,6-diphosphate aldolase and at least one additional temperature-sensitive lesion. Both strains GH352 and NP315, a temperature-sensitiv...

Hereditary fructose intolerance (HFI) is an autosomal recessive metabolic disorder caused by aldolase (fructosediphosphate aldolase, EC 4.1.2.13) B deficiency. The B isoform of aldolase is critical for the metabolism of exogenous fructose by the liver, kidney, and intestine, since it can use fructose-1-phosphate as substrate at physiological concentrations, unlike aldolases A and C. Affected su...

Aldolase C (zebrin) expression in Purkinje cells reveals stripe-shaped compartments in the cerebellar cortex. However, it is not clear how these compartments are related to cerebellar functional localization. Therefore, we identified olivocerebellar projections to aldolase C compartments by labeling climbing fibers with biotinylated dextran injected into various small areas within the inferior ...

The resurgence of aldolase isozymes in cancerous tissues is a well-known but poorly understood phenomenon. This resurgence poses the problem of whether or not adult and fetal aldolase isozymes are produced by the same cells. For clarification of this question, the immunoperoxidase technique was used to locate aldolases A, B, and C in one type of fast-growing hepatoma, the LF hepatoma and, by co...

Isozymes are enzymes catalyzing the same biochemical reaction, but differ in amino acid sequences. They usually display different kinetic parameters, or express different regulatory properties. In addition some isozymes show tissue-specific expressions. These differences are considered that isozymes have properties related to the tissue-specific environments. Aldolase, one example of isozymes, ...

1. Yao DC, Tolan DR, Murray MF, et al. Hemolytic anemia and severe rhabdomyolysis caused by compound heterozygous mutations of the gene for the erythrocyte/muscle isozyme of aldolase, ALDOA (Arg303X/Cys338Tyr). Blood. 2004;103:2401-2403. 2. Kishi H, Mukai T, Hirono A, et al. Human aldolase A deficiency associated with a hemolytic anemia: thermolabile aldolase due to a single base mutation. Proc...

2-Amino-3-ketobutyrate ligase catalyzes the reversible, pyridoxal 5'-phosphate-dependent condensation of glycine with acetyl CoA forming the unstable intermediate, 2-amino-3-ketobutyrate. Several independent lines of evidence indicate that the pure protein obtained in the purification of this ligase from Escherichia coli also has L-threonine aldolase activity. The evidence includes: (a), a cons...

Protein modification (sometimes known as crosslinking) often requires two or more steps to affix a small molecule irreversibly. Two-step reductive alkylation of the enzyme rabbit muscle aldolase with ethyl 3-C-acetoacetate and sodium cyanoborohydride attaches less radioactivity than with cyanoborohydride omitted. The C level incorporated into aldolase corresponds to only about 15–30 modified pr...

Hereditary fructose intolerance (HFI) is an autosomal recessive metabolic disorder caused by aldolase (fructosediphosphate aldolase, EC 4.1.2.13) B deficiency. The B isoform of aldolase is critical for the metabolism of exogenous fructose by the liver, kidney, and intestine, since it can use fructose-1-phosphate as substrate at physiological concentrations, unlike aldolases A and C. Affected su...