Some disturbances of erythrocyte metabolism in galactosaemia

Schwarz, V.; Golberg, L.; Komrower, G. M.; Holzel, A.

doi:10.1042/bj0620034

Cited by 187 publications

(40 citation statements)

References 8 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…These levels of gal-1-P were similar to those reported to occur in erythrocytes of patients with congenital galactosemia ( 11 ).…”

Section: Discussionsupporting

confidence: 89%

See 1 more Smart Citation

Progesterone Effects on Galactose Metabolism in Prepubertal Patients With Congenital Galactosemia and in Rats Maintained on High Galactose Diets*

Pesch¹,

Segal²,

Topper³

1960

J. Clin. Invest.

View full text Add to dashboard Cite

“…These levels of gal-1-P were similar to those reported to occur in erythrocytes of patients with congenital galactosemia ( 11 ).…”

Section: Discussionsupporting

confidence: 89%

“…These manifestations are thought to be the result of excess intracellular accumulation of gal-1-P (11,12). The biochemical lesion in this disease is a genetically determined deficiency of the enzyme P-Gal transuridylase as has been demonstrated by Isselbacher and associates (13) and Kalckar (14).…”

Section: Discussionmentioning

confidence: 90%

Progesterone Effects on Galactose Metabolism in Prepubertal Patients With Congenital Galactosemia and in Rats Maintained on High Galactose Diets*

Pesch¹,

Segal²,

Topper³

1960

J. Clin. Invest.

View full text Add to dashboard Cite

“…In human galactosaemia, D-Gall P can cause serious pathological effects due to genetic lack of D-Gall P uridylyltransferase (Schwarz et al, 1956). D-GallP is also known as an effector molecule of D-galactose-induced stasis of E. coli K12 with a genetic lack of the same enzyme Sundararajan, 1963;Yarmolinsky et al, 1959) as is L-RuSP in L-arabinose-induced stasis of E. coli B/r deficient in L-RuSP 4-epimerase (Englesberg et al, 1962).…”

Section: Discussionmentioning

confidence: 99%

Determination of effector molecules in L-arabinose-induced bulge formation and lysis of Escherichia coli IFO 3545

Tanaka

Muroi

Sunada

et al. 1991

Journal of General Microbiology

View full text Add to dashboard Cite

“…Accordingly, the results in patients with galactosemia are of interest. The concentrations of gal-I-P are increased in erythrocytes of patients with galactosemia (26,48), in liver (34,47), and kidney (47) because of gal-I-P uridyltransferase deficiency (3,26). Elevated gal-I-P levels have been associated with a markedly lower level of Pi in red blood cells (48) and in plasma (29,32), which suggests that a decrease in intracellular hepatic A T P might also occur after galactose ingestion: hepatic A T P content in galactosemic patients is not known.…”

Section: Discussionmentioning

confidence: 99%

Fructose-induced Hyperuricemia: Observations in Normal Children and in Patients with Hereditary Fructose Intolerance and Galactosemia

Kogut

Roe

et al. 1975

Pediatr Res

View full text Add to dashboard Cite

ExtractAfter the infusion of fructose, 0.25 g/kg body wt, the mean peak plasma uric acid lerel was 5.4 0.7 ( S E M ) mg/100 ml in six normal children and was not significantly increased compared with that of the mean basal kalue of 4.1 0.5 mg/100 ml. The mean blood inorganic phosphate ( P , ) levels were significantly less than the mean fasting value after fructose. Blood glucose. lactic acid, and fructose levels were significantly increased after fructose, but serum magnesium levels did not change.In two patients with hereditary fructose intolerance ( H F I ) the peak blood uric acid levels were 12.1 and 7.6 mg/100 ml. respectively, after fructose. In both patients the blood glucose concentrations decreased 69 and 26 mg/100 ml below the fasting levels after fructose. The serum P i level decreased 2.3 and 1.2 mg/100 ml below fasting values, decrements greater than the mean decrement in serum P, of 0.8 * 0.2 mg/100 ml which occurred in six normal children.The mean uric acid excretion, expressed as milligrams per mg urinary creatinine, was 0.6 i 0.1 ( S E M I before fructose in the normal children and increased significantly to 1.0 0.1 mg/mg creatinine after fructose. In two patients with HFI the uric acid excretion increased four-to fivefold after fructose administration; the increased uric acid excretion in HFI exceeded that of normal children.In three patients with galactosemia. increases in blood uric acid levels after galactose ingestion were similar to those in normal children after fructose, but less than those in patients with HFI after fructose. The serum P , levels decreased less in galactosemic patients after galactose administration than in patients with HFI after fructose infusion.These studies support the hypothesis that fructose-induced hyperuricemia results from degradation of adenosine monophosphate. This effect appears to be specific for fructose. The lack of hyperuricemia in galactosemia patients after galactose ingestion may be explained by the observation that galactose is phosphorylated more slowly than fructose. SpeculationFructose administration in individuals predisposed to hyperuricemia and gout may induce urate overproduction by decreasing intrahepatic P i and ATP content and ultimately lead to enhanced nucleotide catabolism. The pathogenesis of increased urate production in glycogen storage disease, type 1, may be similar to that proposed for fructose-induced hyperuricemia.

show abstract

Some disturbances of erythrocyte metabolism in galactosaemia

Cited by 187 publications

References 8 publications

Progesterone Effects on Galactose Metabolism in Prepubertal Patients With Congenital Galactosemia and in Rats Maintained on High Galactose Diets*

Progesterone Effects on Galactose Metabolism in Prepubertal Patients With Congenital Galactosemia and in Rats Maintained on High Galactose Diets*

Determination of effector molecules in L-arabinose-induced bulge formation and lysis of Escherichia coli IFO 3545

Fructose-induced Hyperuricemia: Observations in Normal Children and in Patients with Hereditary Fructose Intolerance and Galactosemia

Contact Info

Product

Resources

About