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Two lipoxygenase isoenzymes (linoleate: oxygen oxidoreductase, EC present in the embryo of germinating barley seed have been purified to homogeneity and characterized. Both isoenzymes are monomeric proteins with a molecular mass of approx. 90 kDa and crossreact on Western blots with antibodies raised against pea lipoxygenase. They have an(More)
Acid sphingomyelinase (sphingomyelin phosphodiesterase, EC was purified from human urine in the presence of 0.1% Nonidet P-40. The activity could be enriched 23,000-fold by sequential chromatography on octyl-Sepharose, concanavalin A-Sepharose, blue Sepharose and DEAE-cellulose. The last purification step yielded an enzyme preparation with a(More)
Labelling of cultured human skin fibroblasts from either control subjects or patients with mucolipidosis II (I-cell disease) with [32P]phosphate resulted in tight association of phosphate with immunoprecipitated glucocerebrosidase, a membrane-associated lysosomal enzyme. Endoglycosidase F digestion of the immunoprecipitated glucocerebrosidase did not(More)
In Petunia hybrida four complementary genes are present, each having, if homozygous recessive a blueing effect on the flower colour. These genes have no qualitative or quantitative effect on anthocyanins and flavonols. In mutants homozygous recessive for one (or more) of the Ph genes the pH of aqueous flower limb homogenates is increased. It is assumed that(More)
In the present investigation, we have demonstrated that three lysosomal-type hydrolases, alpha-glucosidase, alpha-mannosidase and a phosphatase, are present in lamellar bodies isolated from adult human lung. The hydrolase activities that were studied, all showed an acidic pH optimum, which is characteristic for lysosomal enzymes. The properties of acid(More)
In crossing experiments with Petunia hybrida, new mutations, some unstable, have been found in descendants of plants having an unstable allele of the anthocyanin gene An1. One of the unstable mutations affecting the new anthocyanin gene An11 was genetically analyzed, and it was subsequently established in which step of anthocyanin synthesis that An11 is(More)
An anthocyanin 5-O-glucosyltransferase from flowers of Petunia hybrida was purified about 30-fold. Using uridine 5'-diphosphoglucose as glucose donor (Km 0.22 mM), the enzyme glucosylated the 3-(p-coumaroyl)-rutinoside derivatives of delphinidin and petunidin (Km 3 μM), isolated from pollen of Petunia. Delphinidin 3-rutinoside, cyanidin 3-rutinoside and(More)
In patients with cerebro-hepato-renal (Zellweger) syndrome, the absence of peroxisomes results in an impairment of metabolic processes in which peroxisomes are normally involved. These include the catabolism of very long chain (greater than C22) fatty acids, the biosynthesis of ether-phospholipids and of bile acids, the catabolism of phytanic acid and the(More)
A mutable allele of the An1 locus in Petunia hybrida has given rise to a multiple series of stable derivative alleles. Anthocyanin concentration in mature flowers of these mutants (an1(+/ p)/an1) decreases from the wild-type red to the recessive white in a continuous series. Anthocyanin composition changes regularly: the ratio of peonidin to cyanidin is 3.5(More)
An immunogold labeling procedure was applied to ultrathin cryosections and used to study the subcellular localization of glucocerebrosidase in lipid-laden "Gaucher cells" in spleen from a patient with type 1 Gaucher's disease. Glucocerebrosidase protein was associated with the characteristic stored lipid material in large, irregularly shaped vacuoles. As(More)