Corneal clouding is added to the list of clinical and chemical abnormalities which occur both in GM1-generalized gangliosidosis and in Hurler's syndrome (and some other mucopolysaccharidoses). The parents of our patient were first cousin Yemeni and had partial beta-galactosidase deficiency in their leucocytes and cultured fibroblasts.

beta-Galactosidase (beta-gal, EC 3.2.1.23) and beta-D-phosphogalactoside galactohydrolase (beta-Pgal) activities were observed in all of 13 Lactobacillus species studied except L. casei and L. buchneri. Only the latter enzyme was detected in nine strains of L. casei. The beta-gal from L. thermophilus and the beta-Pgal from L. casei were purified and characterized. In comparison with beta-gal, t...

When a strain of Escherichia coli, expressing a hybrid protein GalK-beta-Gal, is shifted to high osmolarity, the beta-galactosidase activity strongly decreases within 20 min of shock. The loss of beta-galactosidase activity results from degradation of the hybrid protein under osmotic stress. The results raise the possibility that osmotic stress induces a specific osmodependent protease.

[14C]Lactose electroinjected into isolated rat hepatocytes is normally autophagocytosed, transferred to lysosomes and degraded by lysosomal beta-galactosidase, but at high concentrations of asparagine the transfer is inhibited and lactose accumulates in prelysosomal autophagic/endocytic vacuoles (amphisomes). The accumulation can be prevented by addition of yeast beta-galactosidase, which is tr...

Using beta-galactosidase and luciferase as reporter enzymes, we have previously shown that enzymatic inactivation occurring during a heat shock is concomitant with protein insolubilization (Nguyen, V. T., Morange, M., and Bensaude, O. (1989) J. Biol. Chem. 264, 10487-10492). In this paper, we observe that pretreatment of cells with D2O and glycerol, compounds known to stabilize protein structur...

As the century drew to a close the orthodox teaching on man's need for food could be expressed in the following manner. There were five proximate principles of foods. Three, proteins, fats and carbohydrates, were organic, and two, salts and water, were inorganic.... It will be observed that this scheme makes no mention of any such food substances as the curative antiscorbutic principle of fresh...

During the preadult development of Drosophila melanogaster, the GLD (glucose dehydrogenase) gene (Gld) is expressed in a variety of tissues, including the immature reproductive tract. At the adult stage the expression of Gld becomes largely restricted to the reproductive tract of males and females. We examined the expression of GLD in the adult reproductive tract of 50 species in the genus Dros...

By use of a capillary electrophoresis-based procedure, it is possible to measure the activity of individual molecules of beta-galactosidase. Molecules from the crystallized enzyme as well as the original enzyme preparation used to grow the crystals both displayed a range of activity of 20-fold or greater. beta-Galactosidase molecules obtained from two different crystals had indistinguishable ac...

The rates of synthesis of beta-galactosidase (EC 3.2.1.23) and the intracellular levels of cyclic 3',5'-adenosine monophosphate (cAMP) soon after the addition of glucose or glycerol to exponentially growing cultures of Escherichia coli have been determined. Within 10 s of its addition, glucose, but not glycerol, lowered the apparent initiation frequency of lac messenger ribonucleic acid. The gl...

molar yields up to 36% were reported by protecting the product in situ against the hydrolytic activity of the same enzyme.'O} However, due to its biological source, this enzyrne seems to be of Iimited availability. Recently we have described a new fi-galactosidase from B. cireulans showing an extremely high specificity for the sequence of Gal-fi(1 .3)-GalNAc-ev-OR.") Because al1 figalactosidase...