Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.2.1.23 (beta-galactosidase)
 14,648 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two types of GM-1 gangliosidosis were studied biochemically. Type 1 liver accumulated non-lipid hexosamine in addition to GM-1 ganglioside, but there was no increase of hexosamine and GM-1 in type 2 liver. The optimum pH of liver beta-galactosidase of type 1 and type 2 was 5--6 while that of the normal control was 4.5. Type 1 brain beta-galactosidase showed a slightly acidic optimum pH, i.e. 4.0 in comparison with that of the normal control. The optimum pH of type 2 brain beta-galactosidase was 5.5, like the liver enzyme. The thermostability of liver beta-galactosidase was the same in type 1 and type 2, while that of brain was different. Beta-galactosidase of type 1 and type 2 liver is more stable at 42 degrees C than the normal control, but a different thermostability was observed in type 1 and type 2 brain. Liver beta-galactosidase of type 1 showed one peak each at acid and neutral pH, and type 2 liver had only one peak at neutral pH.
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PMID:Enzymatic study of GM-1 gangliosidosis. 23 99

Isoenzymes of beta-galactosidase and of N-acetyl-beta-glucosaminidase were assayed during development of rat testis and as a function of hormonal treatments. Isoenzyme 1 of beta-galactosidase was highest in specific activity in the 4-day-old testis, at a point when Sertoli cells and gonocytes were the predominant cell type. Beta-galactosidase II, previously shown to be associated with the sperm acrosome, was undetectable through the spermatocyte stage of development, but increased in specific activity during the formation of spermatids. The specific activities of isoenzymes I and II of N-acetyl-beta-glucosaminidase increased markedly in association with the formation of spermatogonia and spermatocytes, and then declined with the appearance of spermatids. Following hypophysectomy of rats at 26 days of age or in adulthood the specific activities of the lysosomal enzymes beta-galactosidase I and N-acetyl-beta-glucosaminidase I and II increased markedly, while the acrosomal beta-galactosidase II was undetectable. The normal patterns of isoenzyme distributed were restored completely by administration of LH and FSH or testosterone to hypophysectomized animals. These results thus demonstrate specific patterns of isoenzyme concentration during spermatogenesis. Formation of the acrosome in developing spermatids is associated with the induction of new forms of beta-galactosidase (isoenzyme II) and N-acetyl-beta-glucosaminidase (sperm isoenzyme). These molecules appear to be specialized forms which may participate in fertilization, and their induction is dependent upon the actions of gonadotropins or testosterone.
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PMID:Hormonal regulation of isoenzymes of N-acetyl-beta-glucosaminidase and beta-galactosidase during spermatogenesis in the rat. 112 Apr 77

In alkali burned rabbit corneas activities of beta-glucuronidase, N-acetyl-beta-D-glucosaminidase and acid beta-galactosidase were studied histochemically in various time intervals after the traumatization. The technic with semipermeable membranes was employed. Within four days after the injury enzyme activities in the traumatized area were almost lacking. The corresponding activities in the unaffected part of the cornea were within the norm. On the 7th day enzyme activities were on an increase (but still subnormal) in the traumatized area. This area was surrounded by a zone of keratocytes with high levels of enzyme activities. This was particularly remarkable in keratocytes subjacent to the epithelium. The activation of all enzymes studied was present in the basal layer of the epithelium and in the endothelium as well. On the 14th day enzyme activities in the traumatized area were nearly restored and on the 32nd day they could not be distinguished from the normal cornea. Beta-galactosidase displayed a relatively maximal increase in the activity of all enzymes investigated.
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PMID:Alkali burns of the rabbit cornea. I. A histochemical study of beta-glucuronidase, beta-galactosidase and N-acetyl-beta-D-glucosaminidase. 119 85

The possibility of a role of N-acetylglucosaminidase or beta-galactosidase in the morphological change of lung lamellar body to lattice tubular myelin was examined in an in vitro incubation study. Electron microscopic observation of the incubation product and gas-chromatographic analysis of released sugars during incubation were used to understand the mechanism of these enzymes in the change of membrane constitution. N-acetylglucosaminidase proved to play a role in developing membrane of lamellar body toward lattice tubular structure with the support of Ca++. In this process, inositol release was indispensable in the modulation of membrane as well as the release of a small amount of N-acetylglucosamine. However, further reaction of this enzyme caused destruction or fusion of lattice-tubular integration with the release of other membrane components. Beta-galactosidase played no role in this process.
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PMID:Study on the change of lung lamellar body to lattice tubular myelin by N-acetylglucosaminidase with special reference to membrane components and calcium. 139 85

Permissiveness to Moloney Murine Leukemia Virus (MoMuLV) expression was examined during preimplantation and early postimplantation development of the mouse embryo. Blastocysts and 8th, 9th and 10th day postimplantation embryos were infected in vitro with a MoMuLV-based retroviral vector expressing the lacZ gene driven off an internal rat beta-actin promoter. Beta-galactosidase-positive cells were identified in all embryonic tissues including inner cell mass, epiblast, mesoderm, endoderm and definitive ectoderm. In contrast, embryos infected with a MoMuLV-based vector expressing the lacZ gene driven off the viral LTR showed beta-galactosidase-positive cells only in mesoderm and definitive ectoderm. We conclude that permissiveness to transcriptional activity of the LTR is acquired immediately upon differentiation of epiblast during gastrulation of the mouse embryo.
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PMID:Permissiveness to murine leukemia, virus expression during preimplantation and early postimplantation mouse development. 240 Dec 17

Beta-galactosidase conjugated to erythrocyte vesicles was measured by enzymatic and immunologic methods. The immunoassay used a monoclonal antibody and an anti-mouse IgG antibody conjugated to alkaline phosphatase in an adaptation of the enzyme-linked immunosorbent assay. The beta-galactosidase and alkaline phosphatase activities were compared by correlation. The significant correlation indicates that this method is satisfactory for relative quantitation of membrane-associated antigens with exposed epitopes.
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PMID:Assay of membrane-associated antigens with monoclonal antibody. 242 25

The RAD1 and RAD3 genes of Saccharomyces cerevisiae are required for excision repair of UV damaged DNA. In addition, the RAD3 gene is essential since rad3 deletions are recessive lethals. We have examined the induction of the RAD1 and RAD3 genes by DNA damage and during the cell division cycle. We have made fusions of the RAD1 and RAD3 genes with the Escherichia coli lacZ gene encoding beta-galactosidase. Beta-galactosidase activity was measured in a Rad+ yeast strain containing the RAD1-lacZ or the RAD3-lacZ fusion, either in a multicopy replicating plasmid or as a single copy integrant resulting from transformation with an integrating plasmid which transforms yeast by homologous recombination in the yeast genome. No induction of beta-galactosidase activity occurred after ultraviolet light (UV) or 4-nitroquinoline-1-oxide (NQO) treatment. Haploid cells of mating type a were synchronized by treatment with alpha factor and beta-galactosidase activity was determined during different cell cycle stages. No change in beta-galactosidase activity was observed in the strain containing the RAD1-lacZ or the RAD3-lacZ fusion integrated in the yeast genome.
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PMID:Expression of the RAD1 and RAD3 genes of Saccharomyces cerevisiae is not affected by DNA damage or during the cell division cycle. 392 99

Type III GM1-gangliosidosis is a rare hereditary storage disease caused by lack of lysosomal beta-galactosidase and characterized by a slowly progressive course, and extrapyramidal signs, but without prominent skeletal changes or visceromegaly. The storage substance was reported to be located only in the basal ganglia. There has been no detailed report on visceral lesions in type III GM1-gangliosidosis. In this report we describe a case of type III GM1-gangliosidosis, and the histochemical and ultrastructural findings from biopsied rectum. The patient was a 22-year-old female who exhibited dysarthria, gait disturbance, and generalized dystonia with rigidity. Beta-galactosidase activity in leukocytes was absent and sialidase activity in cultured fibroblasts was normal. Many histiocytes were found in biopsied rectal mucosa. Histochemical studies showed that the granules of histiocytes contained acidic glycoconjugates, beta-galactose, beta-N-acetylgalactosamine and sialic acid. Ultrastructural investigations revealed that ganglion cells of Meissner's plexus had many osmiophilic lamellar inclusions, similar to "membranous cytoplasmic bodies". These findings are crucial for the clinical diagnosis of type III GM1-gangliosidosis.
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PMID:Type III (chronic) GM1-gangliosidosis. Histochemical and ultrastructural studies of rectal biopsy. 393 2

It has been shown by us that the human blood-group MN antigenic determinants are not the products of allelomorphic genes as believed so far, but that N is the precursor substance of M and that the allelomorph to the M gene is amorph. The determinant structure of the N antigen is branched and possesses as non-reducing termini beta-d-galactopyranosyl (Gal) and alpha-N-acetylneuraminic acid (NANA) linked to beta-Gal. The M substance differs from N only in that alpha-NANA covers the terminal beta-Gal of the N determinant. Vicia graminea anti-N reacts with terminal beta-Gal of the N antigen as well as its precursor. A human blood-group N-like antigen in the cell surface of the TA3 mammary adenocarcinoma (ascites form) has been found by us. The TA3 cancer occurs as the non-strain specific Ha subline and as the strain-specific St subline. This is the first description of an N-like antigen in a non-primate as well as a tumor. This antigen reacts with Vicia anti-N. In serological specificity the Vicia agglutinin is closely related to the Thomsen-Friedenreich anti-T agglutinin present in most human and animal sera. These sera plus complement kill ordinary TA3-St cells and sialidase-treated Ha cells to less than 95 percent. Untreated TA3-Ha cells are fully resistant even though they absorb cytotoxin. Beta-galactosidase treatment of either Ha or St cells abolishes the killing activity of the sera. The cancer cells absorb anti-T but they lose this capability after exposure to beta-galactosidase. An immunological cross-relationship between the human blood-group MN antigens and the receptor for an oncogenic virus, the avian subgroup B leukosis sarcoma virus has been observed.
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PMID:Relation of human blood-groups MN to cancer cell surface antigens and to receptors for oncogenic viruses. 414 44

Activity of lactosyl ceramide beta-galactosidase (beta-D-galactoside galactohydrolase, EC 3.2.1.23) was found to be extremely low in enzyme preparations from liver, brain, and cultured skin fibroblasts from patients with Krabbe's disease. Leukocytes from one set of parents had enzyme levels approximately half those measured in control leukocytes. The low activity observed for this galactolipid hydrolase is the fourth enzymatic deficiency noted for this genetic disease. Beta-galactosidase activity toward galactocerebroside, psychosine, and monogalactosyl diglyceride is also low in patients with Krabbe's disease. Other lysosomal enzymes measured were found to be in the normal range. This enzymatic defect may provide a better explanation for the pathological and chemical findings previously reported for this syndrome.
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PMID:Globoid cell leukodystrophy: deficiency of lactosyl ceramide beta-galactosidase. 452 95


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