Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: EC:3.2.1.23 (beta-galactosidase)
14,648 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two water-soluble complex carbohydrate storage products were isolated from tissues and urine of patients with an inherited deficiency of lysosomal alpha-L-fucosidase (fucosidosis). The major component was an oligosaccharide of approximate molecular weight 1700, indicating that it was a dekasaccharide. From a combination of sequential digestion with purified exo-glycosidases, periodate oxidation and permethylation in conjunction with gas-liquid chromatography mass spectrometric analysis, the structure was found to be: Fuc(alpha 1 leads to 2)Gal-(beta 1 leads to 4) GlcNAc (beta1 leads to 2)Man [Fuc(alpha1 leads to 2) Gal (beta1 leads to 4) GlcNAc(beta1 leads to 2) Man] (alpha 1 leads to 3/6) Man (beta1 leads to 4) GlcNAc, although there was some evidence for heterogeneity at the mannose branchpoint. This material is structurally related to the stored oligosaccharides in patients with inherited deficiencies of beta-galactosidase (G M1-gangliosidosis) and N-acetyl-beta-hexosaminidase (G M2-gangliosidosis). A dissaccharide with the probable structure Fuc(alpha1 leads to 6)GlcNAc was found in lesser amounts in tissues; both are believed to be derived from the impaired catabolism of large numbers of different glycoproteins.
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PMID:Structure of the accumulating oligosaccharide in fucosidosis. 97 44

1. An endo-beta-galactosidase from Escherichia freundii, specific for the hydrolysis of desulfated keratan sulfate, quantitatively liberated a trisaccharide (Gal-GlcNAc-Gal) from a glycopeptide (Mr 1800) isolated from the liver of a patient with GM 1 (generalized) gangliosidosis. 2. The remaining glycopeptide was susceptible to sequential digestion with purified beta-N-acetylhexosaminidase and exo-beta-galactosidase from Jack Bean meal. 3. These and other studies established the structure of the stored glycopeptide to be:(see article) which probably represents the desulfated linkage region of skeletal keratan sulfate.
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PMID:Structure of the glycopeptide storage material in GM 1 gangliosidosis. Sequence determination with specific endo- and exoglycosidases. 109 58

Two major glycoasparagines (2-acetamido-N-(4'-L-aspartyl)-2-deoxy-beta-D-glycosylamines) were isolated from the urine of patients with aspartylglycosylaminuria (AGU). They were composed of equimolar amounts of sialic acid, galactose, glucosamine, and aspartic acid. They were isomeric with respect to the position of sialic acid attachment, since they produced the same glycoasparagine on incubation with the neuraminidase from Clostridium perfringens. The structure of the resulting sialic acid-free glycoasparagine was determined as beta-Gal-(1 leads to 4)-beta-GlcNAc-Asn based on the following findings. It produced galactose on incubation with beta-galactosidase, and N-acetyllactosamine and aspartic acid on incubation with 4-L-aspartylglycosylamine amindo hydrolase.
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PMID:Characterization of two glycoasparagines isolated from the urine of patients with aspartylglycosylaminuria (AGU). 121 85

Introducing genes into adult neurons in vivo may be a useful experimental tool for studying and modifying neuronal function. In this study two herpes simplex virus type 1 (HSV-1) mutants were used to examine the capability of different types of neostriatal neurons to express a foreign gene introduced through viral infection. In these HSV-1 mutants (7134 and RH105) the Escherichia coli gene, lacZ, under the control of viral promoters active during the early phase of infection, was substituted for viral genes (ICPO and TK, respectively) needed for efficient replication in the nervous system. Adult male rats received unilateral injections of HSV-1 mutant 7134 or RH105 into the neostriatum. Animals survived for 1 to 70 days with no apparent adverse physiological or behavioral effects. At the injection site, both mutant viruses produced focal tissue necrosis and reactive gliosis. Histochemical detection of the lacZ gene product, beta-galactosidase (beta Gal), revealed extensive labeling of neurons with mutant 7134 and relatively limited neuronal labeling with the mutant RH105. Mutant 7134, which is capable of some replication in cells, conferred beta Gal expression in cells over an area that was twofold greater than the necrotic area. In contrast, mutant RH105, which cannot replicate in cells, produced a zone of beta Gal-labeled cells only two-thirds the area of the necrotic core. Both medium- and large-sized neostriatal neurons were positive for beta Gal, and a higher proportion of large cells were labeled as compared to other neuronal populations in the normal striatum. A few glial cells were also beta Gal-positive. Retrograde transport of virus to the substantia nigra pars compacta and to the cortex was minimal and occurred only with mutant 7134. No evidence was seen for anterograde transport. Immunohistochemical localization of beta Gal at the ultrastructural level after inoculation with mutant 7134 revealed that both types of medium-sized neurons (spiny and aspiny types), as well as large neurons, were infected 3 days following inoculation. Immunoreactive neurons ranged from severely pathologic to remarkably healthy. Some of the axon terminals that contacted beta Gal-immunoreactive dendrites and spines were degenerated. These results demonstrate that in the adult rat replication-deficient HSV-1 vectors injected intrastriatally can be used to express a foreign gene in at least three types of neostriatal neurons, while maintaining the long-term survival and general health of the injected animals. The neurotoxicity induced by HSV-1 mutants may still be considerable, however, and ways of minimizing neuropathological effects need to be addressed.
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PMID:Introduction of a foreign gene (Escherichia coli lacZ) into rat neostriatal neurons using herpes simplex virus mutants: a light and electron microscopic study. 131 Dec 66

Defined herpes simplex virus type 1 (HSV-1) mutants KOS/1 and KOS/62 (positive and negative, respectively, for latency-associated transcripts [LATs]) express the Escherichia coli beta-galactosidase (beta-Gal) gene during latency. These mutants were employed to assess the functions of the latency-associated transcription unit on establishment and maintenance of and reactivation from the latent state. It was found that in the trigeminal ganglia, the frequencies of hyperthermia-induced reactivation of KOS/62 and an additional LATs- mutant (KOS/29) were reduced by at least 80%. Quantification of latently infected neurons expressing the beta-Gal gene revealed that the LATs- mutant KOS/62 established approximately 80% fewer latent infections in the trigeminal ganglia than did KOS/1 (LATs+). This reduction in establishment which is evident in the trigeminal ganglia could account for the reduced frequency of reactivation from this site. In striking contrast, both LATs- mutants reactivated with wild-type frequencies from lumbosacral ganglia. Quantification of beta-Gal-positive neurons at this site revealed that KOS/62 established as many as or more latent infections than the LATs+ virus, KOS/1. Colocalization of HSV antigen and beta-Gal suggested that the decreased establishment by LATs- mutants in trigeminal ganglia was the result of inefficient viral shutoff. Thus, one function of the HSV-1 LATs transcription unit is to promote the establishment of latency in trigeminal but not lumbosacral ganglia. Such a function may be relevant to understanding the distinct clinical recurrent disease patterns of HSV-1 and HSV-2.
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PMID:Herpes simplex virus type 1 latency-associated transcription unit promotes anatomical site-dependent establishment and reactivation from latency. 131 26

The retrograde transneuronal viral tracing method was used to study the CNS nuclei that innervate the parasympathetic preganglionic neurons controlling the submandibular gland in the rat. A genetically engineered beta-galactosidase expressing Bartha strain of pseudorabies virus (PRV) was injected into the submandibular gland of rats. After 4 days, PRV infected tissues were reacted with the Bluo-Gal substrate (halogenated indolyl-beta-D-galactoside) and labeled cell bodies were identified throughout the brain. In the medulla oblongata, cell body labeling was seen in the superior salivatory nucleus, and throughout the medullary reticular formation as well as in the nucleus of the solitary tract, spinal trigeminal nucleus, and deep cerebellar nuclei. In the pons, PRV labeled neurons were found bilaterally in the locus ceruleus, subceruleus region, and parabrachial complex. In the mesencephalon, labeled cells were found in the Edinger-Westphal nucleus, deep mesencephalic nucleus, and central grey matter. Several hypothalamic regions were labeled including the lateral, perifornical and paraventricular hypothalamic nuclei. In the telencephalon, PRV-positive cell bodies were observed in the substantia innominata, bed nucleus of the stria terminalis and central nucleus of the amygdala. The results suggest that widespread areas of the CNS are involved in control of salivation.
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PMID:CNS cell groups projecting to the submandibular parasympathetic preganglionic neurons in the rat: a retrograde transneuronal viral cell body labeling study. 131 71

Escherichia coli promoters that are more active at low temperature (15 to 20 degrees C) than at 37 degrees C were identified by using the transposon Tn5-lac to generate promoter fusions expressing beta-galactosidase (beta-Gal). Tn5-lac insertions that resulted in low-temperature-regulated beta-Gal expression were isolated by selecting kanamycin-resistant mutants capable of growth on lactose minimal medium at 15 degrees C but which grew poorly at 37 degrees C on this medium. Seven independent mutants were selected for further studies. In one such strain, designated WQ11, a temperature shift from 37 degrees C to either 20 or 15 degrees C resulted in a 15- to 24-fold induction of beta-Gal expression. Extended growth at 20 or 15 degrees C resulted in 36- to 42-fold-higher beta-Gal expression over that of cells grown at 37 degrees C. Treatment of WQ11 with streptomycin, reported to induce a response similar to heat shock, failed to induce beta-Gal expression. In contrast, treatment with either chloramphenicol or tetracycline, which mimics a cold shock response, resulted in a fourfold induction of beta-Gal expression in strain WQ11. Hfr genetic mapping studies complemented by physical mapping indicated that in at least three mutants (WQ3, WQ6, and WQ11), Tn5-lac insertions mapped at unique sites where no known cold shock genes have been reported. The Tn5-lac insertions of these mutants mapped to 81, 12, and 34 min on the E. coli chromosome, respectively. The cold-inducible promoters from two of the mutants (WQ3 and WQ11) were cloned and sequenced, and their temperature regulation was examined. Comparison of the nucleotide sequences of these two promoters with the regulatory elements of other known cold shock genes identified the sequence CCAAT as a putative conserved motif.
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PMID:Identification and characterization of novel low-temperature-inducible promoters of Escherichia coli. 133 67

The cDNA coding for pre-peanut agglutinin (PNA) was isolated from a bacterial expression library. It codes for a polypeptide of 273 amino acids composed of a hydrophobic signal peptide of 23 amino acids and a mature protein of 250 amino acids. The sequence of the latter is identical to that of native PNA, determined very recently by conventional methods, except that it contains 14 additional amino acids at the C-terminus. Bacterial cells harboring a plasmid with the prePNA-cDNA, produced two PNA cross-reacting proteins: one migrated on SDS-PAGE identically with the native lectin (apparent mol. wt. 31 kDa); the other, at 35 kDa, was a beta-galactosidase pre-PNA fusion protein. The former protein possessed an N-terminal sequence identical to that of the mature, native PNA, suggesting that it was processed from the 35 kDa prePNA precursor. Only the 31 kDa protein was exported into the bacterial periplasmic space, and had the ability to bind to galactose-Sepharose. The isolated processed protein had the same hemagglutinating activity as the native lectin, when assayed with sialidase-treated human erythrocytes. Like the native lectin, it did not agglutinate the untreated cells, was not inhibited by N-acetylgalactosamine, and was inhibited by Gal beta 1----3GalNAc 30-times more strongly than by galactose.
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PMID:Cloning, sequence analysis and expression in Escherichia coli of the cDNA encoding a precursor of peanut agglutinin. 133 58

Tammar wallaby (Macropus eugenii) mammary glands contain a UDP-GlcNAc:Gal beta 1----3Gal beta 1----4Glc beta 1----6-N-acetylglucosaminyltransferase (GlcNAcT) whose activity has been characterized with respect to the effect of pH, apparent Km for acceptor, effects of bivalent metal ions, acceptor specificity and identity of products. The enzyme did not show an absolute requirement for any bivalent metal ion but its activity was increased markedly by Mg2+, Ca2+ and Ba2+ and, to a lesser extent, by Mn2+. When Gal beta 1----3Gal beta 1----4Glc was used as acceptor, the product was Gal beta 1----3[GlcNAc beta 1----6]Gal beta 1----4Glc. With Gal beta 1----3Gal beta 1----3Gal beta 1----4Glc as acceptor, the product was shown, by 1H-NMR spectroscopy and exo-beta-galactosidase digestion, to be a novel pentasaccharide with the structure Gal beta 1----3[GlcNAc beta 1----6]Gal beta 1----3Gal beta 1----4Glc, suggesting that the enzyme recognises the non-reducing end of the acceptor substrate, rather than the reducing end.
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PMID:Biosynthesis of marsupial milk oligosaccharides. II: Characterization of a beta 6-N-acetylglucosaminyltransferase in lactating mammary glands of the tammar wallaby, Macropus eugenii. 138 55

In order to screen for developmentally active chromosomal domains during zebrafish embryogenesis, we generated transgenic fish by microinjecting two different lacZ reporter constructs into fertilized eggs. Transgenic fish were screened among the progeny of injected fish (F0) crossed to non-injected fish. Groups of 15 to 20 progeny of each cross were tested for lacZ expression and/or transmission of injected sequences using PCR and Southern hybridizations. Progeny from 2 of 102 fish injected with supercoiled constructs containing Rous sarcoma virus promoter sequences showed apparently spatially regulated beta-galactosidase (beta-Gal) activity. However, we were not able to detect this reporter construct in DNA from fins of F1 fish. Injections of a linear reporter construct containing mouse heat-shock promoter sequences revealed transmission of injected sequences to F1 progeny in about 6% of cases (8 of 129 fish, tested with PCR). We found one lacZ-expressing line that showed a spatially and temporally restricted expression of lacZ and, therefore, features typical characteristics of "enhancer trap" lines. In this line, lacZ expression starts at 16 hours post-fertilization in trigeminal ganglion cells. At about 24 hours lacZ expression can be detected in trigeminal ganglion neurons and Rohon-Beard neurons, indicating that the development of these two cell types shows common features. The reporter gene has integrated as a single copy. The founder fish was mosaic: 19% of its offspring (3 of 16 tested animals) carried the reporter construct in their fins; about 51% (13 of 27 tested animals) of the progeny of F1 fish were beta-Gal positive indicating full hemizygosity.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:A transgene containing lacZ is expressed in primary sensory neurons in zebrafish. 142 33


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