Autosomal recessive disorder; beta-galactosidase deficiency; neuronal storage of GM1 ganglioside and visceral storage of ... The GM1 gangliosidoses (or GM1 gangliosidosis) are caused by a deficiency of beta-galactosidase, with resulting abnormal ... Prenatal diagnosis is possible by measurement of Acid Beta Galactosidase in cultured amniotic cells. Bley, Annette E.; ... resulting from marked deficiency of Acid Beta Galactosidase. GM1 has three forms: early infantile, late infantile, and adult. ...
... beta-galactosidase (EC 3.2.1.23). Mammalian beta-galactosidase is a lysosomal enzyme (gene GLB1) which cleaves the terminal ...
... beta-galactosidase (EC 3.2.1.23); 6-phospho-beta-galactosidase (EC 3.2.1.85); 6-phospho-beta-glucosidase (EC 3.2.1.86); lactase ... 6-phospho-beta-galactosidase InterPro: IPR005928 GBA3; KL; KLB; LCT; LCTL; GH1 in CAZypedia Henrissat B, Callebaut I, Mornon JP ... Glycoside hydrolase family 1 CAZY GH_1 comprises enzymes with a number of known activities; beta-glucosidase (EC 3.2.1.21); ... hydrolase (EC 3.2.1.62), lactase (EC 3.2.1.108); beta-mannosidase (EC 3.2.1.25); myrosinase (EC 3.2.1.147). ...
... endo-beta-1,3-glucanase (EC 3.2.1.39); endo-beta-1,3-1,4-glucanase (EC 3.2.1.6); endo-beta-galactosidase (EC 3.2.1.103). ...
Galactosidase, beta 1-like 3 is a protein in humans that is encoded by the GLB1L3 gene. GRCh38: Ensembl release 89: ... ENSG00000166105 - Ensembl, May 2017 "Human PubMed Reference:". "Mouse PubMed Reference:". "Entrez Gene: Galactosidase, beta 1- ...
Langley, K. E.; Villarejo, M. R.; Fowler, A. V.; Zamenhof, P. J.; Zabin, I. (1975). "Molecular basis of beta-galactosidase ... β-galactosidase is a protein encoded by the lacZ gene of the lac operon, and it exists as a homotetramer in its active state. ... The presence of an active β-galactosidase can be detected by X-gal, a colourless analog of lactose that may be cleaved by β- ... However, a mutant β-galactosidase derived from the M15 strain of E. coli has its N-terminal residues 11-41 deleted and this ...
The bacterial lacZ gene encodes a beta-galactosidase enzyme. When media containing certain galactosides (e.g. X-gal) is added, ...
1991). "Human beta-galactosidase gene mutations in GM1-gangliosidosis: a common mutation among Japanese adult/chronic cases". ... 1994). "Normal serum beta-galactosidase in juvenile GM1 gangliosidosis". Pediatr. Neurol. 10 (4): 317-9. doi:10.1016/0887-8994( ... Chakraborty S, Rafi MA, Wenger DA (1994). "Mutations in the lysosomal beta-galactosidase gene that cause the adult form of GM1 ... 1991). "Combined deficiency of beta-galactosidase and neuraminidase: natural history of the disease in the first 18 years of an ...
... beta-galactosidase (EC 3.2.1.23); beta-mannosidase (EC 3.2.1.25); beta-glucuronidase (EC 3.2.1.31). These enzymes contain a ... The catalytic domain of Beta-galactosidases have a TIM barrel core surrounded several other largely beta domains. The sugar ... Matthews BW, Jacobson RH, Zhang XJ, DuBose RF (1994). "Three-dimensional structure of beta-galactosidase from E. coli". Nature ... beta-galactosidase from Escherichia coli". J. Biol. Chem. 267 (16): 11126-11130. PMID 1350782. ...
Neuraminidase deficiency with beta-galactosidase deficiency at NIH's Office of Rare Diseases Hide & Seek Foundation for ... in 1979 by measuring beta-galactosidase and neuraminidase activities in cultured amniotic fluid cells. Koike K, Hamaguchi T, ... This in turn leads to a secondary deficiency of beta-galactosidase (GLB1) and neuraminidase 1 (NEU1).[citation needed]There are ... "Prenatal diagnosis of sialidosis with combined neuraminidase and beta-galactosidase deficiency". Clinical Genetics. 16 (1): 60- ...
ATCC Flood, M. T.; Kondo, M (2004). "Toxicity evaluation of a beta-galactosidase preparation produced by Penicillium multicolor ... "Isolation and characterization of a beta-primeverosidase-like enzyme from Penicillium multicolor". Bioscience, Biotechnology, ...
In the lysosome, this enzyme is part of a heterotrimeric complex together with beta-galactosidase and cathepsin A (the latter ... Activation, stabilization and association with beta-galactosidase and its protective protein". Eur. J. Biochem. 149 (2): 315-21 ... "Association of N-acetylgalactosamine-6-sulfate sulfatase with the multienzyme lysosomal complex of beta-galactosidase, ...
... beta-galactosidase". Biochemistry. 40 (49): 14781-94. doi:10.1021/bi011727i. PMID 11732897. Fernandez P, Cañada FJ, Jiménez- ... Lactase (also known as lactase-phlorizin hydrolase, or LPH), a part of the β-galactosidase family of enzymes, is a glycoside ... The Lactase Protein E. coli β-galactosidase: PDB: 1JYY​ Gene Ontology for Lactase Making of the Fittest: Got Lactase? The Co- ... Hermida C, Corrales G, Cañada FJ, Aragón JJ, Fernández-Mayoralas A (Jul 2007). "Optimizing the enzymatic synthesis of beta-D- ...
Olson and Eglen; Eglen, RM (2007). "beta Galactosidase complementation: A cell-based luminescent assay platform for drug ...
... beta-galactosidase) Luciferase, including ReBiL (recombinase enhanced bimolecular luciferase) and the commercial products ... "Monitoring protein-protein interactions in intact eukaryotic cells by beta-galactosidase complementation". Proceedings of the ... The following proteins have been used in split protein PCAs: Beta-lactamase Dihydrofolate reductase (DHFR) Focal adhesion ... "Bacterial beta-lactamase fragmentation complementation strategy can be used as a method for identifying interacting protein ...
Fabrazyme (agalsidase beta, or Alpha-galactosidase) was licensed to the Genzyme Corporation. It is an enzyme replacement ... A deficiency of the enzyme alpha galactosidase A (a-GAL A, encoded by GLA) due to mutation causes a glycolipid known as ... to measure the level of alpha-galactosidase activity. An enzyme assay is not reliable for the diagnosis of disease in females ...
Ullmann, A.; Jacob, F.; Monod, J. (1968). "On the subunit structure of wild-type versus complemented beta-galactosidase of ... "The role of the inducible alleles and the constitutive alleles in the synthesis of beta-galactosidase in zygotes of Escherichia ... of a peptide fraction of Escherichia coli beta-galactosidase". Journal of Molecular Biology. 12 (3): 918-923. doi:10.1016/S0022 ... by in vitro complementation of a peptide corresponding to an operator-proximal segment of the beta-galactosidase structural ...
"Affibody-beta-galactosidase immunoconjugates produced as soluble fusion proteins in the Escherichia coli cytosol". J. Immunol. ...
"Analysis of synphilin-1 and synuclein interactions by yeast two-hybrid beta-galactosidase liquid assay". Neurosci. Lett. 325 (2 ... "Analysis of synphilin-1 and synuclein interactions by yeast two-hybrid beta-galactosidase liquid assay". Neurosci. Lett. 325 (2 ...
5 minutes for beta-galactosidase[citation needed]). Prokaryotic small ribosomal subunit (30S) Ribosomal RNA 23S methyl RNA ...
"Th1-associated immune responses to beta-galactosidase expressed by a replication-defective herpes simplex virus". Journal of ...
The glycosyl hydrolase 42 family CAZY GH_42 comprises beta-galactosidase enzymes (EC 3.2.1.23). These enzyme catalyse the ... beta-galactosidase gene (pbg) is located in the 3'-flanking region". Microbiol Immunol. 39 (9): 677-86. doi:10.1111/j.1348- ... hydrolysis of terminal, non-reducing terminal beta-D-galactoside residues. The middle domain of these three-domain enzymes is ...
"Efficient technique for screening drugs for activity against Trypanosoma cruzi using parasites expressing beta-galactosidase." ...
Matsumura, I; Ellington, AD (Jan 12, 2001). "In vitro evolution of beta-glucuronidase into a beta-galactosidase proceeds ...
"Analysis of synphilin-1 and synuclein interactions by yeast two-hybrid beta-galactosidase liquid assay". Neuroscience Letters. ... Beta amyloid Synuclein Contursi Terme - the village in Italy where a mutation in the α-synuclein gene led to a family history ... Uéda K, Saitoh T, Mori H (Dec 1994). "Tissue-dependent alternative splicing of mRNA for NACP, the precursor of non-A beta ... A single molecule study in 2008 suggests alpha-synuclein exists as a mix of unstructured, alpha-helix, and beta-sheet-rich ...
Beta-galactosidase. *Hexosaminidase. *mannosidase *alpha-Mannosidase. *beta-mannosidase. *Aspartylglucosaminidase. *Fucosidase ...