Galactosidase Beta (GLb)

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GL-B; b-GAL; bGAL; ELNR1; EBP; GLB1; Lactase; Elastin Receptor 1,67kDa; Elastin Receptor 1(67kD); Acid Beta-Galactosidase

Galactosidase Beta (GLb)

Galactosidase Beta (GLb) is a hydrolase enzyme that catalyzes the hydrolysis of β-galactosides into monosaccharides. Substrates of different β-galactosidases include ganglioside GM1, lactosylceramides, lactose, and various glycoproteins. Alternate or nicknames are "beta-gal" or "β-gal". Lactase is often confused as an alternative name for β-galactosidase, but it is actually simply a sub-class of β-galactosidase.Galactosidase, beta 1 is a beta-galactosidase that cleaves the terminal beta-galactose from ganglioside substrates and other glycoconjugates. GM1-gangliosidosis is a lysosomal storage disease that can be caused by a deficiency of β-galactosidase (GLB1). Some cases of Morquio syndrome B have been shown to be due to GLP1 mutations that cause patients to have abnormal elastic fibers. β-galactosidase

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA196Hu01 Recombinant Galactosidase Beta (GLb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA196Hu01 Polyclonal Antibody to Galactosidase Beta (GLb) WB; ICC; IHC-P; IHC-F; ELISA.
MAA196Hu22 Monoclonal Antibody to Galactosidase Beta (GLb) WB; IHC; ICC; IP.
LAA196Hu71 Biotin-Linked Polyclonal Antibody to Galactosidase Beta (GLb) WB; IHC; ICC.
LAA196Hu81 FITC-Linked Polyclonal Antibody to Galactosidase Beta (GLb) WB; IHC; ICC; IF.
Assay Kits SEA196Hu ELISA Kit for Galactosidase Beta (GLb) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA196Mu01 Recombinant Galactosidase Beta (GLb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA196Mu01 Polyclonal Antibody to Galactosidase Beta (GLb) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Galactosidase Beta (GLb) CLIA Kit Customized Service Offer
n/a ELISA Kit for Galactosidase Beta (GLb) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA196Ra01 Recombinant Galactosidase Beta (GLb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA196Ra01 Polyclonal Antibody to Galactosidase Beta (GLb) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Galactosidase Beta (GLb) CLIA Kit Customized Service Offer
n/a ELISA Kit for Galactosidase Beta (GLb) ELISA Kit Customized Service Offer

Organism species: Rhesus monkey (Simian)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA196Si01 Recombinant Galactosidase Beta (GLb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA196Si01 Polyclonal Antibody to Galactosidase Beta (GLb) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Galactosidase Beta (GLb) CLIA Kit Customized Service Offer
n/a ELISA Kit for Galactosidase Beta (GLb) ELISA Kit Customized Service Offer

Organism species: Canis familiaris; Canine (Dog)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA196Ca01 Recombinant Galactosidase Beta (GLb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA196Ca01 Polyclonal Antibody to Galactosidase Beta (GLb) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Galactosidase Beta (GLb) CLIA Kit Customized Service Offer
n/a ELISA Kit for Galactosidase Beta (GLb) ELISA Kit Customized Service Offer

Organism species: Bos taurus; Bovine (Cattle)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA196Bo01 Recombinant Galactosidase Beta (GLb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA196Bo01 Polyclonal Antibody to Galactosidase Beta (GLb) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Galactosidase Beta (GLb) CLIA Kit Customized Service Offer
n/a ELISA Kit for Galactosidase Beta (GLb) ELISA Kit Customized Service Offer

Organism species: Danio rerio (Zebrafish)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPA196Fi01 Recombinant Galactosidase Beta (GLb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAA196Fi01 Polyclonal Antibody to Galactosidase Beta (GLb) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Galactosidase Beta (GLb) CLIA Kit Customized Service Offer
n/a ELISA Kit for Galactosidase Beta (GLb) ELISA Kit Customized Service Offer
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  19. "A homozygous missense arginine to histidine substitution at position 482 of the beta-galactosidase in an Italian infantile GM1-gangliosidosis patient."Hum. Genet. 90:247-250(1992) [PubMed] [Europe PMC] [Abstract]
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  27. "Novel mutations (Asn 484 Lys, Thr 500 Ala, Gly 438 Glu) in Morquio B disease."Biochim. Biophys. Acta 1588:247-253(2002) [PubMed] [Europe PMC] [Abstract]
  28. "Six novel beta-galactosidase gene mutations in Brazilian patients with GM1-gangliosidosis."Hum. Mutat. 13:401-409(1999) [PubMed] [Europe PMC] [Abstract]
  29. "Characterization of beta-galactosidase mutations Asp332-->Asn and Arg148-->Ser, and a polymorphism, Ser532-->Gly, in a case of GM1 gangliosidosis."Biochem. J. 348:621-632(2000) [PubMed] [Europe PMC] [Abstract]
  30. "Beta-galactosidase gene mutations affecting the lysosomal enzyme and the elastin-binding protein in GM1-gangliosidosis patients with cardiac involvement."Hum. Mutat. 15:354-366(2000) [PubMed] [Europe PMC] [Abstract]
  31. "Mutation analyses in 17 patients with deficiency in acid beta-galactosidase: three novel point mutations and high correlation of mutation W273L with Morquio disease type B."Hum. Genet. 109:159-166(2001) [PubMed] [Europe PMC] [Abstract]
  32. "Modulating action of the new polymorphism L436F detected in the GLB1 gene of a type-II GM1 gangliosidosis patient."Hum. Genet. 113:44-50(2003) [PubMed] [Europe PMC] [Abstract]
  33. "Four novel mutations in patients from the Middle East with the infantile form of GM1-gangliosidosis."Hum. Mutat. 24:352-352(2004) [PubMed] [Europe PMC] [Abstract]
  34. ErratumHum. Mutat. 24:536-537(2004)
  35. "Role of beta-galactosidase and elastin binding protein in lysosomal and nonlysosomal complexes of patients with GM1-gangliosidosis."Hum. Mutat. 25:285-292(2005) [PubMed] [Europe PMC] [Abstract]
  36. "Magnetic resonance imaging findings and novel mutations in GM1 gangliosidosis."J. Child Neurol. 20:57-60(2005) [PubMed] [Europe PMC] [Abstract]
  37. "Dystonia and parkinsonism in GM1 type 3 gangliosidosis."Mov. Disord. 20:1366-1369(2005) [PubMed] [Europe PMC] [Abstract]
  38. "Twenty-one novel mutations in the GLB1 gene identified in a large group of GM1-gangliosidosis and Morquio B patients: possible common origin for the prevalent p.R59H mutation among Gypsies."Hum. Mutat. 27:1060-1060(2006) [PubMed] [Europe PMC] [Abstract]
  39. "Elastogenesis in cultured dermal fibroblasts from patients with lysosomal beta-galactosidase, protective protein/cathepsin A and neuraminidase-1 deficiencies."J. Med. Invest. 53:103-112(2006) [PubMed] [Europe PMC] [Abstract]
  40. "Identification of 14 novel GLB1 mutations, including five deletions, in 19 patients with GM1 gangliosidosis from South America."Clin. Genet. 71:273-279(2007) [PubMed] [Europe PMC] [Abstract]
  41. "Identification of a novel pseudodeficiency allele in the GLB1 gene in a carrier of GM1 gangliosidosis."Clin. Genet. 72:109-111(2007) [PubMed] [Europe PMC] [Abstract]
  42. "GM1 gangliosidosis and Morquio B disease: expression analysis of missense mutations affecting the catalytic site of acid beta-galactosidase."Hum. Mutat. 30:1214-1221(2009) [PubMed] [Europe PMC] [Abstract]