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Western Blot (WB) (Western blot analysis of extracts of various cell lines, using GLB1 antibody.)

Rabbit GLB1 Polyclonal Antibody | anti-GLB1 antibody

GLB1 (Beta-Galactosidase 1, EBP, Elastin Receptor 1, ELNR1, MPS4B) (FITC)

Gene Names
GLB1; EBP; ELNR1; MPS4B
Reactivity
Human, Mouse, Rat
Applications
Immunohistochemistry, Western Blot
Purity
Purified by affinity chromatography.
Synonyms
GLB1; Polyclonal Antibody; GLB1 (Beta-Galactosidase 1; EBP; Elastin Receptor 1; ELNR1; MPS4B) (FITC); anti-GLB1 antibody
Ordering
For Research Use Only!
Host
Rabbit
Reactivity
Human, Mouse, Rat
Clonality
Polyclonal
Isotype
IgG
Specificity
Recognizes GLB1. Species Crossreactivity: human, mouse, rat
Purity/Purification
Purified by affinity chromatography.
Form/Format
Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).
Concentration
~1mg/ml (varies by lot)
Applicable Applications for anti-GLB1 antibody
Immunohistochemistry (IHC), Western Blot (WB)
Application Notes
IHC: 1:50-1:200
WB: 1:500-1:2000
Applications are based on unconjugated antibody.
Immunogen
Recombinant protein corresponding to human GLB1.
Conjugate
FITC
Note
Preservative Free
Preparation and Storage
Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western blot analysis of extracts of various cell lines, using GLB1 antibody.)

Western Blot (WB) (Western blot analysis of extracts of various cell lines, using GLB1 antibody.)
Product Categories/Family for anti-GLB1 antibody

NCBI and Uniprot Product Information

NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Accession #
Molecular Weight
76,075 Da
NCBI Official Full Name
beta-galactosidase isoform a preproprotein
NCBI Official Synonym Full Names
galactosidase, beta 1
NCBI Official Symbol
GLB1
NCBI Official Synonym Symbols
EBP; ELNR1; MPS4B
NCBI Protein Information
beta-galactosidase; lactase; acid beta-galactosidase; elastin receptor 1, 67kDa
UniProt Protein Name
Beta-galactosidase
Protein Family
UniProt Gene Name
GLB1
UniProt Synonym Gene Names
ELNR1; Lactase
UniProt Entry Name
BGAL_HUMAN

NCBI Description

This gene encodes beta-galactosidase-1, a lysosomal enzyme that hydrolyzes the terminal beta-galactose from ganglioside substrates and other glycoconjugates. Defects in this gene are the cause of GM1-gangliosidosis and Morquio B syndrome. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2008]

Uniprot Description

GLB1: Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans. Defects in GLB1 are the cause of GM1-gangliosidosis type 1 (GM1G1); also known as infantile GM1- gangliosidosis. GM1-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM1 gangliosides, glycoproteins and keratan sulfate primarily in neurons of the central nervous system. GM1G1 is characterized by onset within the first three months of life, central nervous system degeneration, coarse facial features, hepatosplenomegaly, skeletal dysmorphology reminiscent of Hurler syndrome, and rapidly progressive psychomotor deterioration. Urinary oligosaccharide levels are high. It leads to death usually between the first and second year of life. Defects in GLB1 are the cause of GM1-gangliosidosis type 2 (GM1G2); also known as late infantile/juvenile GM1- gangliosidosis. GM1G2 is characterized by onset between ages 1 and 5. The main symptom is locomotor ataxia, ultimately leading to a state of decerebration with epileptic seizures. Patients do not display the skeletal changes associated with the infantile form, but they nonetheless excrete elevated amounts of beta-linked galactose-terminal oligosaccharides. Inheritance is autosomal recessive. Defects in GLB1 are the cause of GM1-gangliosidosis type 3 (GM1G3); also known as adult or chronic GM1- gangliosidosis. GM1G3 is characterized by a variable phenotype. Patients show mild skeletal abnormalities, dysarthria, gait disturbance, dystonia and visual impairment. Visceromegaly is absent. Intellectual deficit can initially be mild or absent but progresses over time. Inheritance is autosomal recessive. Defects in GLB1 are the cause of mucopolysaccharidosis type 4B (MPS4B); also known as Morquio syndrome B. MPS4B is a form of mucopolysaccharidosis type 4, an autosomal recessive lysosomal storage disease characterized by intracellular accumulation of keratan sulfate and chondroitin-6-sulfate. Key clinical features include short stature, skeletal dysplasia, dental anomalies, and corneal clouding. Intelligence is normal and there is no direct central nervous system involvement, although the skeletal changes may result in neurologic complications. There is variable severity, but patients with the severe phenotype usually do not survive past the second or third decade of life. Belongs to the glycosyl hydrolase 35 family. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Glycan Metabolism - other glycan degradation; Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series; Glycan Metabolism - glycosaminoglycan degradation; Lipid Metabolism - sphingolipid; EC 3.2.1.23; Carbohydrate Metabolism - galactose; Hydrolase

Chromosomal Location of Human Ortholog: 3p21.33

Cellular Component: Golgi apparatus; lysosomal lumen; perinuclear region of cytoplasm; cytoplasm; vacuole

Molecular Function: protein binding; galactoside binding; beta-galactosidase activity

Biological Process: keratan sulfate metabolic process; sphingolipid metabolic process; dolichol-linked oligosaccharide biosynthetic process; glycosaminoglycan metabolic process; pathogenesis; post-translational protein modification; galactose catabolic process; glycosaminoglycan catabolic process; cellular protein metabolic process; carbohydrate metabolic process; protein amino acid N-linked glycosylation via asparagine; glycosphingolipid metabolic process; cellular carbohydrate metabolic process; keratan sulfate catabolic process

Disease: Gm1-gangliosidosis, Type Iii; Mucopolysaccharidosis Type Ivb; Gm1-gangliosidosis, Type Ii; Gm1-gangliosidosis, Type I

Research Articles on GLB1

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Product Notes

The GLB1 glb1 (Catalog #AAA6418568) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The GLB1 (Beta-Galactosidase 1, EBP, Elastin Receptor 1, ELNR1, MPS4B) (FITC) reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's GLB1 can be used in a range of immunoassay formats including, but not limited to, Immunohistochemistry (IHC), Western Blot (WB). IHC: 1:50-1:200 WB: 1:500-1:2000 Applications are based on unconjugated antibody. Researchers should empirically determine the suitability of the GLB1 glb1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "GLB1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.

Precautions

All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.

Disclaimer

Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.

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