Rb X b Galactosidase

Species Reactivity	Key Applications	Host	Format	Antibody Type
Vrt, E. coli	ELISA, IP, WB, IHC	Rb	Serum	Polyclonal Antibody

Species Reactivity

Key Applications

Host

Format

Antibody Type

Vrt, E. coli

ELISA, IP, WB, IHC

Serum

Polyclonal Antibody

Description
Catalogue Number	AB986
Brand Family	Chemicon®
Trade Name	Chemicon
Description	Anti-β Galactosidase Antibody, bacterial

Description

Catalogue Number

AB986

Brand Family

Chemicon®

Trade Name

Chemicon

Description

Anti-β Galactosidase Antibody, bacterial

Product Information
Format	Serum
Presentation	Rabbit antisera prepared by delipidation and defibrination. Lyophilized. Reconstitute with 2 mL of sterile deionized water. Contains 0.02M Potassium Phosphate, 0.15M NaCl and 0.01% sodium azide after reconstitution.

Product Information

Format

Serum

Presentation

Rabbit antisera prepared by delipidation and defibrination. Lyophilized. Reconstitute with 2 mL of sterile deionized water. Contains 0.02M Potassium Phosphate, 0.15M NaCl and 0.01% sodium azide after reconstitution.

Applications
Application	Anti-β Galactosidase Antibody, bacterial detects level of β Galactosidase & has been published & validated for use in ELISA, IP, WB, IH.
Key Applications	ELISA Immunoprecipitation Western Blotting Immunohistochemistry
Application Notes	Immunoblotting: 1:500-1:2,000 Immunoprecipitation Immunohistochemistry (Zhang, 2002) ELISA: 1:2,000-1:10,000 when tested against 1 μg of immunogen by a standard sandwich ELISA. Optimal working dilutions must be determined by the end user.

Applications

Application

Anti-β Galactosidase Antibody, bacterial detects level of β Galactosidase & has been published & validated for use in ELISA, IP, WB, IH.

Key Applications

ELISA
Immunoprecipitation
Western Blotting
Immunohistochemistry

Application Notes

Immunoblotting: 1:500-1:2,000

Immunoprecipitation

Immunohistochemistry (Zhang, 2002)

ELISA: 1:2,000-1:10,000 when tested against 1 μg of immunogen by a standard sandwich ELISA.

Optimal working dilutions must be determined by the end user.

Biological Information
Immunogen	Beta galactosidase from E-coli.
Epitope	bacterial
Host	Rabbit
Specificity	Specific for Beta galactosidase from E coli by IEP. May cross react with Beta galactosidase from other species.
Species Reactivity	Vertebrates E. coli
Antibody Type	Polyclonal Antibody
Entrez Gene Number	NM_000404.2 NM_001079811.1
Entrez Gene Summary	The GLB1 gene encodes beta-galactosidase-1 (EC 3.2.1.23), a lysosomal hydrolase that cleaves the terminal beta-galactose from ganglioside substrates and other glycoconjugates (Yoshida et al., 1991 [PubMed 1907800]). Beta-galactosidase also occurs in a complex with neuraminidase (NEU1; MIM 608272) and protective protein/cathepsin A (PPCA; MIM 256540), which is a component of certain cell surface receptors (Hinek, 1996 [PubMed 8922281]). See also galactosylceramidase (GALC; MIM 606890) (EC 3.2.1.46), a genetically distinct beta-galactosidase that is involved in the catabolism of other lipid compounds.[supplied by OMIM]
Gene Symbol	GLB1 Lactase S-Gal ELNR1 EBP EC 3.2.1.23
UniProt Number	P16279
UniProt Summary	FUNCTION: SwissProt: P16279 # This protein has no beta-galactosidase catalytic activity, but plays functional roles in the formation of extracellular elastic fibers (elastogenesis) and in the development of connective tissue. Seems to be identical to the elastin-binding protein (EBP), a major component of the non- integrin cell surface receptor expressed on fibroblasts, smooth muscle cells, chondroblasts, leukocytes, and certain cancer cell types. In elastin producing cells, associates with tropoelastin intracellularly and functions as a recycling molecular chaperone which facilitates the secretions of tropoelastin and its assembly into elastic fibers.\| P16278 # Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans. SIZE: 546 amino acids; 60552 Da SUBCELLULAR LOCATION: Cytoplasm, perinuclear region. Note=Localized to the perinuclear area of the cytoplasm but not to lysosomes. DOMAIN: SwissProt: P16279 DISEASE: SwissProt: P16278 # Defects in GLB1 are the cause of GM1-gangliosidosis type I [MIM:230500]; also known as infantile GM1-gangliosidosis. This autosomal recessive disorder is characterized by the accumulation in visceral tissues, and ultimately excessive excretion in the urine, of beta-linked galactose-terminal oligosaccharides. Patients show central nervous system degeneration, and the coarse facial features, hepatosplenomegaly and skeletal dysmorphology reminiscent of Hurler syndrome. The infantile form is rapidly progressive leading to death usually between the first and second year. & Defects in GLB1 are the cause of GM1-gangliosidosis type II [MIM:230600]; also known as late infantile/juvenile type GM1- gangliosidosis. 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 III [MIM:230650]; also known as adult or chronic GM1- gangliosidosis. Patients show mild skeletal abnormalities, dysarthria, gait disturbance, dystonia and visual impairment. Visceromegaly is absent. Inheritance is autosomal recessive. & Defects in GLB1 are the cause of mucopolysaccharidosis IV B (MPS4B) [MIM:253010]; also known as Morquio syndrome B. MPS4B is a rare autosomal recessive disorder characterized by severe bone deformities without CNS involvement. SIMILARITY: SwissProt: P16279 ## Belongs to the glycosyl hydrolase 35 family. \| P16278 ## Belongs to the glycosyl hydrolase 35 family.

Biological Information

Immunogen

Beta galactosidase from E-coli.

Epitope

bacterial

Host

Rabbit

Specificity

Specific for Beta galactosidase from E coli by IEP. May cross react with Beta galactosidase from other species.

Species Reactivity

Vertebrates
E. coli

Antibody Type

Polyclonal Antibody

Entrez Gene Number

Entrez Gene Summary

The GLB1 gene encodes beta-galactosidase-1 (EC 3.2.1.23), a lysosomal hydrolase that cleaves the terminal beta-galactose from ganglioside substrates and other glycoconjugates (Yoshida et al., 1991 [PubMed 1907800]). Beta-galactosidase also occurs in a complex with neuraminidase (NEU1; MIM 608272) and protective protein/cathepsin A (PPCA; MIM 256540), which is a component of certain cell surface receptors (Hinek, 1996 [PubMed 8922281]). See also galactosylceramidase (GALC; MIM 606890) (EC 3.2.1.46), a genetically distinct beta-galactosidase that is involved in the catabolism of other lipid compounds.[supplied by OMIM]

Gene Symbol

GLB1
Lactase
S-Gal
ELNR1
EBP
EC 3.2.1.23

UniProt Number

P16279

UniProt Summary

FUNCTION: SwissProt: P16279 # This protein has no beta-galactosidase catalytic activity, but plays functional roles in the formation of extracellular elastic fibers (elastogenesis) and in the development of connective tissue. Seems to be identical to the elastin-binding protein (EBP), a major component of the non- integrin cell surface receptor expressed on fibroblasts, smooth muscle cells, chondroblasts, leukocytes, and certain cancer cell types. In elastin producing cells, associates with tropoelastin intracellularly and functions as a recycling molecular chaperone which facilitates the secretions of tropoelastin and its assembly into elastic fibers.| P16278 # Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans.
SIZE: 546 amino acids; 60552 Da
SUBCELLULAR LOCATION: Cytoplasm, perinuclear region. Note=Localized to the perinuclear area of the cytoplasm but not to lysosomes.
DOMAIN: SwissProt: P16279
DISEASE: SwissProt: P16278 # Defects in GLB1 are the cause of GM1-gangliosidosis type I [MIM:230500]; also known as infantile GM1-gangliosidosis. This autosomal recessive disorder is characterized by the accumulation in visceral tissues, and ultimately excessive excretion in the urine, of beta-linked galactose-terminal oligosaccharides. Patients show central nervous system degeneration, and the coarse facial features, hepatosplenomegaly and skeletal dysmorphology reminiscent of Hurler syndrome. The infantile form is rapidly progressive leading to death usually between the first and second year. & Defects in GLB1 are the cause of GM1-gangliosidosis type II [MIM:230600]; also known as late infantile/juvenile type GM1- gangliosidosis. 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 III [MIM:230650]; also known as adult or chronic GM1- gangliosidosis. Patients show mild skeletal abnormalities, dysarthria, gait disturbance, dystonia and visual impairment. Visceromegaly is absent. Inheritance is autosomal recessive. & Defects in GLB1 are the cause of mucopolysaccharidosis IV B (MPS4B) [MIM:253010]; also known as Morquio syndrome B. MPS4B is a rare autosomal recessive disorder characterized by severe bone deformities without CNS involvement.
SIMILARITY: SwissProt: P16279 ## Belongs to the glycosyl hydrolase 35 family. | P16278 ## Belongs to the glycosyl hydrolase 35 family.

Product Usage Statements
Usage Statement	Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Product Usage Statements

Usage Statement

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

Storage and Shipping Information
Storage Conditions	Maintain lyophilized material at 2-8°C for up to 12 months. After reconstitution maintain in undiluted aliquots at -20°C for up to 6 months. Avoid repeated freeze/thaw cycles.

Storage and Shipping Information

Storage Conditions

Maintain lyophilized material at 2-8°C for up to 12 months. After reconstitution maintain in undiluted aliquots at -20°C for up to 6 months. Avoid repeated freeze/thaw cycles.

Packaging Information
Material Size	2 mL

Packaging Information

Material Size

2 mL

实验耗材

分子生物学试剂

蛋白与免疫学

生化试剂

细胞及检测试剂

色谱及层析

仪器设备

实验服务

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