GLB1 / Beta-galactosidase Recombinant Rabbit Monoclonal Antibody [JE40-77]

About this product

Cleaves beta-linked terminal galactosyl residues from gangliosides, glycoproteins, and glycosaminoglycans. Isoform 2 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. Defects in GLB1 are the cause of mucopolysaccharidosis type 4B (MPS4B) [MIM:253010]; 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.

Specifications

Catalog number

HA721562

Size / count

100 µL

Dry ice

No

Hazardous

No

Discontinued

No

CAS Number

9000-83-3

Product Name Alternative

Acid beta galactosidase antibody Acid beta-galactosidase antibody Beta galactosidase 1 antibody Beta galactosidase antibody Beta-galactosidase antibody BGAL_HUMAN antibody EBP antibody EBP, included antibody Elastin receptor 1 (67kD) antibody Elastin receptor 1 67kDa antibody Elastin receptor 1 antibody Elastin receptor 1, included antibody Elastin-binding protein, included antibody ELNR1 antibody Galactosidase beta 1 antibody GLB 1 antibody GLB1 antibody Lactase antibody MPS4B antibody S-GAL, included antibody

Abbreviation

Beta-galactosidase Acid beta-galactosidase (Lactase) Elastin receptor 1 GLB1 ELNR1

Swiss Prot

P16278 Human, P23780 Mouse,

Cellular Locus

Lysosome and Cytoplasm.

Host

Rabbit

Species Reactivity

Human, Mouse, Rat

Immunogen

Synthetic peptide within Human GLB1 aa 628-677 / 677.

Isotype

IgG

Clone

JE40-77

Conjugation

Non-conjugated

Type

Recombinant Rabbit monoclonal Antibody

Applications

WB, FC, IHC-P

Positive Control

SK-Br-3 cell lysate, Saos-2 cell lysate, U-87 MG cell lysate, MCF7 cell lysate, HepG2 cell lysate, K-562 cell lysate, Daudi cell lysate, NIH/3T3 cell lysate, Rat brain tissue lysate, mouse placenta tissue, SK-Br-3.

Concentration

1 mg/mL

Dilution

WB: 1:1,000 ;FC: 1:500-1:1,000 ;IHC-P: 1:1,000

Purity

Protein A affinity purified.

Form

Liquid

Buffer

1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.

Molecular Weight

Predicted band size: 76 kDa

Storage Conditions

Store at +4°C after thawing. Aliquot store at -20°C. Avoid repeated freeze/thaw cycles.

Recombinant Antibody

Yes