GLB1 / Beta-galactosidase Recombinant Rabbit Monoclonal Antibody [JE40-77]
Catalog# HA721562
GLB1 / Beta-galactosidase Recombinant Rabbit Monoclonal Antibody [JE40-77]
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WB
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FC
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IHC-P
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Human
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Mouse
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Rat
概述
产品名称
GLB1 / Beta-galactosidase Recombinant Rabbit Monoclonal Antibody [JE40-77]
抗体类型
Recombinant Rabbit monoclonal Antibody
免疫原
Synthetic peptide within Human GLB1 aa 628-677 / 677.
种属反应性
Human, Mouse, Rat
验证应用
WB, FC, IHC-P
分子量
Predicted band size: 76 kDa
阳性对照
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.
偶联
unconjugated
克隆号
JE40-77
RRID
产品特性
形态
Liquid
浓度
1ug/ul
存放说明
Store at +4℃ after thawing. Aliquot store at -20℃. Avoid repeated freeze / thaw cycles.
存储缓冲液
1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
亚型
IgG
纯化方式
Protein A affinity purified.
应用稀释度
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WB
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1:1,000
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FC
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1:500-1:1,000
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IHC-P
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1:1,000
靶点
功能
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.
背景文献
1. Hinek A., Zhang S., Smith A.C., Callahan J.W. Impaired elastic-fiber assembly by fibroblasts from patients with either Morquio B disease or infantile GM1-gangliosidosis is linked to deficiency in the 67-kD spliced variant of beta-galactosidase. Am. J. Hum. Genet. 67:23-36 (2000).
2. Hinek A. Biological roles of the non-integrin elastin/laminin receptor. Biol. Chem. 377:471-480 (1996).
亚细胞定位
Lysosome and Cytoplasm.
别名
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
展开图片
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Western blot analysis of GLB1 / Beta-galactosidase on different lysates with Rabbit anti-GLB1 / Beta-galactosidase antibody (HA721562) at 1/1,000 dilution.
Lane 1: SK-Br-3 cell lysate (20 µg/Lane)
Lane 2: Saos-2 cell lysate (20 µg/Lane)
Lane 3: U-87 MG cell lysate (20 µg/Lane)
Lane 4: MCF7 cell lysate (20 µg/Lane)
Lane 5: HepG2 cell lysate (20 µg/Lane)
Lane 6: K-562 cell lysate (20 µg/Lane)
Lane 7: Daudi cell lysate (20 µg/Lane)
Lane 8: NIH/3T3 cell lysate (20 µg/Lane)
Lane 9: Rat brain tissue lysate (40 µg/Lane)
Predicted band size: 76 kDa
Observed band size: 76/100 kDa
Exposure time: 3 minutes; ECL: K1801;
4-20% SDS-PAGE gel.
Proteins were transferred to a PVDF membrane and blocked with 5% NFDM/TBST for 1 hour at room temperature. The primary antibody (HA721562) at 1/1,000 dilution was used in 5% NFDM/TBST at 4℃ overnight. Goat Anti-Rabbit IgG - HRP Secondary Antibody (HA1001) at 1:100,000 dilution was used for 1 hour at room temperature. -
Immunohistochemical analysis of paraffin-embedded mouse placenta tissue with Rabbit anti-GLB1 / Beta-galactosidase antibody (HA721562) at 1/1,000 dilution.
The section was pre-treated using heat mediated antigen retrieval with Tris-EDTA buffer (pH 9.0) for 20 minutes. The tissues were blocked in 1% BSA for 20 minutes at room temperature, washed with ddH2O and PBS, and then probed with the primary antibody (HA721562) at 1/1,000 dilution for 1 hour at room temperature. The detection was performed using an HRP conjugated compact polymer system. DAB was used as the chromogen. Tissues were counterstained with hematoxylin and mounted with DPX. -
Flow cytometric analysis of SK-Br-3 cells labeling GLB1 / Beta-galactosidase.
Cells were fixed and permeabilized. Then stained with the primary antibody (HA721562, 1ug/ml) (red) compared with Rabbit IgG Isotype Control (green). After incubation of the primary antibody at +4℃ for an hour, the cells were stained with a iFluor™ 488 conjugate-Goat anti-Rabbit IgG Secondary antibody (HA1121) at 1/1,000 dilution for 30 minutes at +4℃. Unlabelled sample was used as a control (cells without incubation with primary antibody; black).
请注意: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"