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Recombinant Human GLB1 protein, His-tagged

Cat.No. : GLB1-7758H
Product Overview : Recombinant Human GLB1(Leu24-Val677) fused with His tag at C-terminal was expressed in HEK293.
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Description : β Galactosidase is a lysosomal β Galactosidase that hydrolyzes the terminal β Galactose from Ganglioside and Keratan sulfate. In lysosome, the mature β Galactosidase protein associates with Cathepsin A and Neuraminidase 1 to form the lysosomal multienzyme complex . An alternative splicing at the RNA level of β Galactosidase results a catalytically inactive β Galactosidase that plays an important role in vascular development. Defects of β-galactosidase (GLB1) are the cause of diseases like GM1-gangliosidosis which is a lysosomal storage disease and Morquio Syndrome B that cause patients to have abnormal elastic fibers. More than 100 mutations have been identified for β Galactosidase, which result in different residual activities of the mutant enzymes and a spectrum of symptoms in the two related diseases.
Source : HEK293
Species : Human
Tag : His
Form : Supplied as a 0.2 μm filtered solution of 20mM TrisHCl, 150mM NaCl, pH 8.0.
AA Sequence : LRNATQRMFEIDYSRDSFLKDGQPF RYISGSIHYSRVPRFYWKDRLLKMK MAGLNAIQTYVPWNF HEPWPGQYQFSEDHDVEYFLRLAHE LGLLVILRPGPYICAEWEMGGLPAW LLEKESILLRSSDPD YLAAVDKWLGVLLPKMKPLLYQNGG PVITVQVENEYGSYFACDFDYLRFL QKRFRHHLGDDVVLF TTDGAHKTFLKCGALQGLYTTVDFG TGSNITDAFLSQRKCEPKGPLINSE FYTGWLDHWGQPHST IKTEAVASSLYDILARGASVNLYMF IGGTNFAYWNGANSPYAAQPTSYDY DAPLSEAGDLTEKYF ALRNIIQKFEKVPEGPIPPSTPKFA YGKVTLEKLKTVGAALDILCPSGPI KSLYPLTFIQVKQHY GFVLYRTTLPQDCSNPAPLSSPLNG VHDRAYVAVDGIPQGVLERNNVITL NITGKAGATLDLLVE NMGRVNYGAYINDFKGLVSNLTLSS NILTDWTIFPLDTEDAVRSHLGGWG HRDSGHHDEAWAHNS SNYTLPAFYMGNFSIPSGIPDLPQD TFIQFPGWTKGQVWINGFNLGRYWP ARGPQLTLFVPQHIL MTSAPNTITVLELEWAPCSSDDPEL CAVTFVDRPVIGSSVTYDHPSKPVE KRLMPPPPQKNKDSW LDHVVDHHHHHH
Endotoxin : Less than 0.1 ng/µg (1 IEU/µg) as determined by LAL test.
Purity : Greater than 95% as determined by reducing SDS-PAGE.
Storage : Store at < -20 centigrade, stable for 6 months after receipt.
Please minimize freeze-thaw cycles.
Gene Name : GLB1 galactosidase, beta 1 [ Homo sapiens ]
Official Symbol : GLB1
Synonyms : GLB1; galactosidase, beta 1; elastin receptor 1 (67kD) , elastin receptor 1, 67kDa , ELNR1; beta-galactosidase; EBP; lactase; acid beta-galactosidase; elastin receptor 1, 67kDa; ELNR1; MPS4B;
Gene ID : 2720
mRNA Refseq : NM_000404
Protein Refseq : NP_000395
MIM : 611458
UniProt ID : P16278
Chromosome Location : 3p22.3
Pathway : Galactose metabolism, organism-specific biosystem; Galactose metabolism, conserved biosystem; Glycosaminoglycan degradation, organism-specific biosystem; Glycosaminoglycan degradation, conserved biosystem; Glycosphingolipid biosynthesis - ganglio series, organism-specific biosystem; Glycosphingolipid biosynthesis - ganglio series, conserved biosystem; Glycosphingolipid metabolism, organism-specific biosystem;
Function : beta-galactosidase activity; cation binding; galactoside binding; hydrolase activity, hydrolyzing O-glycosyl compounds; protein binding;

For Research Use Only. Not intended for any clinical use. No products from Creative BioMart may be resold, modified for resale or used to manufacture commercial products without prior written approval from Creative BioMart.

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How does GLB1 protein deficiency impact the nervous system? 07/04/2022

GLB1 deficiency can lead to neurodegeneration, causing progressive deterioration of the nervous system.

What are the different subtypes of GLB1 deficiency? 02/15/2020

There are three subtypes: GM1 gangliosidosis, mucopolysaccharidosis IVB, and combined GM1 gangliosidosis and mucopolysaccharidosis IVB.

Can GLB1 deficiency be managed through dietary interventions? 12/11/2019

While dietary interventions may help manage certain symptoms, they do not address the underlying cause of the deficiency.

What are the symptoms of GLB1 deficiency? 08/29/2018

Symptoms may include developmental delay, skeletal abnormalities, vision and hearing loss, and neurological deterioration.

Can GLB1 deficiency be detected prenatally? 05/21/2016

Yes, prenatal testing through genetic analysis of amniotic fluid or chorionic villus sampling can detect GLB1 deficiency.

Customer Reviews (3)

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12/10/2021

    Their support has been invaluable in troubleshooting and providing efficient solutions, enhancing the overall efficiency and productivity of my experiments.

    04/03/2019

      Its versatility allows for exploring its various roles and functions, shedding light on its significance in crucial biochemical pathways and cellular processes.

      05/16/2016

        Their knowledgeable and responsive team has consistently demonstrated their ability to address any concerns or challenges I have encountered along the way.

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