Cell Biology Antibodies 7
Anti-GLB1 Antibody (CAB1863)
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- SKU:
- CAB1863
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Research Area:
- Cell Biology
Description
| Antibody Name: | Anti-GLB1 Antibody |
| Antibody SKU: | CAB1863 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB IF |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-270 of human GLB1 (NP_001073279.1). |
| Application: | WB IF |
| Recommended Dilution: | WB 1:500 - 1:2000 IF 1:50 - 1:200 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | A549, HeLa, Mouse kidney |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 1-270 of human GLB1 (NP_001073279.1). |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | MFEI DYSR DSFL KDGQ PFRY ISGS IHYS RVPR FYWK DRLL KMKM AGLN AIQT YVPW NFHE PWPG QYQF SEDH DVEY FLRL AHEL GLLV ILRP GPYI CAEW EMGG LPAW LLEK ESIL LRSS DPDY LAAV DKWL GVLL PKMK PLLY QNGG PVIT VQVE NEYG SYFA CDFD YLRF LQKR FRHH LGDD VVLF TTDG AHKT FLKC GALQ GLYT TVDF GTGS NITD AFLS QRKC EPKG PLIN SEFY TGWL DHWG QPHS TIKT EAVA SSLY DILA RG |
| Gene ID: | 2720 |
| Uniprot: | P16278 |
| Cellular Location: | Cytoplasm, Lysosome, perinuclear region |
| Calculated MW: | 60kDa/72kDa/76kDa |
| Observed MW: | 64kDa |
| Synonyms: | GLB1, EBP, ELNR1, MPS4B |
| Background: | This gene encodes a member of the glycosyl hydrolase 35 family of proteins. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed to generate the mature lysosomal enzyme. This enzyme catalyzes the hydrolysis of a terminal beta-linked galactose residue from ganglioside substrates and other glycoconjugates. Mutations in this gene may result in GM1-gangliosidosis and Morquio B syndrome. |
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | Protein type:Lipid Metabolism - sphingolipid; EC 3.2.1.23; Glycan Metabolism - glycosaminoglycan degradation; Hydrolase; Carbohydrate Metabolism - galactose; Glycan Metabolism - other glycan degradation; Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series Chromosomal Location of Human Ortholog: 3p21.33 Cellular Component: cytoplasm; Golgi apparatus; intracellular membrane-bound organelle; lysosomal lumen; vacuole Molecular Function:beta-galactosidase activity; exo-alpha-sialidase activity; protein binding Biological Process: cellular carbohydrate metabolic process; glycosaminoglycan catabolic process; glycosphingolipid metabolic process; keratan sulfate catabolic process Disease: Gm1-gangliosidosis, Type I; Gm1-gangliosidosis, Type Ii; Gm1-gangliosidosis, Type Iii; Mucopolysaccharidosis Type Ivb |
| NCBI Summary: | This gene encodes a member of the glycosyl hydrolase 35 family of proteins. Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed to generate the mature lysosomal enzyme. This enzyme catalyzes the hydrolysis of a terminal beta-linked galactose residue from ganglioside substrates and other glycoconjugates. Mutations in this gene may result in GM1-gangliosidosis and Morquio B syndrome. [provided by RefSeq, Nov 2015] |
| UniProt Code: | P16278 |
| NCBI GenInfo Identifier: | 215273939 |
| NCBI Gene ID: | 2720 |
| NCBI Accession: | P16278.2 |
| UniProt Secondary Accession: | P16278,P16279, B2R7H8, B7Z6B0, |
| UniProt Related Accession: | P16278 |
| Molecular Weight: | 72,751 Da |
| NCBI Full Name: | Beta-galactosidase |
| NCBI Synonym Full Names: | galactosidase beta 1 |
| NCBI Official Symbol: | GLB1 |
| NCBI Official Synonym Symbols: | EBP; ELNR1; MPS4B |
| NCBI Protein Information: | beta-galactosidase |
| UniProt Protein Name: | Beta-galactosidase |
| UniProt Synonym Protein Names: | Acid beta-galactosidase; Lactase; Elastin receptor 1 |
| Protein Family: | Globulin |
| UniProt Gene Name: | GLB1 |
| UniProt Entry Name: | BGAL_HUMAN |
 | Western blot analysis of extracts of various cell lines, using GLB1 antibody (CAB1863) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 30s. |
 | Immunofluorescence analysis of L929 cells using GLB1 antibody (CAB1863) at dilution of 1:100. Blue: DAPI for nuclear staining. |
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