Western Blot (WB)
(Western Blot detection against Immunogen (35.64kD).)
Mouse anti-Human GBA Monoclonal Antibody | anti-GBA antibody
GBA (Glucosylceramidase, Acid beta-glucosidase, Alglucerase, Beta-glucocerebrosidase, D-glucosyl-N-acylsphingosine Glucohydrolase, Imiglucerase, GC, GLUC) (HRP)
Applications are based on unconjugated antibody.
Western Blot (WB)
(Western Blot detection against Immunogen (35.64kD).)
Western Blot (WB)
(Western Blot detection against Immunogen (35.64kD).)
Western Blot (WB)
(GBA monoclonal antibody, Western Blot analysis of GBA expression in MCF-7.)
Western Blot (WB)
(GBA monoclonal antibody, Western Blot analysis of GBA expression in MCF-7.)
Western Blot (WB)
(Western Blot analysis of GBA expression in transfected 293T cell line by GBA monoclonal antibody. Lane 1: GBA transfected lysate (60kD). Lane 2: Non-transfected lysate.)
Western Blot (WB)
(Western Blot analysis of GBA expression in transfected 293T cell line by GBA monoclonal antibody. Lane 1: GBA transfected lysate (60kD). Lane 2: Non-transfected lysate.)
Immunohistochemistry (IHC)
(Immunoperoxidase of monoclonal antibody to GBA on formalin-fixed paraffin-embedded human breast cancer. [antibody concentration 3ug/ml].)
Immunohistochemistry (IHC)
(Immunoperoxidase of monoclonal antibody to GBA on formalin-fixed paraffin-embedded human breast cancer. [antibody concentration 3ug/ml].)
Immunofluorescence (IF)
(Immunofluorescence of monoclonal antibody to GBA on HeLa cell. [antibody concentration 10ug/ml].)
Immunofluorescence (IF)
(Immunofluorescence of monoclonal antibody to GBA on HeLa cell. [antibody concentration 10ug/ml].)
Testing Data
(Detection limit for recombinant GST tagged GBA is ~1ng/ml as a capture antibody.)
Testing Data
(Detection limit for recombinant GST tagged GBA is ~1ng/ml as a capture antibody.)
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a lysosomal membrane protein that cleaves the beta-glucosidic linkage of glycosylceramide, an intermediate in glycolipid metabolism. Mutations in this gene cause Gaucher disease, a lysosomal storage disease characterized by an accumulation of glucocerebrosides. A related pseudogene is approximately 12 kb downstream of this gene on chromosome 1. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2010]
Uniprot Description
GBA: Defects in GBA are the cause of Gaucher disease (GD); also known as glucocerebrosidase deficiency. GD is the most prevalent lysosomal storage disease, characterized by accumulation of glucosylceramide in the reticulo-endothelial system. Different clinical forms are recognized depending on the presence (neuronopathic forms) or absence of central nervous system involvement, severity and age of onset. Defects in GBA are the cause of Gaucher disease type 1 (GD1); also known as adult non-neuronopathic Gaucher disease. GD1 is characterized by hepatosplenomegaly with consequent anemia and thrombopenia, and bone involvement. The central nervous system is not involved. Defects in GBA are the cause of Gaucher disease type 2 (GD2); also known as acute neuronopathic Gaucher disease. GD2 is the most severe form and is universally progressive and fatal. It manifests soon after birth, with death generally occurring before patients reach two years of age. Defects in GBA are the cause of Gaucher disease type 3 (GD3); also known as subacute neuronopathic Gaucher disease. GD3 has central nervous manifestations. Defects in GBA are the cause of Gaucher disease type 3C (GD3C); also known as pseudo-Gaucher disease or Gaucher-like disease. Defects in GBA are the cause of Gaucher disease perinatal lethal (GDPL). It is a distinct form of Gaucher disease type 2, characterized by fetal onset. Hydrops fetalis, in utero fetal death and neonatal distress are prominent features. When hydrops is absent, neurologic involvement begins in the first week and leads to death within 3 months. Hepatosplenomegaly is a major sign, and is associated with ichthyosis, arthrogryposis, and facial dysmorphism. Perinatal lethal Gaucher disease is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders. Defects in GBA contribute to susceptibility to Parkinson disease (PARK). A complex neurodegenerative disorder characterized by bradykinesia, resting tremor, muscular rigidity and postural instability. Additional features are characteristic postural abnormalities, dysautonomia, dystonic cramps, and dementia. The pathology of Parkinson disease involves the loss of dopaminergic neurons in the substantia nigra and the presence of Lewy bodies (intraneuronal accumulations of aggregated proteins), in surviving neurons in various areas of the brain. The disease is progressive and usually manifests after the age of 50 years, although early-onset cases (before 50 years) are known. The majority of the cases are sporadic suggesting a multifactorial etiology based on environmental and genetic factors. However, some patients present with a positive family history for the disease. Familial forms of the disease usually begin at earlier ages and are associated with atypical clinical features. Belongs to the glycosyl hydrolase 30 family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 3.2.1.45; Glycan Metabolism - other glycan degradation; Hydrolase; Lipid Metabolism - sphingolipid
Chromosomal Location of Human Ortholog: 1q21
Cellular Component: lysosomal lumen; lysosomal membrane
Molecular Function: glucosylceramidase activity; protein binding; receptor binding
Biological Process: ceramide biosynthetic process; glucosylceramide catabolic process; glycosphingolipid metabolic process; mitochondrion organization and biogenesis; negative regulation of inflammatory response; negative regulation of interleukin-6 production; negative regulation of MAP kinase activity; negative regulation of protein homooligomerization; neurite development; positive regulation of protein amino acid dephosphorylation; positive regulation of protein complex disassembly; positive regulation of protein metabolic process; regulation of cellular protein metabolic process; regulation of macroautophagy; sphingosine biosynthetic process
Disease: Dementia, Lewy Body; Gaucher Disease, Perinatal Lethal; Gaucher Disease, Type I; Gaucher Disease, Type Ii; Gaucher Disease, Type Iii; Gaucher Disease, Type Iiic; Parkinson Disease, Late-onset
Research Articles on GBA
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Product Notes
The GBA gba (Catalog #AAA6152642) is an Antibody produced from Mouse and is intended for research purposes only. The product is available for immediate purchase. The GBA (Glucosylceramidase, Acid beta-glucosidase, Alglucerase, Beta-glucocerebrosidase, D-glucosyl-N-acylsphingosine Glucohydrolase, Imiglucerase, GC, GLUC) (HRP) reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's GBA can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Immunohistochemistry (IHC) Paraffin, Western Blot (WB). IHC-P: 3ug/ml Applications are based on unconjugated antibody. Researchers should empirically determine the suitability of the GBA gba for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "GBA, Monoclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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