Anti-GBA Rabbit pAb
- 100ug (NP20973) Please inquiry
Antibody type:Primary antibody
Label:Unconjugated
Modification:Unmodification
Isotype:Rabbit IgG
Host:Rabbit
Application:WB,IHC
Purify method:Affinity Chromatography
Species:Human,Rat
Gene Name:
Synonyms:Alglucerase; betaGC; GBA1; GCase; GCB; GLUC; Glucosylceramidase; Imiglucerase;
Gene Synonyms:
Gene Full Name:
Gene Infomation:Defects in GBA are the cause of Gaucher disease (GD) [MIM:230800]; 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.
Antigen:A synthesized peptide derived from human GBA
Antigen Synonyms:
Clonality:Polyclonal antibody
Source:
Reaction:GBA Antibody detects endogenous levels of total GBA
Form:Liquid
Tested Applications:
- Western blot (1:100 to 1:500)
- Immunofluorescence (1:50 to 1:400)
- Immunohistochemistry (1:200 to 1:500)
- Flow cytometry analysis (1:200 to 1:500)
- Enzyme-linked Immunosorbent Assay (1:100-1:5000)
Clone:
Dilution:WB 1:500~1:2000 IHC 1:50~1:200
Mole Mass:60kDa
Location:Lysosome membrane > Peripheral membrane protein > Lumenal side;
Concentration:
Sequence Similarity:Belongs to the glycosyl hydrolase 30 family.
Gene Id:
SwissProt ID:P04062
Unigene:
Nucleotide Accession:
Tissue specificity:GBA Antibody detects endogenous levels of total GBA
Storage:Store at +4°C short term. Store at -20°C long term. Avoid freeze / thaw cycle.
Buffer condition:Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Background:Defects in GBA are the cause of Gaucher disease (GD) [MIM:230800]; also known as glucocerebrosidase deficiency. GD is the most prevalent lysosomal storage disease, characterized by accumulation of glucosylceramide in the reticulo-endothelial system.
Molar Function:
Western Blot:Stability: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.
Storage: Store at 4°C for frequent use. Stored at -20°C in a manual defrost freezer for one year without detectable loss of activity. Avoid repeated freeze-thaw cycles.
Notes: For In vitro laboratory use only. Not for any clinical, therapeutic, or diagnostic use in humans or animals. Not for animal or human consumption.