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Rabbit Anti-GANAB Recombinant Antibody (EG1283) (CBMAB-EN1547-LY)

The product is antibody recognizes GANAB. The antibody EG1283 immunoassay techniques such as: WB: 1:500~1:1000 ELISA: 1:5000.
See all GANAB antibodies

Summary

Host Animal
Rabbit
Specificity
Human, Mouse
Clone
EG1283
Antibody Isotype
IgG
Application
WB: 1:500~1:1000 ELISA: 1:5000

Basic Information

Immunogen
The antibody was produced against synthesized peptide derived from internal of human GANAB.
Specificity
Human, Mouse
Antibody Isotype
IgG
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
glucosidase II alpha subunit
Introduction
This gene encodes the alpha subunit of glucosidase II and a member of the glycosyl hydrolase 31 family of proteins. The heterodimeric enzyme glucosidase II plays a role in protein folding and quality control by cleaving glucose residues from immature glycoproteins in the endoplasmic reticulum. Expression of the encoded protein is elevated in lung tumor tissue and in response to UV irradiation. Mutations in this gene cause autosomal-dominant polycystic kidney and liver disease.
Entrez Gene ID
Human23193
Mouse14376
UniProt ID
HumanQ14697
MouseQ8BHN3
Alternative Names
G2AN; GIIA; PKD3; GLUII
Function
Catalytic subunit of glucosidase II that cleaves sequentially the 2 innermost alpha-1,3-linked glucose residues from the Glc2Man9GlcNAc2 oligosaccharide precursor of immature glycoproteins (PubMed:10929008).

Required for PKD1/Polycystin-1 and PKD2/Polycystin-2 maturation and localization to the cell surface and cilia (PubMed:27259053).
Biological Process
Carbohydrate metabolic process Source: InterPro
N-glycan processing Source: UniProtKB
Cellular Location
Endoplasmic reticulum; Golgi apparatus; Melanosome. Identified by mass spectrometry in melanosome fractions from stage I to stage IV.
Involvement in disease
Polycystic kidney disease 3 with or without polycystic liver disease (PKD3):
A form of polycystic kidney disease, a disorder characterized by progressive formation and enlargement of cysts in both kidneys, typically leading to end-stage renal disease in adult life. Cysts also occur in other organs, particularly the liver. PKD3 inheritance is autosomal dominant. GANAB variations may act as a disease modifier in autosomal dominant polycystic liver disease in patients who have causative mutations in other genes, such as PKHD1 or ALG8.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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