Rabbit Anti-GANAB Recombinant Antibody (EG1283) (V2LY-1225-LY643)

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Basic Information

Host Animal
Rabbit
Clone
EG1283
Application
WB, IP, IHC
Immunogen
Recombinant protein within human alpha Glucosidase 2 (aa 29-450).
Host Species
Rabbit
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
ApplicationNote
WB1:1,000-1:5,000
IHC-P1:1,000

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

Format
Liquid
Buffer
PBS, BSA, glycerol
Preservative
Sodium azide
Concentration
1 mg/ml
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
More Infomation

Target

Full Name
glucosidase II alpha subunit
Entrez Gene ID
Human23193
Mouse14376
UniProt ID
HumanQ14697
MouseQ8BHN3
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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