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Product Name Anti-Glucosidase 2 subunit beta/AF594 Chinese Name AF594标记的葡萄糖苷酶2β/Glucosidase IIβ抗体 Alias 80K-H protein; AGE-binding receptor 2; AGE-R2; G19P1; GLU2B_HUMAN; Glucosidase 2 subunit beta; Glucosidase II beta subunit; Glucosidase II subunit beta; Hepatocystin; PCLD; PKCSH; PLD1; PRKCSH; Protein kinase C substrate 60.1 kDa protein heavy chain; Protein kinase C substrate 80 Kda protein; Protein kinase C substrate 80K-H; Protein kinase C substrate, 80 Kda protein. Research Area Tumour Cell biology Signal transduction Kinases and Phosphatases Immunogen Species Rabbit Clonality Polyclonal React Species (predicted: Human, Mouse, Rat, Pig, Cow, Horse, ) Applications ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Molecular weight 58kDa Form Lyophilized or Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human Glucosidase 2 subunit beta Lsotype IgG Purification affinity purified by Protein A Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. Product Detail background:
Trimming of glucoses from N-linked core glycans on newly synthesized glycoproteins occurs sequentially through the action of Glucosidases I and II in the endoplasmic reticulum (ER). Glucosidase II is an ER-localized enzyme that contains a and b subunits (Glucosidase IIa and Glucosidase IIb) which form a defined heterodimeric complex. Glucosidase IIa is the catalyitc core of the enzyme and can function independently of the b subunit. The sequence of Glucosidase IIb encodes protein rich in glutamic and aspartic acid with a putative ER retention signal (HDEL) at the C-terminus. The phosphorylated form of Glucosidase IIb is localized in the plasma membrane and is highly expressed in FGF-stimulated fibroblasts and epidermal carcinoma cells. Glucosidase IIb was first purified from a human carcinoma cell line as a potential substrate for protein kinase C. Through the HDEL signal at the C-terminus, Glucosidase IIb retains the complete complex in the ER.
Function:
Regulatory subunit of glucosidase II.
Subunit:
Heterodimer of a catalytic alpha subunit (GANAB) and abeta subunit (PRKCSH). Binds glycosylated PTPRC (By similarity).
Subcellular Location:
Endoplasmic reticulum.
DISEASE:
Defects in PRKCSH are a cause of polycystic liver disease (PCLD) [MIM:174050]. PCLD is an autosomal dominant disorder and is characterized by the presence of multiple liver cysts of biliary epithelial origin. PCLD is a distinct clinical and genetic entity that can occur independently from autosomal dominant polycystic kidney disease (ADPKD) [MIM:173900], which in a considerable but uncertain proportion of cases is associated with hepatic cysts.
Similarity:
Contains 2 EF-hand domains.
Contains 1 PRKCSH domain.
Database links:Entrez Gene: 5589 Human
Entrez Gene: 19089 Mouse
Omim: 177060 Human
SwissProt: P14314 Human
SwissProt: O08795 Mouse
Unigene: 610830 Human
Unigene: 214593 Mouse
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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