Rabbit Anti-Glucosidase 2 subunit beta/FITC Conjugated antibody

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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; Prot

Cat:

SL13389R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Pig,Cow,Horse,)

Immunogen：

KLH conjugated synthetic peptide derived from human Glucosidase 2 subunit beta

Format：

Lyophilized or Liquid

Storage instructions：

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 ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

58kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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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 SLCterminus. 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 SLCterminus, 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

Entrez Gene: 300445 Rat

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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