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GeneBio Systems

Anti-PGAP1 Antibody Picoband®

Anti-PGAP1 Antibody Picoband®

SKU:A08640

Regular price ¥94,200 JPY
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Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human

Applications: WB,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x106 cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: PGAP1

Specificity:

Background: Post-GPI attachment to proteins 1 is a protein that in humans is encoded by the PGAP1 gene. The protein encoded by this gene functions early in the glycosylphosphatidylinositol (GPI) biosynthetic pathway, catalyzing the inositol deacylation of GPI. The encoded protein is required for the production of GPI that can attach to proteins, and this may be an important factor in the transport of GPI-anchored proteins from the endoplasmic reticulum to the Golgi. Defects in this gene are a cause an autosomal recessive form of cognitive impairment.

Immunogen: E.coli-derived human PGAP1 recombinant protein (Position: K166-R904). Human PGAP1 shares 89% and 88.5% amino acid (aa) sequence identity with mouse and rat PGAP1, respectively.

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Bosch, D. G. M., Boonstra, F. N., Kinoshita, T., Jhangiani, S., de Ligt, J., Cremers, F. P. M., Lupski, J. R., Murakami, Y., de Vries, B. B. A. Cerebral visual impairment and intellectual disability caused by PGAP1 variants. Europ. J. Hum. Genet. 23: 1689-1693, 2015. 2. Granzow, M., Paramasivam, N., Hinderhofer, K., Fischer, C., Chotewutmontri, S., Kaufmann, L., Evers, C., Kotzaeridou, U., Rohrschneider, K., Schlesner, M., Sturm, M., Pinkert, S., Eils, R., Bartram, C. R., Bauer, P., Moog, U. Loss of function of PGAP1 as a cause of severe encephalopathy identified by whole exome sequencing: lessons of the bioinformatics pipeline. Molec. Cell. Probes 29: 323-329, 2015. 3. Murakami, Y., Tawamie, H., Maeda, Y., Buttner, C., Buchert, R., Radwan, F., Schaffer, S., Sticht, H., Aigner, M., Reis, A., Kinoshita, T., Jamra, R. A. Null mutation in PGAP1 impairing Gpi-anchor maturation in patients with intellectual disability and encephalopathy. PLoS Genet. 10: e1004320, 2014. Note: Electronic Article.

Uniprot ID: Q75T13

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross reactivity with other proteins.

Isotype: IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 100 kDa, 58 kDa

Calculated Molecular Weight: 52588 MW

Gene ID: 80055

Protein Name: GPI inositol-deacylase

Gene Full Name: post-GPI attachment to proteins inositol deacylase 1

Synonyms: Bst1; GPI inositol deacylase; hPGAP1; ISPD3024; PGAP1

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