Anti-PMM2 Antibody Picoband®

SKU: A03146-1

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,Mouse,Rat

Applications: WB,ICC,IF,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: PMM2

Specificity:

Background: Phosphomannomutase 2 is an enzyme that in humans is encoded by the PMM2 gene. The protein encoded by this gene catalyzes the isomerization of mannose 6-phosphate to mannose 1-phosphate, which is a precursor to GDP-mannose necessary for the synthesis of dolichol-P-oligosaccharides. Mutations in this gene have been shown to cause defects in glycoprotein biosynthesis, which manifests as carbohydrate-deficient glycoprotein syndrome type I.

Immunogen: E.coli-derived human PMM2 recombinant protein (Position: D48-S246). Human PMM2 shares 92.4% amino acid (aa) sequence identity with mouse PMM2.

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. Bjursell, C., Erlandson, A., Nordling, M., Nilsson, S., Wahlstrom, J., Stibler, H., Kristiansson, B., Martinsson, T. PMM2 mutation spectrum, including 10 novel mutations, in a large CDG type 1A family material with a focus on Scandinavian families. Hum. Mutat. 16: 395-400, 2000. 2. Bjursell, C., Wahlstrom, J., Berg, K., Stibler, H., Kristiansson, B., Matthijs, G., Martinsson, T. Detailed mapping of the phosphomannomutase 2 (PMM2) gene and mutation detection enable improved analysis for Scandinavian CDG type I families. Europ. J. Hum. Genet. 6: 603-611, 1998. 3. Bohles, H., Sewell, A. C., Gebhardt, B., Reinecke-Luthge, A., Kloppel, G., Marquardt, T. Hyperinsulinaemic hypoglycaemia: leading symptom in a patient with congenital disorder of glycosylation Ia (phosphomannomutase deficiency). J. Inherit. Metab. Dis. 24: 858-862, 2001.

Uniprot ID: O15305

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: 28 kDa

Calculated Molecular Weight: 52588 MW

Gene ID: 5373

Protein Name: Phosphomannomutase 2

Gene Full Name: phosphomannomutase 2

Synonyms: PMM2; Phosphomannomutase 2; PMM 2