Anti-FBP1 Antibody Picoband® Fluoro647 Conjugated

Fructose-1,6-bisphosphatase 1 is a protein that in humans is encoded by the FBP1 gene. Fructose-1,6-bisphosphatase 1, a gluconeogenesis regulatory enzyme, catalyzes the hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate and inorganic phosphate. Fructose-1,6-diphosphatase deficiency is associated with hypoglycemia and metabolic acidosis.

Error-prone DNA polymerase specifically involved in DNA repair. Plays an important role in translesion synthesis, where the normal high-fidelity DNA polymerases cannot proceed and DNA synthesis stalls. Favors Hoogsteen base-pairing in the active site. Inserts the correct base with high-fidelity opposite an adenosine template. Exhibits low fidelity and efficiency opposite a thymidine template, where it will preferentially insert guanosine. May play a role in hypermutation of immunogobulin genes. Forms a Schiff base with 5'-deoxyribose phosphate at abasic sites, but may not have lyase activity.

1. El-Maghrabi, M. R., Lange, A. J., Jiang, W., Yamagata, K., Stoffel, M., Takeda, J., Fernald, A. A., Le Beau, M. M., Bell, G. I., Baker, L., Pilkis, S. J. Human fructose-1,6-bisphosphatase gene (FBP1) : exon-intron organization, localization to chromosome bands 9q22.2-q22.3, and mutation screening in subjects with fructose-1,6-bisphosphatase deficiency. Genomics 27: 520-525, 1995. 2. Kikawa, Y., Inuzuka, M., Jin, B. Y., Kaji, S., Koga, J., Yamamoto, Y., Fujisawa, K., Hata, I., Nakai, A., Shigematsu, Y., Mizunuma, H., Taketo, A., Mayumi, M., Sudo, M. Identification of genetic mutations in Japanese patients with fructose-1,6-bisphosphatase deficiency. Am. J. Hum. Genet. 61: 852-861, 1997. 3. Kikawa, Y., Inuzuka, M., Jin, B. Y., Kaji, S., Yamamoto, Y., Shigematsu, Y., Nakai, A., Taketo, A., Ohura, T., Mikami, H., Mizunuma, H., Suzuki, Y., Narisawa, K., Sudo, M. Identification of a genetic mutation in a family with fructose-1,6-bisphosphatase deficiency. Biochem. Biophys. Res. Commun. 210: 797-804, 1995.