Involvement in diseaseDefects in FBP1 are the cause of fructose-1,6-bisphosphatase deficiency (FBPD) [MIM:229700]. FBPD is inherited as an autosomal recessive disorder mainly in the liver and causes life-threatening episodes of hypoglycemia and metabolic acidosis (lactacidemia) in newborn infants or young children.
Sequence similaritiesBelongs to the FBPase class 1 family.
ICC/IF image of ab109732 stained HeLa cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody (ab109732, 10µg/ml) overnight at +4°C. The secondary antibody (green) was ab96899, DyLight® 488 goat anti-rabbit IgG (H+L) used at a 1/250 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.
Western blot - FBP1 antibody [EPR4620] (ab109732)
All lanes : Anti-FBP1 antibody [EPR4620] (ab109732) at 1/1000 dilution
Lane 1 : Human stomach lysate Lane 2 : Human fetal liver lysate Lane 3 : MCF7 lysate
Lysates/proteins at 10 µg per lane.
Predicted band size : 37 kDa
References for Anti-FBP1 antibody [EPR4620] (ab109732)
This product has been referenced in:
Li N et al. Clinical and Molecular Characterization of Patients with Fructose 1,6-Bisphosphatase Deficiency. Int J Mol Sci18:N/A (2017).
Read more (PubMed: 28420223) »
Du Y et al. Lysine malonylation is elevated in type 2 diabetic mouse models and enriched in metabolic associated proteins. Mol Cell Proteomics14:227-36 (2015).
Read more (PubMed: 25418362) »