| Structural highlights
Disease
F16P1_HUMAN Defects 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.[1] [2]
Function
F16P1_HUMAN
Publication Abstract from PubMed
A novel sulfonylureido pyridine series exemplified by compound 19 yielded potent inhibitors of FBPase showing significant glucose reduction and modest glycogen lowering in the acute db/db mouse model for Type-2 diabetes. Our inhibitors occupy the allosteric binding site and also extend into the dyad interface region of tetrameric FBPase.
Orally active aminopyridines as inhibitors of tetrameric fructose-1,6-bisphosphatase.,Hebeisen P, Haap W, Kuhn B, Mohr P, Wessel HP, Zutter U, Kirchner S, Ruf A, Benz J, Joseph C, Alvarez-Sanchez R, Gubler M, Schott B, Benardeau A, Tozzo E, Kitas E Bioorg Med Chem Lett. 2011 Jun 1;21(11):3237-42. Epub 2011 Apr 20. PMID:21550236[3]
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.
See Also
References
- ↑ Kikawa Y, Inuzuka M, Jin BY, 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. 1997 Oct;61(4):852-61. PMID:9382095
- ↑ Matsuura T, Chinen Y, Arashiro R, Katsuren K, Tamura T, Hyakuna N, Ohta T. Two newly identified genomic mutations in a Japanese female patient with fructose-1,6-bisphosphatase (FBPase) deficiency. Mol Genet Metab. 2002 Jul;76(3):207-10. PMID:12126934
- ↑ Hebeisen P, Haap W, Kuhn B, Mohr P, Wessel HP, Zutter U, Kirchner S, Ruf A, Benz J, Joseph C, Alvarez-Sanchez R, Gubler M, Schott B, Benardeau A, Tozzo E, Kitas E. Orally active aminopyridines as inhibitors of tetrameric fructose-1,6-bisphosphatase. Bioorg Med Chem Lett. 2011 Jun 1;21(11):3237-42. Epub 2011 Apr 20. PMID:21550236 doi:10.1016/j.bmcl.2011.04.044
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