2y5k

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Template:STRUCTURE 2y5k

Contents 1 ORALLY ACTIVE AMINOPYRIDINES AS INHIBITORS OF TETRAMERIC FRUCTOSE 1,6-BISPHOSPHATASE 2 Disease 3 About this Structure 4 See Also 5 Reference

ORALLY ACTIVE AMINOPYRIDINES AS INHIBITORS OF TETRAMERIC FRUCTOSE 1,6-BISPHOSPHATASE

Template:ABSTRACT PUBMED 21550236

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]

About this Structure

2y5k is a 4 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

See Also

• Fructose-1%2C6-bisphosphatase

Reference

• 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

1. ↑ 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
2. ↑ 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