2wbd

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Template:STRUCTURE 2wbd

Contents 1 FRUCTOSE-1,6-BISPHOSPHATASE(D-FRUCTOSE-1,6-BISPHOSPHATE-1-PHOSPHOHYDROLASE) (E.C.3.1.3.11) COMPLEXED WITH AN AMP SITE INHIBITOR 2 Disease 3 About this Structure 4 See Also 5 Reference

FRUCTOSE-1,6-BISPHOSPHATASE(D-FRUCTOSE-1,6-BISPHOSPHATE-1-PHOSPHOHYDROLASE) (E.C.3.1.3.11) COMPLEXED WITH AN AMP SITE INHIBITOR

Template:ABSTRACT PUBMED 19969452

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

2wbd is a 8 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

See Also

• Fructose-1%2C6-bisphosphatase

Reference

• Kitas E, Mohr P, Kuhn B, Hebeisen P, Wessel HP, Haap W, Ruf A, Benz J, Joseph C, Huber W, Sanchez RA, Paehler A, Benardeau A, Gubler M, Schott B, Tozzo E. Sulfonylureido thiazoles as fructose-1,6-bisphosphatase inhibitors for the treatment of type-2 diabetes. Bioorg Med Chem Lett. 2010 Jan 15;20(2):594-9. Epub 2009 Nov 22. PMID:19969452 doi:10.1016/j.bmcl.2009.11.093

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