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This Sandbox is Reserved from January 10, 2010, through April 10, 2011 for use in BCMB 307-Proteins course taught by Andrea Gorrell at the University of Northern British Columbia, Prince George, BC, Canada.

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Introduction

spinqualitylabelsall models PDB ID 1m7x Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image
1m7x, resolution 2.30Å ()
Activity:	1,4-alpha-glucan branching enzyme, with EC number 2.4.1.18
Structural annotation: Resources: CATH : 1M7Xa02, 1M7Xa01, 1M7Xa03, 1M7Xb03, 1M7Xb02, 1M7Xb01, 1M7Xc02, 1M7Xc03, 1M7Xc01, 1M7Xd02, 1M7Xd01, 1M7Xd03 InterPro : Ipr006047, Ipr004193, Ipr006407, Ipr013780, Ipr013783, Ipr013781, Ipr014756, Ipr006048, Ipr013782 Pfam : PF02922, PF00128, PF02806 SCOP : d1m7xa2, d1m7xa1, d1m7xa3, d1m7xb3, d1m7xb1, d1m7xb2, d1m7xc1, d1m7xc3, d1m7xc2, d1m7xd1, d1m7xd2, d1m7xd3 UniProt : P07762
Functional annotation: Biological process: carbohydrate metabolic process glycogen biosynthetic process Molecular function: catalytic activity hydrolase activity, hydrolyzing O-glycosyl compounds 1,4-alpha-glucan branching enzyme activity cation binding transferase activity transferase activity, transferring glycosyl groups
Evolutionary conservation: Toggle Conservation Colors: Rows = identical sequences: Further Information: Caveats, Explanation, Complete Results at ConSurfDB
Resources:	FirstGlance, OCA, RCSB, PDBsum
Coordinates:	save as pdb, mmCIF, xml

^{[1] [1]} Escherichia coli Branching Enzyme (BE) (1,4-a-glucan 6-glucosyltransferase)catalyzes the formation of a-1,6 branch points of glycogen (similar process for start with starch branching points). The enzyme contains three domains: a NH2-terminal seven stranded b-sandwich domain, a COOH-terminal domain, and a central a/b-barrel domain containing the enzyme active site. The branching enzyme belongs to the a-amylase family of enzymes.

Contents 1 Introduction 2 Glyogen Storage Disease type IV 3 References

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Glyogen Storage Disease type IV

Mutations in the gene of Branching Enzyme can result in the accumulation of insoluble glycogen in the cell known as Glycogen Storage Disease type IV (GSD-IV). This results in an impaired enzyme which prevents the formation of branch points in glycogen, producing an insoluble polymer. Different forms of GSD-Iv affects the liver, muscular tissue, and/or the central and peripheral nervous system.

References

1. ↑ ^{1.0 1.1} Abad MC, Binderup K, Rios-Steiner J, Arni RK, Preiss J, Geiger JH. The X-ray crystallographic structure of Escherichia coli branching enzyme. J Biol Chem. 2002 Nov 1;277(44):42164-70. Epub 2002 Aug 23. PMID:12196524 doi:10.1074/jbc.M205746200

E.ColiBranchingEnzyme