Glycogens structure

Earner, J., 1990. Insulin and the stimulation of glycogen synthesis The road from glycogen structure to glycogen synthase to cyclic AMP-dependent protein kinase to insulin mediators. Advances in Enzymology 63 173-231. 

Type Deficient Enzyme Cardinal Clinical Features Glycogen Structure... 

The most common glycogen storage disease. Type I or von Gierke disease, is a deficiency in glucose 6-phosphatase in which glycogen structures are normal however, the liver is unable to dephosphory-late glucose 6-phosphate, and it remains trapped In the cell. 

Comparison of theoretical and experimental aspects of glycogen structure. 

Normal glycogen structure increased glycogen stored... 

Figure 21.1. Glycogen Structure. In this structure of two outer branches of a glycogen molecule, the residues at the nonreducing ends are shown in red and residue that starts a branch is shown in green. The rest of the glycogen molecule is represented by R.

R. Melendez, E. Melendez-Hevia, and M. Cascante. 1997. How did glycogen structure evolve to satisfy the requirement for rapid mobilization of glucose A problem of physical constraints in stmcture building J. Mol. Evol. 45 446-455. (PuhMed)... 

Type Name No. of cases reported Organs affected Glycogen structure Enzyme deficiency... 

Manners, D.J., Recent developments in our understanding of glycogen structure. CarbohydrPolym 16 37-82, 1991. 

Complete breakdown of glycogen also requires a debranching reaction to hydrolyze the glycosidic bonds of the glucose residues at branch points in the glycogen structure. The enzyme that catalyzes the first of these reactions is glycogen phosphorylase the second reaction is catalyzed by phosphoglucomutase. 

Fig. 4.—Structures Around the Outermost Branch-points in the Limit Dextrins Remaining After Action of foeta-Amylase on Amylopectin and Glycogen. [Structure (b) is obtained uniquely by foeta-amylase action on phosphorylase limit-dextrins (see p. 315). For an explanation of the symbols, see footnote 49.]...

Muscle phosphorylase (EC 2.4.1.1) deficient. Glycogen structure normal. Glycogen accumulates only in muscle. Exercise causes muscle cramps myoglobin from damaged muscle may appear in urine. Patients symptomless if they refrain from strenuous exercise. Prognosis favorable. 

Phosphofructokinase (mu e isoenzyme) (EC 2.7.1.11) defident. Glycogen structure normal. Glyco-... 

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