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Kamis, Juni 6

GLYCOGEN METABOLISM

INTRODUCTION 

Glycogen is the stored form of carbohydrates in the body are the main animals and analogous to starch in plants. This element is mainly present in the liver (up 6%) and the amount of muscle that rarely exceed 1%. However, due to the much larger mass, the amount of glycogen stored in the muscle could reach three to four times the amount in the liver. Such as starch, glycogen is a polymer branched aD-glucose 

Glikogenesis MAINLY OCCUR IN MUSCLE AND hepatic 
 
Muscle glycogen serves to be a source of hexoses available for the process of glycolysis in the muscle itself. Liver glycogen is mostly associated with sim ¬ hexose storage services and shipping out to defend ¬ blood glucose levels, especially in times before breakfast. After 12-18 hours of fasting, hatnpir all deposits in the hepatic glycogen depleted, while the new muscle gene ¬ gliko experience significant depletion after someone ¬ people doing strenuous exercise and long. Glycogen disease (glycogen storage disease) is a group of inherited disorders characterized by impaired mobilization of glycogen and accumulation forms gliko ¬ abnormal gene, thus resulting in muscle weakness and even death of the sufferer. 

Major Factors Controlling Glycogen Metabolism in the Liver is the concentration of phosphorylase a 
 
This enzyme not only controls the phase boundary ¬ glycogenolysis dalarn speed, but also inhibits the activity of protein phosphatase-1 and thus Mengen ¬ grip on glycogen synthesis). Fosfori ¬ lase inactivation occurs as a result of allosteric inhibition by glucose when levels of these compounds to increase after eating.
Activation of 5'-AMP is caused by a reaction to ATP depletion. Administration of insulin causes inactivation-phosphorylation immediately followed by activation of gli ¬ cogeneration synthase. The insulin effect requires the presence of glucose. 

CLINICAL ASPECTS Deposit Disease Glycogen (Glycogen Storage Diseases) is a Congenital Diseases 

The term "glycogen disease (glycogen storage diseases)" is a generic term that meant un ¬ to explain a group of inherited disorders in ¬ marked by a buildup of glycogen in the amount or type of abnormal tissue in the body.
Abnormalities important glikogeno ¬ sis summarized in the Table. Adenil enzyme deficiency and protein kinase ¬ cAMP-dependent kinase has also been reported. Some abnormalities in ¬ explained successfully rescued by liver transplantation.
Table. Glycogen disease stroge

Glikogenosis
Names
Abnormalities diseases
Characteristics
Type I
Von Gierke's disease
Deficiency of glucose-6-phosphatase
Liver cells and renal tubular cells contain glycogen, hypoglycemia, laktiasidemia, ketosis, hyperlipemia.
Type II
disease Pump
Deficiency of lysosomal and 1Q4-1 ® 6 glucosidase (acid maltase)
Fatal, accumulation of glycogen in lysosomes in heart failure.
Type III
Limit dextrinosis, forbes disease or cori
The absence of enzyme breaker
Typical branched polysaccharide accumulation
Type IV
Amilopektinosis, disease andersen
The absence of branching enzyme
Accumulation of polysaccharides that have multiple branching points, deaths due to heart failure or liver in the first year of life
Type V
Miofosforilase deficiency, McArdle syndrome
The absence of muscle phosphorylase
Loss of tolerance to physical exercise, muscle glycogen contains abnormal (2.5-4%). Little or no lactate in the blood after exercise
Type VI
herd disease
Deficiency of liver phosphorylase
High content of glycogen in the liver, the trend toward hipogelikemia
Type VII
Tarui disease
Phosphofructokinase deficiency in muscle and erittrosi
Such as type V but also possible hemolytic anemia
Type VIII

Liver kinase deficiency forforilase
Such as type VI

CONCLUSION

(1) Glycogen is the stored form of carbohydrate in the main body of mammals and is found mainly in the liver and muscles.
(2) In the liver, the function utan: a glycogen is to serve other body tissues through the formation of blood glucose. In muscle, this element only meet the needs of the organ itself as a source of metabolic fuel yanv ready to use.
(3) Glycogen is synthesized from glucose and other precursors through the trajectory of his ¬ glikogenesis. The solution occurs through a separate path known as gliko ¬ genolisis. Glycogenolysis led to the formation of glu ¬ vocabulary in lactate formation in the liver and muscle, respectively caused by the presence or absence of the enzyme glucose-6-fospatase.
(4) regulation of cyclic AMP integrates glycogenolysis and glycogenesis by promoting mutual phosphorylase enzyme activity and inhibition of the enzyme glycogen synthase.
(5) Congenital abnormalities deficiency of specific enzymes in the metabolism of glycogen in the liver and muscle glycogen is the cause. 

REFERENCES 
 
Murray, Robert K. , 1999. BIOCHEMISTRY HARPER / Robert K. Murray. Ed. 24. Jakarta: EGC. It 190-198.
http://addiansyah.wordpress.com/2010/05/14/metabolisme-glikogen/


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