Living organisms are mainly based on four main classes of biomolecules.
Carbohydrates, proteins, nucleotides, and lipids are involved in and regulate many biochemical reactions.
Of these biomolecules, carbohydrates are the most abundant biomolecules and show various functions in living organisms such as energy transport, which are structural components.
They are also involved in the immune system, in the blood clotting process, among others.
These biomolecules are composed of carbon, oxygen and hydrogen with the general formula C n (H 2 O) n.
They are basically polyhydroxy aldehydes and ketones.
Carbohydrates are made up of tens to hundreds to several thousand monosaccharide units and are also called polysaccharides.
Common polysaccharides contain glucose as a monosaccharide unit and are synthesized by plants, animals, and humans.
They mainly act as reserve food, participate in structural support (the cell wall in the plant cell and the external skeleton of insects in the form of chitin) and are metabolized to produce energy.
Polysaccharides act mainly as food preservatives, in the food reserve of plants in the form of starch.
In humans and other animals it is stored as glycogen in the liver and muscles.
Glucose and other carbohydrates are the main sources of energy for the human body.
When we consume sugars and carbohydrates, we are increasing the available energy molecules that can be used for cellular respiration and the production of adenosine triphosphate (ATP).
Glycogen is a polysaccharide that is formed from excess glucose in the body.
Simple glucose molecules can form glycosidic bonds to form larger macromolecules.
As we consume sugar, either as individual molecules or in the form of starches, we break these bonds to release the glucose and monosaccharides necessary for the production of ATP.
Any excess glucose is stored as glycogen in the liver and muscle cells for future use in case energy needs increase dramatically.
Glucose that is not used immediately is stored as glycogen in the liver and muscles through the process of glycogenesis.
Some tissues, such as the liver and skeletal muscle, store glucose as glycogen, which can be rapidly mobilized.
For example, when blood glucose is high and glycogen breaks down and releases glucose into the bloodstream, while in the case of low blood sugar such as fasting muscle uses its glycogen stores for energy and serves as a buffer. to maintain blood glucose levels.
Glycogen consists of long polymeric chains of glucose units that are linked by an alpha bond.
A bond is formed by the combination of carbonyl group and alcoholic group.
If the carbonyl group is an aldehyde group (-CHO), it is called hemiacetal and if there is a ketone group, it forms a hemiketal bond.
If two alkoxy groups are attached on the same carbon atom, they called it acetal group and the bond was called acetal bond.
In the case of glycogen, all alpha-D-glucose are linked together by an alpha-acetal bond between C1 of one monomeric unit and C4 of another monomeric unit, which is why it is called αα-1,4-glucosidic bond.
Since glycogen is a branched polymer, branching occurs at intervals of 8-10 glucose units.
These branches are formed by an acetal bond between C1 and C6.
There are possible forms of glycogen, proglycogen having a molecular mass of around 400 kDa and macroglycogen with a very high molecular mass.
Glycogen can be organized in a globular form in which the glucose chains are organized globularly around a central protein glycogenin.
It seems that the branches of a tree originated from a central point.
Excess glucose is stored in the form of glycogen.
Glycogen is stored in the liver and muscles that act as storage sites.
For all normal movements and functions, energy is first provided by glucose contained in the bloodstream and, after consumption of this glucose, energy is extracted in the form of glycogen from storage sites.
The synthesis of glycogen from glucose is called glycogenesis.
The synthesis of glycogen is called glycogenesis and it is an endergonic process, therefore, a certain contribution of energy is required, provided by uridine triphosphate.
Glycogenesis is a four-step process in which each step is catalyzed by certain enzymes.
-In the first step, glucose is converted to glucose-6-phosphate in the presence of glucokinase or hexokinase enzymes.
This glucose-6-phosphate forms glucose-1-phosphate by the action of phosphoglucomutase.
Uridine triphosphate reacts with glucose-1-phosphate to form UDP-glucose and pyrophosphate in the presence of UDP-glucose pyrophosphorylase or Uridyl Transferase.
Pyrophosphate additionally hydrolyzed into 2 molecules in the presence of pyrophosphatase.
UDP-glucose acts as monomeric units progressively lengthens the glycogen chain with (α1â † ‘4) bound glucose in the presence of the enzyme glycogen.
- Excess glucose is stored as glycogen at storage sites.
- In case of low blood sugar, a glucagon hormone is released which involves the conversion of glycogen into blood sugar, a fuel source.
- This process is known as glycogenolysis.
- The process of glycogenolysis is initiated by the enzyme glycogen phosphorylase which involves the cleavage of terminal glycogen residues by inorganic phosphate.
- This enzyme requires a covalently linked pyridoxal phosphate co-factor (vitamin B6).
- Cleavage of the terminal glycogen residue forms glucose-1-phosphate, which is further converted to glucose-6-phosphate by phosphoglucomutes.
- The complete breakdown of glycogen requires the action of a second bifunctional enzyme known as a debranching enzyme as oligo (αα-1,4 -> αα-1,4) glucantransferase.
- Hence the glycogen breakdown process completed in three steps.
- The conversion of glycogen to glucose 1-phosphate.
Remodeling of the glycogen substrate for further degradation:
- Formation of glucose-6-phosphate from glucose-1-phosphate for increased metabolism.
- Two hormones, glucagon from the pancreas and epinephrine from the adrenal glands, regulate the process of glycogenolysis.
- Both processes are related to glycogen, glycogenesis and glycogenolysis regulated by two enzymes, one involved in the synthesis of glycogen, which is glycogen synthase, and the other in the degradation of glycogen, which is glycogen phosphorylase.
- The action of both enzymes is regulated by circulating hormones including insulin, glucagon and epinephrine and by the level of energy and metabolites available to the cell.
- The end product of glycogenolysis, glucose 6-phosphate can be involved in several pathways.
- It acts as a precursor to glycolysis.
- Involve in the processing of the pentose phosphate pathway to form NADPH and ribose derivatives.
- During the need for energy, it can be converted into glucose that provides energy during various metabolic processes.
- Hepatic glycogen acts as a glucose reserve that releases hepatocytes when there is a need to maintain normal blood sugar levels. There is approximately 40 Kcal in body fluids, while liver glycogen can provide approximately 600 Kcal after an overnight fast.
- Glucose from glycogen stores remains within cells in skeletal and cardiac muscles and is used as an energy source for muscle work.
- The brain includes a small amount of glycogen in astrocytes. It accumulates during sleep and is mobilized when walking. Glycogen stores also ensure a moderate degree of protection against hypoglycemia.
- It has a specialized role in fetal type II lung cells. These cells begin to accumulate glycogen at approximately 26 weeks’ gestation and then synthesize lung surfactant.
Glycogen storage disorders
The used glycogen is stored as soon as we eat something to avoid depletion of the glycogen level.
In low glycogen, the body begins to break down proteins to use them as fuel sources that are harmful to the body.
Glycogen deficiency and abnormal functions cause many storage disorders.
Since the formation and breakdown of glycogen involves many enzymes, there are many different types of glycogen storage disorders in which each disorder is related to the lack or malfunction of different enzymes.
For example, the problem associated with the production of those enzymes that participate in the synthesis of glycogen, can produce abnormal units of glycogen.
Similarly, defects with enzymes related to glycogenolysis can lead to low glucose in the body which is known as hypoglycemia, accumulation of glycogen in the muscles and liver and responsible for innate errors of metabolism.
Glycogen storage disorders primarily affect the liver and muscles with other parts of the body such as the kidney, heart, blood vessels, nervous system, and intestine.
Glycogen storage diseases
Glycogen is the form of sugar that your body stores in your liver and muscles for future energy needs.
Glycogen storage diseases are complex genetic conditions in which certain enzymes, which are involved in creating glycogen or breaking it down to sugar for your body to use, are either missing or not working properly.
This can lead to liver, heart, muscle, and breathing problems.
There are several different types of glycogen storage diseases, the most common of which are:
- Von Gierke disease (type I).
- Pompe disease (type II).
- Forbes-Cori disease (type III).
- Andersen’s disease (type IV).
- McArdle disease (GSD V).
- Glycogen storage diseases VI.
- Glycogen storage diseases IX.
- Glycogen storage diseases 0.
This class of diseases is most often diagnosed in babies, but it can also be diagnosed in adults.
Glycogen storage disease complications
Complications vary depending on the type of glycogen storage disease; however, they can include:
- Liver problems
- Low blood sugar
- Gastrointestinal problems such as inflammatory bowel disease.
- Growth and developmental delays.
- Lung problems.
- Heart problems.
- Additional complications can include muscle disease, blood disorders, and kidney problems.
Diagnosis of glycogen storage diseases
It is based on family history and medical tests to diagnose glycogen storage diseases.
The following tests can be ordered:
It can be used to monitor liver, kidney, and muscle health, and to ensure adequate blood sugar levels.
It can discover the presence of genetic changes that cause disease.
It is used to check for certain markers of diseases and hereditary traits.
Tissue samples taken from the liver and muscle are tested for disease or abnormal cell function.
Contrast-enhanced ultrasound, CT, and MRI create detailed images of the size, structure, and function of organs and vessels.
This non-surgical alternative to a liver biopsy uses ultrasound to check for stiffness of the liver due to scarring, called liver fibrosis.
Monitoring of preventive diseases
Living with glycogen storage disease means closely monitoring the results of laboratory tests, as well as periodic tests and examinations to diagnose complications when they arise.
Severe forms of glycogen storage disease can damage the heart and lungs and cause infections.
The effects of some forms of glycogen storage disease can be reversed by maintaining healthy levels of vitamins, minerals, and enzymes for proper growth and development.
Depending on the condition, special diets may include meals high in carbohydrates and starch, frequent meals to maintain blood sugar levels, cornstarch therapy to avoid low blood sugar, or limiting the foods you eat. body cannot decompose.
Physical and occupational therapy
People with glycogen storage disorders often work with physical and occupational therapists to build strength and promote proper development.
These therapies can help with motor skills to perform the tasks of daily living.
Weakened muscles and developmental delays related to glycogen storage disorders can affect speech.
Speech therapy is used to teach children how to do the correct mouth movements to improve their words and language acquisition.
Surgery may be necessary if the liver, heart, or digestive tract are affected by the disease.
If serious damage occurs, organ transplants may be recommended.