Introduction: Glycogen storage disease is the result of a defect in the synthesis or breakdown of glycogen that is found in muscles, the liver and many other cell types. This disease may be genetic or acquired and is usually caused by a defect in certain enzymes that are important in the metabolism of glycogen. To date, there are 11 different classifications for glycogen storage disease but this paper will focus on glycogen storage disease type 1 (GSD I), also known as von Gierke’s disease, after the
Glycogen Storage Disorder There are many different things that can go wrong in our bodies. A metabolic disorder can be one. There are many different kinds that maybe deadly if left untreated and others can be treated but still left to deal with for the rest of one's life. One disorder can be glycogen storage disorder this can affect many different age groups from new born to the elderly. It deals with the function of the body to obtain its greatest source from which it obtains energy from. It deals
The Hereditary Disease of Tay-Sachs Disease Tay-Sachs disease is a rare hereditary disease found mainly in infants but is also found in juveniles and adults. It is caused by the abnormal metabolism of fats and is characterized by mental deterioration, blindness, and paralysis. There is no available treatment for this disease. The ethnic group of Ashkenazi Jews, is very tightly knit. Their religion teaches them to remain among their own small group. Ashkenazi Jews rarely marry outside of their
Sandhoff disease is an uncommon hereditary condition that—along with several other disorders—was previously known as amaurotic idiocy. Sandhoff disease destroys nerve cells (neurons) in the brain and spinal cord little by little over time. The disease is described as the buildup of lipid-containing cells in the internal organs and in the nervous system, mental retardation, and loss of sight. It is one of over 50 hereditarily inherited conditions identified as Lysosomal Storage Diseases The disease was
intracellular fluid, which in turn is alkaline with a high pH level. Fortunately the lysosomes have a surrounding membrane that protects any damage to the cells cytosol. There are well over 50 known diseases associated with lysosome dysfunctions, such as Gaucher disease, Tay Sachs disease, Pompe disease, beta-galactosidase deficiency. Including many in which lysosomes cannot break down material that enters the cell therefore retaining it rather than disintegrating them, there are others where the lysosom
Fabry disease affects every 1 in 40,000 -60,000 males. It occurs less frequently in females. In general population is 1 in 117,000 people. It affects all ethnicity groups. Fabry disease is a rare hereditary disorder that’s from buildup glycolipids called GL-3 in the body’s cells, tissue, or other organs. The damage to the cells, tissues, or organs can cause a wide range of mild to severe symptoms that can be life threatening. Signs and symptoms’ may be noticed in childhood or adolescence. Many patients
There are numerous metabolic diseases that affect people all around the world. One of the most common metabolic diseases is diabetes which involves carbohydrate metabolism. Carbohydrate metabolism starts with digestion in the small intestine where simple carbohydrates are absorbed into the blood stream (1). Blood sugar (glucose) concentrations are managed by three hormones: insulin, glucagon, and epinephrine. When glucose concentrations in the blood become elevated, insulin is secreted by the pancreas
or sucrose by hydrolysis within the small intestine, and is then absorbed into the blood. Elevated concentrations of glucose in blood stimulate release of insulin, and insulin acts on cells thoughout the body to stimulate uptake, utilization and storage of glucose. The effects of insulin on glucose metabolism vary depending on the target tissue. Two important effects are: Insulin facilitates entry of glucose into muscle, adipose and several other tissues. The only mechanism by which cells can take
clinics in Vienna. Knowing that Europe would engage in war, they took measures into their own hands, and applied for jobs overseas in America. In 1922, they moved to New York, where Carl took a job at the State Institute for the Study of Malignant Diseases, and Gerty was hired as an assistant pathologist. Although they were discouraged from working with one another, they continued to anyways. They dedicated their work on how energy is created and transferred in the human body. Focusing in biochemistry
from eating refined sugar can override the “I’ve had enough to eat” mechanism from other digestive hormones. This means that you can be at greater risk of eating more than what your body actually needs. Liver: The liver stores excess sugar as glycogen (storage friendly version of glucose). If you eat refined sugar daily (above the natural sugar
To Intermittent Fast, or not to Intermittent Fast, that is the question Have you ever heard of intermittent fasting? Could you have ever imagined that not eating breakfast could bring you untold health benefits? Chances are you never came across intermittent fasting, well; trust me I guarantee you are missing out if you are unaware of it. By the end, of this article I am certain you will understand what intermittent fasting is and why it is so popular. What is intermittent fasting? Intermittent
the blood and hyperglycaemia occurs. This abnormally high level of blood glucose is able to harm the nervous system, tiny blood vessels in the kidneys, heart and the eyes. Type 1 Diabetes is fatal when left untreated as it then causes heart disease, kidney disease, damage to the nerves, stroke and
glucose and the storage of nutrients evidence by the resistance to the actions of the insulin or an absolute deficiency of insulin. Diabetes mellitus is classified into four clinical classes. Included are type 1 DM and type 2 DM. Other categories comprehend gestational diabetes mellitus and other specific types of diabetes and these can occur secondary to other conditions. The insulin resistance and the metabolic syndrome is a problem that can lead to diabetes mellitus and other diseases. Diabetes is
Creatine is one of the most beneficial substances that aid and help athletes improve performance. Creatine was first discovered back in 1832 when researcher Michel Eugene Chevreul “isolated it from the basified water-extract of skeletal muscle. He later named the crystallized precipitate after the Greek word for meat, κρέας (kreas)” (Creatine Wikipedia). Even though creatine was discovered back then, it was dropped and then picked back up in 1912, when Otto Folin and Willy Glover Denis from Harvard
The body digests fat easiest and first. Carbohydrates are digested next and may depend on the type. Simple carbs (white bread, white sugars) are fast and easy to digest by the body and can lead to an insulin surge that will lead to increased fat storage. Complex carbohydrates are more slowly absorbed by the body and should be the main source of carbs in the diet. Protein takes the most effort by the body and will be digested last. It is another myth that protein is never stored as fat - it does not
What Exactly Is Forskolin? How Does It Work? Forkskolin grew in popularity after Dr. Oz featured it on his show. It comes from the mint family. Forskolin comes from the root of the Plectranthus barbatus, or Coleus forskohlii, plant. It helps break down fat. That is why it is widely used by people who are interested in improving their health and fitness. Not only can Forskolin help people burn more fat, but it can also improve performance and increase metabolism. Furthermore, it assists in a process
All that was known about the pancreas was that it had an endocrine system and what it looked like. Nothing was known about the glycogenic function of the liver because Bernard was the first person to find glycogen inside the liver. Also for his discovery of vasomotor nerves he was the first to discover that the vasomotor nerves control blood supply and blood vessels (“Bernard”). The only award Bernard received was the Copley Medal from the Royal Society of
Introduction Exertional rhabdomyolysis (ER) is a concern for those who handle horses, especially in the case of performance horses. Rhabdomyolysis is a disease affects the muscles, leading to muscle deterioration often after a period of prolonged exertion or exercise. ER will exhibit a variety of symptoms depending on the severity and the breed. Some horses will present with stiffness in gait, muscle soreness and general poor performance 1. Upon examination muscles will appear swollen and/ or hard
endothelial function. (Davis, et al., 2011) Endothelial function is associated with an increase in cardiovascular disease. (Davis, et al., 2011) Also low carb diets show a short term improvement in cardiovascular risk factors such as insulin resistance and HDL cholesterol. (Friedman, et al., 2012) These studies indicate that short term low carb diets are beneficial in reducing cardiovascular disease. Another benefit is that low carb diets do not have any effect on the kidneys. It was found that being on
equilibrium in its levels could lead to diseases and ultimately death. Glucose is used as energy source for muscle contraction as its breakdown produce ATP (Chen et al., 2010). Blood glucose levels (BGL) are regulated by hormones produced in the pancreas; Insulin (Perelis et al., 2015) and Glucagon (Tirosh, 2014), which work together to maintain healthy levels in the body. Insulin promote glucose uptake from the blood into muscle and fat to be converted into glycogen and used as energy reservoir. Insulin