Hereditary Fuctose Intolerance Research Paper

The biochemical basis of this disease is related to the metabolism of fat. It is caused by an abnormality in the ABCD1 gene located on the X chromosome. This prevents the production of ALDP which functions as a

Lustig addresses is excess fat production related to fructose consumption. People who consume high amounts of fructose tend to gain excess weight, which can lead to other health issues. When fructose enters the body, it is transported to the liver where it will be broken down into a usable form of energy. Fructose is eventually turned into pyruvate and undergoes pyruvate dehydrogenase creating acetyl-CoA. From acetyl-CoA, there are two possible pathways for fructose to take; fatty acid metabolism or the TCA cycle. The TCA cycle is limited in the rate that acetyl-CoA can enter. Because of this, when an excessive amount of sugar is consumed more acetyl-CoA will undergo fatty acid metabolism. Fatty acids are stored in the body as triglycerides. When too much sugar is consumed, fatty liver disease may result. This occurs when too many fatty acids are created, thus the body cannot ship them out to storage. Instead, these fatty acids become stored in the liver. An excessive amount of sugar intake will lead to fat production, but with proper exercise this problem can be mediated. Exercise can speed up the TCA cycle as well as allow gluconeogenesis, which drastically decreases the amount of fat production. This leads to the conclusion that not every person who consumes fructose will accumulate excess

In correlation with weight gain, HFCS significantly increases the rate of type II diabetes in the United States. Diabetes occurs when there is an insufficient amount of insulin to transport glucose to body cells to be used as fuel. As a result of this, the cells starve, causing the degeneration of tissues and organs. HFCS increases the likelihood of diabetes because the obesity caused by HFCS consumption vastly contributes to the inability of

• Mutations in the ALDOB gene reduce the function of the enzyme, impairing the ability to metabolize fructose.

The National Institute of Health (2014) listed the most common form of familial hyperinsulinism to be an autosomal recessive genetic defect that is common among the Ashkenazi Jewish population. This condition affects 1 in 66 carriers who are Ashkenazi Jews since they have reported consanguineous marriages (NIH, 2014). Among Ashkenazi Jews, two single ABCC8 mutations account for 90 percent of cases of familiar hyperinsulinism (Mazor-Aronovitch, et al, 2007). The incidence of FHI in individuals of northern European descendants is approximately 1:30,000 live births. The incidence is increased in genetically isolated populations with a high prevalence of consanguinity (UpToDate.com).

However sugar has been linked to some of these problems too but I personally avoid H.F.C.S. at all costs, but not necessarily sugar.

"Crystalline fructose" is an especially deadly form of HFCS. It has a significantly higher percentage of fructose in addition to dangerous chemicals like arsenic and chlorine and metals such as lead. Almost all processed foods contain high fructose corn syrup or some version of it. You will find it in soft drinks and fruit juice. But it is also in breakfast cereals, mayonnaise, peanut butter and jellies, ketchup, yogurt, cookies, candy, mustard, canned soup, potato chips, pancake syrup, beer, and the list goes

We all know that High Fructose Corn Syrup is in our food but we don’t know what it truly is and its hidden health risks it carries. High Fructose Corn Syrup is mad of two main things: fructose and glucose. Fructose is a sugar that is found in many fruits and vegetables, and glucose is a sugar that helps our bodies retain energy. Fructose is a tough energy source for the body to use due to it has to be processed through the liver. “It is also more lipogenic, or fat-producing,

Fructose was considered to be beneficial to diabetics because it is absorbed only forty percent

Familial Dysautonmia is passed on through genetics. It is very rare because it is a recessive gene. Both parents will have to have a mutilation for a child to get Familial Dysautonmia. Some people may have the mutated gene, but not show symptoms of Familial Dysautonmia. It is from a mutation in the IKBKAP gene. Think of genes

hereditary disorder of Caucasians in the United States and is the most common cause of chronic

Some people have become susceptible to poisoning via fructose, a sugar commonly found in plants (Moalem 3). “One single letter change in a series of billions of letters and you’ve got bones that break with the slightest of pressure. Another small shift and you wouldn’t feel that broken bone at all” (Moalem 171). The examples given in that quote are exceedingly rare mutations. One example of a much more common mutation is a mutation in lactase, an enzyme responsible for the digestion of milk. Production of lactase is typically ceased at about age 4, but some carry a mutation that allows them to digest milk their entire lives (“What is Mutation?” Learn.Genetics). In fact, this mutation is so common here in America that most people find it strange when someone is lactose-intolerant. This mutation in the production of lactase is one commonly found in people of European descent, and everyone in my family carries this mutation.

The lipoprotein gene mutation has a penetrance of greater than 90% (Henderson et al., 2016). Heterozygosity takes place when an individual has two dissimilar alleles of a gene. A single dominant gene can manifest itself in a heterozygous person particularly when there is high penetrance property. This is the reason why multiple generation can be affected by the FH disorder as seen in the proband and family members of the followed case discussion. Monogenic FH is largely attributed to mutations in the LDL-receptor (LDLR), apolipoprotein B (APOB), and proprotein convertase, subtilisin/kexin-type 9 [PCSK9] genes (Brautbar et al.,

Although cells require sugar for energy, it is glucose the cells prefer as a source. However, much of the excess sugar consumed today is fructose. Excess fructose consumption has similar effects on the liver as excessive alcohol intake, as the liver metabolizes alcohol the same way as sugar and converting the carbohydrate into fat (Brandis, Lustig, & Schmidt, 2012). High concentrations of fructose is rare in nature and is typically found along side fiber. However, in the American diet, the fiber is

The most common is Type 2 diabetes. Type 2 diabetes is diagnosed when your body doesn’t use the insulin right. This happens when the pancreas can’t keep up with producing insulin. Whenever the process slows down it causes high blood sugar (Symptoms & Causes of Diabetes, niddk). The cause of it is most likely lifestyle factors, but can be genes.