Phenylalanine

Dzevdet Smajlovic, professor from the Department of Neurology at the University of Tuzla in Bosnia stated that

The central nervous system (CNS) comprises grey matter, which contains neuron cell bodies and white matter, which contains the nerve axons. Most of the nerve axons are concentrically wrapped around by lipid-rich biological membrane, known as the myelin sheath. In the CNS, myelin is produced by oligodendrocyte. a type of glial cell. (Pfeiffer et al., 1993). These electrical insulating, multilamellar membranes significantly increase the electrical resistance, in which to prevent leakage of electrical currents from the axons, as well as decrease electrical capacitance to reduce the ability of the axons to store electrical energy (Shivane &

Write a brief description of the three slides (Neuron, Spinal Cord Smear and C.S of Myelinated Nerve Fibers) as though you were explaining it to someone who cannot see the slide.

The breakdown of the myelin sheath is caused from a mutation of the gene that makes the Adrenoleukodystrophy protein (ALDP). This ALD protein helps the body metabolize saturated very-long-chain fatty acids found in the serum and tissues of the central nervous system. The newly mutated gene no longer acts as a help aid to breaking down the long-chain fats. Therefore, the body starts accumulating an abnormal amount of fat in the nervous system, adrenal gland and testes that sets off an unusual response in the immune system; demyelination.

Myelin, the common factor in each disease, is a subsatnce that surrounds and insulates axons on some nerve cells, allowing for a faster transporting of signals and proper functioning of the nervous system. A demyelinating disease results in the damage of nerve fibers in the brain and spinal cord due to the myelin sheath being destroyed, which is life threatening.

In people with PKU Phenylalanine’s most apparent and benifical side affect is itrs function as a necessary building block for protein; It may also help create chemicals that work to regulate appetite and mood.

With this in mind, many people who suffer with PKU use many low protein, dairy free, fruits and vegetables products to maintain their health and even stated that “PKU isn't just a diet thing. It's a brain thing.” Since all 50 states and territories of the United States require the screening of PKU, it is normally tested right after the baby is born. By taking a few drops of blood from the heel of the baby, the sample will be sent to a laboratory to test the amount of phenylalanine in the sample. Since PKU is also known as a genetic disorder, many health care providers also suggested the idea of genetic testing so it can into detail about the mutations in the genes that will cause

The occipital lobe is in a posterior position and the function of the occipital lobe is vision including perception and colour and movement (Stannard Gromisch, 2012). Atrophy is described as a process of tissue wastage or cell death (Lippincott Williams & Wilkins, 2006). According to (Jones & Gaillard, 2005-2013) the pathology of PCA includes Neuritic plagues and neurofibrillary tangles which are found in the parieto-occipital and temporo-occipital areas, as well as occasionally in the primary visual cortex. It is thought that PCA is a version of AD, with very little difference in the pathology. “Neuritic plagues or senile plagues are extracellular and composed of a central core of beta-amyloid peptides aggregated together with fibrils of beta-amyloid, dystrophic neurites, reactive astrocytes, phagocytic cells, and other proteins and protein fragments derived from degenerating cells or liberated from neurons” (Weerakkody & Gaillard, 2005-2013).

Untreated PKU can lead to brain damage, intellectual disabilities, behavioral symptoms, or seizures. The defective gene does not produce the enzyme necessary to break down and rid the body of phenylalanine. The symptoms of phenylketonuria result from a buildup of (Global Genes, 2012) I eat differently. My friends understand PKU is very difficult, but-non PKU people think I am VERY different. I’ve had to adjust to other’s perceptions and learn to cope with what other people expect is the norm.”(Global Genes, 2012) this researcher would describe this disorder as A birth defect that causes an amino acid called phenylalanine to build up in the body. Phenylketonuria is an internal disorder. delayed development, failure to thrive, short stature, or slow growth

Approximately 100 billion neurons are in the brain, to get a grasp of that number, imagine counting every neuron in the brain. If one were to count two neurons for every second, then approximately 1600 years would have passed, by the time 100 billion neurons were accounted for. Every neuron has a purpose in the brain, whether it be used for an emotion, memory, or action. The basic components of a neuron is the axon, myelin sheath, dendrites, cell body, and axon terminals. Concerning the myelin sheath or myelination, this fatty-like material main purpose is to increase the speed of the electrical impulse, which is given by other neurons. Whenever myelin is present, the electrical impulse leaks out at a slower rate. This aids in faster mental processing, without myelination, the individual’s thoughts would take seconds to create. In turn,

Current guidelines dictate a slow correction of sodium, not exceeding 10 mmol/24 hrs to prevent central pontine myelinolysis (CPM) (Greenberg et al, 2015, Spasovski et al, 2014, Verbalis et al, 2007, Verbalis et al, 2013). CPM is a non-inflammatory demyelinating disease characterized by the loss of myelin in the base of the pons and carries a poor prognosis, often times resulting in death. It was first described by Adams et al in 1958, in patients with hyponatremia in the setting of alcoholism and malnutrition (Adams, et al, 1958, Martin, 2004, Staikov, et al, 2015). The exact mechanism of demyelination remains unknown, however one proposed theory is that when osmotically active substances such as sodium are very low in the serum in the setting of hyponatremia, free water shifts into the brain and the brain adapts by allocating water into the cerebrospinal fluid and redirecting intracellular solutes out of the cells. If serum osmolarity rises back to normal too quickly, an osmotic gradient develops and causes destruction of myelin. The pons is vulnerable as it is less capable of transporting solutes across membranes than other areas of the brain leading to localized pontine demyelination. Catastrophic outcomes such as pseudobulbar palsy, spastic tetraparesis, locked-in syndrome and death can occur (Martin, 2004). In the patient described above, he developed

Phenylketonuria is a condition is when phenylalanine is in your body and you can’t break down an amino acid "PKU (Phenylketonuria) in your baby | March of Dimes." Also, other people say it is an inborn disorder of metabolism, which characterized by a deficiency or lack of a vital enzyme phenylalanine hydroxylase (Genetics of Phenylketonuria). All proteins and some artificial sweeteners contain Phenylalanine in it (Phenylketonuria." Healthline). Your body uses an enzyme called phenylalanine hydroxylase to convert phenylalanine into tyrosine, a non-essential amino acid (Phenylketonuria." Healthline). Without tyrosine, your body would have nothing to create neurotransmitters, such as norepinephrine, epinephrine, and dopamine (Phenylketonuria." Healthline).

by neurological diseases such as Alzheimer’s disease. A study on the deterioration of myelin in people

Phenylketonuria (PKU) is a rare problem in which babies are born without their body’s ability to efficiently break down the amino acid known as phenylalanine. These infants are missing the enzyme phenylalanine hydroxylase. Babies are now screened after birth.

Most of the A peptides produced are 40 amino acids long, however it is thought in