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Freely available amino acids are used to create proteins. If amino acids exist in excess, the body has no capacity or mechanism for their storage; thus, they are converted into glucose or ketones, or they are decomposed. Amino acid decomposition results in hydrocarbons and nitrogenous waste. However, high concentrations of nitrogen are toxic. The urea cycle processes nitrogen and facilitates its excretion from the body.

Urea cycle

The urea cycle    is a set of biochemical reactions that produces urea from ammonium ions in order to prevent a toxic level of ammonium in the body. It occurs primarily in the liver and, to a lesser extent, in the kidney. Prior to the urea cycle, ammonium ions are produced from the breakdown of amino acids. In these reactions, an amine group, or ammonium ion, from the amino acid is exchanged with a keto group on another molecule. This transamination    event creates a molecule that is necessary for the Krebs cycle and an ammonium ion that enters into the urea cycle to be eliminated.

In the urea cycle, ammonium is combined with CO 2 , resulting in urea and water. The urea is eliminated through the kidneys in the urine ( [link] ).

Urea cycle

This image shows the reactions of the urea cycle and the organelles in which they take place.
Nitrogen is transaminated, creating ammonia and intermediates of the Krebs cycle. Ammonia is processed in the urea cycle to produce urea that is eliminated through the kidneys.

Amino acids can also be used as a source of energy, especially in times of starvation. Because the processing of amino acids results in the creation of metabolic intermediates, including pyruvate, acetyl CoA, acetoacyl CoA, oxaloacetate, and α-ketoglutarate, amino acids can serve as a source of energy production through the Krebs cycle ( [link] ). [link] summarizes the pathways of catabolism and anabolism for carbohydrates, lipids, and proteins.

Energy from amino acids

This figure  shows the different reactions in which products of carbohydrate breakdown are converted into different amino acids.
Amino acids can be broken down into precursors for glycolysis or the Krebs cycle. Amino acids (in bold) can enter the cycle through more than one pathway.

Catabolic and anabolic pathways

This diagram shows the different metabolic pathways, and how they are connected.
Nutrients follow a complex pathway from ingestion through anabolism and catabolism to energy production.

Disorders of the...

Metabolism: pyruvate dehydrogenase complex deficiency and phenylketonuria

Pyruvate dehydrogenase complex deficiency (PDCD) and phenylketonuria (PKU) are genetic disorders. Pyruvate dehydrogenase is the enzyme that converts pyruvate into acetyl CoA, the molecule necessary to begin the Krebs cycle to produce ATP. With low levels of the pyruvate dehydrogenase complex (PDC), the rate of cycling through the Krebs cycle is dramatically reduced. This results in a decrease in the total amount of energy that is produced by the cells of the body. PDC deficiency results in a neurodegenerative disease that ranges in severity, depending on the levels of the PDC enzyme. It may cause developmental defects, muscle spasms, and death. Treatments can include diet modification, vitamin supplementation, and gene therapy; however, damage to the central nervous system usually cannot be reversed.

PKU affects about 1 in every 15,000 births in the United States. People afflicted with PKU lack sufficient activity of the enzyme phenylalanine hydroxylase and are therefore unable to break down phenylalanine into tyrosine adequately. Because of this, levels of phenylalanine rise to toxic levels in the body, which results in damage to the central nervous system and brain. Symptoms include delayed neurological development, hyperactivity, mental retardation, seizures, skin rash, tremors, and uncontrolled movements of the arms and legs. Pregnant women with PKU are at a high risk for exposing the fetus to too much phenylalanine, which can cross the placenta and affect fetal development. Babies exposed to excess phenylalanine in utero may present with heart defects, physical and/or mental retardation, and microcephaly. Every infant in the United States and Canada is tested at birth to determine whether PKU is present. The earlier a modified diet is begun, the less severe the symptoms will be. The person must closely follow a strict diet that is low in phenylalanine to avoid symptoms and damage. Phenylalanine is found in high concentrations in artificial sweeteners, including aspartame. Therefore, these sweeteners must be avoided. Some animal products and certain starches are also high in phenylalanine, and intake of these foods should be carefully monitored.

Chapter review

Digestion of proteins begins in the stomach, where HCl and pepsin begin the process of breaking down proteins into their constituent amino acids. As the chyme enters the small intestine, it mixes with bicarbonate and digestive enzymes. The bicarbonate neutralizes the acidic HCl, and the digestive enzymes break down the proteins into smaller peptides and amino acids. Digestive hormones secretin and CCK are released from the small intestine to aid in digestive processes, and digestive proenzymes are released from the pancreas (trypsinogen and chymotrypsinogen). Enterokinase, an enzyme located in the wall of the small intestine, activates trypsin, which in turn activates chymotrypsin. These enzymes liberate the individual amino acids that are then transported via sodium-amino acid transporters across the intestinal wall into the cell. The amino acids are then transported into the bloodstream for dispersal to the liver and cells throughout the body to be used to create new proteins. When in excess, the amino acids are processed and stored as glucose or ketones. The nitrogen waste that is liberated in this process is converted to urea in the urea acid cycle and eliminated in the urine. In times of starvation, amino acids can be used as an energy source and processed through the Krebs cycle.

Questions & Answers

what is anatomy
Oyindarmola Reply
Anatomy is the identification and description of the structures of living things
Kamara
what's the difference between anatomy and physiology
Oyerinde Reply
Anatomy is the study of the structure of the body, while physiology is the study of the function of the body. Anatomy looks at the body's organs and systems, while physiology looks at how those organs and systems work together to keep the body functioning.
AI-Robot
what is enzymes all about?
Mohammed Reply
Enzymes are proteins that help speed up chemical reactions in our bodies. Enzymes are essential for digestion, liver function and much more. Too much or too little of a certain enzyme can cause health problems
Kamara
yes
Prince
how does the stomach protect itself from the damaging effects of HCl
Wulku Reply
little girl okay how does the stomach protect itself from the damaging effect of HCL
Wulku
it is because of the enzyme that the stomach produce that help the stomach from the damaging effect of HCL
Kamara
function of digestive system
Ali Reply
function of digestive
Ali
the diagram of the lungs
Adaeze Reply
what is the normal body temperature
Diya Reply
37 degrees selcius
Xolo
37°c
Stephanie
please why 37 degree selcius normal temperature
Mark
36.5
Simon
37°c
Iyogho
the normal temperature is 37°c or 98.6 °Fahrenheit is important for maintaining the homeostasis in the body the body regular this temperature through the process called thermoregulation which involves brain skin muscle and other organ working together to maintain stable internal temperature
Stephanie
37A c
Wulku
what is anaemia
Diya Reply
anaemia is the decrease in RBC count hemoglobin count and PVC count
Eniola
what is the pH of the vagina
Diya Reply
how does Lysin attack pathogens
Diya
acid
Mary
I information on anatomy position and digestive system and there enzyme
Elisha Reply
anatomy of the female external genitalia
Muhammad Reply
Organ Systems Of The Human Body (Continued) Organ Systems Of The Human Body (Continued)
Theophilus Reply
what's lochia albra
Kizito
what are the layers of the skin
Helen Reply
It is made up of three layers, the epidermis, dermis, and the hypodermis, all three of which vary significantly in their anatomy and function. The skin's structure is made up of an intricate network which serves as the body's initial barrier against pathogens, UV light, and chemicals, and mechanical
Omer
what is diabetes?
Ifeoluwa
Diabetes is a chronic (long-lasting) health condition that affects how your body turns food into energy. Your body breaks down most of the food you eat into sugar (glucose) and releases it into your bloodstream. When your blood sugar goes up, it signals your pancreas to release insulin. Insulin act
Omer
what is gastric lavage and their implications
Ifeoluwa
what is velium
chizzy
what is a purpose
chizzy
what's fibroid
Kizito
what are disorders of connective tissue
Ester Reply
Rheumatoid arthritis (RA) Scleroderma. Granulomatosis with polyangiitis (GPA) Churg-Strauss syndrome. Lupus. Microscopic polyangiitis. Polymyositis/dermatomyositis. Marfan syndrome.
Omer
arthritis vasculitis
Enitan
what is cardiac output
Okoye Reply
(CO) amount of blood pumped by each ventricle during one minute; equals HR multiplied by SV
AI-Robot
what is SV and HR stand for
David
SV- Stroke Volume HR- Heart Rate
Ebelechukwu
Cardiac output is the amount of blood pumped by the heart in one minute. It's calculated by multiplying the heart rate (the number of times the heart beats in one minute) by the stroke volume (the amount of blood pumped out by the heart with each beat). So, cardiac output = heart rate x stroke volum
Dickson

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Source:  OpenStax, Anatomy & Physiology. OpenStax CNX. Feb 04, 2016 Download for free at http://legacy.cnx.org/content/col11496/1.8
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