About PKU and amino acids


 

 

 

 

(Q AM-06) H.C, a 4 month-old male infant, emigrated with his parents from a Latin American country to the United States a month ago. He was normal at birth but in the past several days a tremor in his extremities has appeared. Last night he presented gross twitching movements in his crib. When you examine the patient, you note a musty odor to the baby’s wet diaper.

 

Immediately you order a screening test for PKU and it results positive.

 

Besides diet restrictions, but warranting an appropriate amount of the essential amino acids, which of the following amino acids you should supplement in higher quantities than in a normal diet, since it is essential for this patient?

 

a)     Alanine

 

b)     Asparagine

 

c)      Aspartate

 

d)     Glutamate

 

e)     Phenyl alanine

 

f)       Serine

 

g)     Tyrosine

 

Answer

 

     

A: About Protein Classification


 

Original Question

 

Answer: (e) a complete protein.

 

Explanation:

 

Proteins can be classified according to different criteria. There are very complex ways of doing it. Anyway, simple and common ways of classifying proteins in Biochemistry textbooks are the following:

 

1.- Based on the shape or role of the proteins:

 

a)   Globular proteins (spheroproteins):

 

These proteins do not aggregate. Major backbone conformation include the helix, the strand, and the turn. These proteins do metabolic work: catalysis, transport, regulation, protection…These functions require solubility in blood and other aqueous media of cells and tissues. All globular proteins are constructed with a defined interior and exterior. In aqueous solutions, hydrophobic amino acids usually are in the interior of the globular proteins, while hydrophilic ones are interacting with water.

Ex. Myoglobin and Hemoglobin.

 

b)   Fibrous proteins (scleroproteins) :

 

These proteins are water insoluble proteins with elongated shapes.  

Aggregate tightly into fibers or sheet. Most play a structural and/or mechanical role. Tend to form elongated, curvilinear structures of high regularity. The regular structure comes from high primary sequence regularity.  Usually present modified amino acids. Ex. keratin and collagen.

 

2.- Based on the composition:

 

a)     Simple Proteins: Formed only by amino acids linked through peptide bonds (polypeptide chains)

 

b)     Conjugate proteins: Formed by amino acids and a different compound.

In conjugate proteins, the polypeptide portion is called apoprotein and the nonpolypeptide moiety is called prosthetic groups. According to the kind of prosthetic group, conjugates proteins can be nucleoproteins, glycoproteins, hemoproteins, flavoproteins, etc.

 

3.- According to the Nutritional Value, proteins can be classified as:

 

a)   Complete: A protein that contains all the essential amino acids. Generally comes from animals.

 

b)   Incomplete: A protein insufficient in one or more essential amino acids. Generally comes from plants.

 

The only information in the question that allows us to classify the protein X is the fact that it has all the essential amino acids. It allow us to classify it as a complete protein (the lack of some non essential amino acid is not significant from the nutritional point of view, since we can synthesize non essential amino acids)

 

We lack information about the conformation of the protein, its function,  so we can not classify it as a fibrous or globular protein, a simple or conjugate protein, or according to its function.

 

Is it possible to follow a vegetarian diet and obtain all the essential amino acids in the diet?

 

Yes, it is possible. Besides the fact that Soy protein in fact contains the essential amino acids, the combination of incomplete proteins in the diet allow to compensate for the lack of specific amino acids in any of them, so if we combine two proteins with low nutritional value, we can obtain a mixture with a higher value that the two original proteins.This is called the  supplementary action of the proteins.

 

In other words, if Protein A lacks lysine and protein B lacks methionine, the combination of Proteins A+B will have a higher nutritional value than the two separate proteins. Nowadays it is considered that it is not necessary to combine incomplete proteins in the same meal.

 

 

More information:

 

http://en.wikipedia.org/wiki/Complete_protein

 

http://www.nlm.nih.gov/medlineplus/ency/article/002467.htm

 

http://www.ivu.org/faq/protein.html

 

MedEdPORTAL announces Web Conferences for July and August


 

MedEdPORTAL, a program of the Association of American Medical Colleges, has announced the schedule of the free online training workshops for July and August.  

 

Since I had already a positive experience participating in a recent MedEdPORTAL web workshop, I would like to share this information with you:

 

“The web conference is free to US and Canadian participants (international participants will be provided with a toll-free number and a toll number and may use either number to connect to the audio portion of the workshop).

The training will consist of an overview of the MedEdPORTAL system and provide examples of the various types of materials published and available for free to educators and students around the globe.  

Participants will learn about the submission and peer review processes as well as the criteria used to evaluate the scholarship of published resources.

The seminar will also address issues associated with intellectual property and explain how MedEdPORTAL staff helps potential authors address those issues.  Participant interactivity is encouraged by providing the ability to ask questions using a chat mechanism and embedded live polling throughout the entirety of the presentation. Questions will be answered live at the conclusion in the order of which they were received.

The 2008 MedEdPORTAL online training workshops are scheduled to take place on:

·         July 11, 2008 at 3:00 PM North American Eastern Daylight Time (EDT): Registration Open

·         July 14, 2008 at 4:00 PM North American Eastern Daylight Time (EDT): Registration Open

·         August 4, 2008 at 3:00 PM North American Eastern Daylight Time (EDT): Registration will open in July/August

·         August 14, 2008 at 1:00 PM North American Eastern Daylight Time (EDT): Registration will open in August

 

 

 

More information about these workshops can be found at:

 

http://services.aamc.org/jsp/mededportal/goLinkPage.do?link=lastUpdate#OTW