Busy. Please wait.
Log in with Clever

show password
Forgot Password?

Don't have an account?  Sign up 
Sign up using Clever

Username is available taken
show password

Make sure to remember your password. If you forget it there is no way for StudyStack to send you a reset link. You would need to create a new account.
Your email address is only used to allow you to reset your password. See our Privacy Policy and Terms of Service.

Already a StudyStack user? Log In

Reset Password
Enter the associated with your account, and we'll email you a link to reset your password.
Didn't know it?
click below
Knew it?
click below
Don't Know
Remaining cards (0)
Embed Code - If you would like this activity on your web page, copy the script below and paste it into your web page.

  Normal Size     Small Size show me how

Block-6 Amino Disord

Block-6 Amino Metabolism Deficiency

To what are amino acids converted when we are in the well-fed state? alpha-keto acids which go on to glycolysis, gluconeogenesis, and TCA
If an amino acid is converted to glucose it is called? If metabolized to acetyl-Co-A? Glucogenic Ketogenic
What 2 amino acids are exclusively Ketogenic? Lysine and Leucine
Two general symptoms of amino acid metabolism? retardation and failure to grow
Acetyl-CoA is converted into? Fatty Acid, used in TCA, Steriods/cholesterol
All newborns are screened for what amino acid disorder? PKU - phenylalanine Hydroxylase Deficiency
What physical traits and mental traits develop in PKU? Why? pale skin, blond hair, mental retardation, autism, loss motor control due to high levels of phenylalanine
What is the problem in PKU? deficient in phenyalanine hydroxylase to convert phenylalanine to tyrosine
Tyrosine is converted to another compound which can cause problems if not metabolised properly? Homogentisic Acid needs to be converted to maleyacetoacte by homogentisate oxidase (HO)
What symptoms appear with homogentisate Oxidase Deficiency? Name of disorder? Alcaptonuria, dark urine due to oxygenation when exposed to oxygen, dark pigment at joints (ochrononis)
Another disease (not PKU) is also screened for at birth. What are the enzyme responsible for it? Maple syrup urine disease, Isoleucine, Leucine, Valine (I Love Vermont)
What is the missing enzyme? problem if untreated? Branched-chain ketoacid dehydrogenase. If untreated infants become lethargic, fail to grow, Ketoacidosis: coma and death.
What amino acid is required as a cofactor for Branched-chain ketoacid d'hase? thiamine
What happens with proprionyl-CoA and Methymalonyl-CoA Mutase Deficiency? Like MSUD - results in neonatal keto, acidosis
What enzymes must be restricted in MSUD and proprionyl-CoA and Methymalonyl-CoA mutase defi... valine, methionine, isoluecine, threonine
What happens with a break down of cystathionine synthase? DVT, cardiovascular disease, homocysteine is highly reactive, binds LDL to form cholesterol fatty streaks/foam cells - die by age 25 of MI
Created by: El Diablo
Popular Biochemistry sets




Use these flashcards to help memorize information. Look at the large card and try to recall what is on the other side. Then click the card to flip it. If you knew the answer, click the green Know box. Otherwise, click the red Don't know box.

When you've placed seven or more cards in the Don't know box, click "retry" to try those cards again.

If you've accidentally put the card in the wrong box, just click on the card to take it out of the box.

You can also use your keyboard to move the cards as follows:

If you are logged in to your account, this website will remember which cards you know and don't know so that they are in the same box the next time you log in.

When you need a break, try one of the other activities listed below the flashcards like Matching, Snowman, or Hungry Bug. Although it may feel like you're playing a game, your brain is still making more connections with the information to help you out.

To see how well you know the information, try the Quiz or Test activity.

Pass complete!
"Know" box contains:
Time elapsed:
restart all cards