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Amino Acid Degredation and Urea Cycle
Power point over amino acid degradation and urea cycle
51
Chemistry
Professional
09/17/2009

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Cards

Term
Amino acids contain what?
Definition
nitrogen
Term
What are the three stages aa are transfered into the urea cycle?
Definition
1. transamination
2. formation of amonnia
3. formation of carbamoyl phosphate
Term
What part gets removed for transamination?
Definition
H2N1
Term
What gets used in transamination?
Definition
r group, COOH, H
Term
Urea cycle takes place where?
Definition
Liver
Term
Amino acids give us what?
Definition
1. purines
2. pyrimidines
3. porphyrins
Term
What is pulled off while making urea?
Definition
nitrogen
Term
What is left over after nitrogen is pulled off?
Definition
carbon skeleton
Term
What are the ketogenic intermediates?
Definition
acetoacetate
acetyl CoA
Term
Where do we get essential amino acids from?
Definition
our diet
Term
What are the branched chains of amino acids?
Definition
Isoleucine, leucine, and valine,
a third of our muscle mass is from branched chain AA
Term
What forms non essential AA formed?
Definition
alpha keto acids by transamination
Term
What does transamination do?
Definition
permits biosynthesis, degradation and equilibrates amino groups among available alpha keto acids.
Term
What must be obtained in the diet as amino acids?
Definition
nitrogen
Term
What are the key players in transam.?
Definition
alphaketoglutarate and glutamate
Term
What are the enzymes involved in transam? and how many are there?
Definition
12 transaminases, one for each enzyme
Term
What coenzyme do you have to have for transamination?
Definition
Pyridoxal phosphate, B6
Term
What enzymes are useful for us medically everday?
Definition
ALT AST
Term
What is ALT also known as?
Definition
SGPT
Term
What is AST also known as?
Definition
SGOT
Term
What are AST and AST used for?
Definition
testing liver enzymes
Term
When we strip off the nitrogen of alanine it becomes what?
Definition
pyruvate
Term
When we add an amine group to alpha ketoglutarate we get what?
Definition
glutamate
Term
Aspartate donates its amino group and becomes what?
Definition
oxaloacetate
Term
AST is more/less sensitive?
Definition
more
Term
What is stage two?
Definition
Oxidative Deamination, going the other way it would be reductive amination
Term
What does stage two use?
Definition
NADP+
Term
What are we creating through oxidative deamination?
Definition
ammonia
Term
Where are the enzymes for the urea cycle contained?
Definition
liver
Term
Kidney has all urea cycle enzymes except what?
Definition
arginase
Term
Where are the five steps of step two?
Definition
Step 1 and 2= mitochondria
Step 3,4,5= Cytosol
Term
What is the committing step in Oxid. Deam?
Definition
Carbamoyl Phosphate Synthase I
Term
What activates the committing step enzyme?
Definition
N acetylglutamate
Term
When glutamate is high, you have a lot of what?
Definition
N acetylglutamate
Term
What is the disease when you have a lack of enzyme of any cycle?
Definition
Hyperammonemia
Term
What is the treatment for Hyperammonemia?
Definition
liver transplant, limit protein intake
Term
What is an enzyme used to diagnose liver or renal disease?
Definition
Blood Urea Nitrogen
Term
What is the normal range of BUN?
Definition
7-18 g/dL
Term
When is BUN elevated?
Definition
amino acid catabolism, renal insufficiency, Kidney disease
Term
When is BUN decreased?
Definition
Hepatic failure, Liver disease
Term
What makes up a carbon skeleton?
Definition
R group, COOH, and H
Term
What 7 intermediates are made from the carbon skeletons?
Definition
oxaloacetate,alpha-ketoglutarate, pyruvate,
fumarate, succinyl CoA, acetyl CoA, acetoacetyl
CoA
Term
Amino acids are degraded through what process?
Definition
transamination
Term
What is an important inhibitory neurotransmitter?
Definition
GABA
Term
What is a test that is helpful in determining kidney function?
Definition
Creatine Clearance test
Term
What can be made through transamination?
Definition
catocholamines, serotonin
Term
What is Phenylketonuria?
Definition
Term
What is maple syrup disease?
Definition
Term
What is albinism?
Definition
Term
What is homocystinuria?
Definition
Term
What is Alkaponuria?
Definition
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