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Biochem
Essential AA plus More
9
Medical
Professional
07/30/2010

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Cards

Term
What are the Essential A.As?
Definition
PVT TIM HALL: Phe, Val, Thr; Trp, Iso, Met; His, Arg, Lys, Leu
Term
When in starvation what organs get the most amount of energy?
Definition
Brain, Heart and Kidney
Term
Which a.a's becomes essential if one of the 10 normally essential a.a's are lacking?
Definition
Tyrosine if Phe is lacking and Cys if Met is lacking
Term
Phenylketonuria is deficient in which enzyme?
Definition
Phenylalanine hydroxylase
Term
Phenylketonuria is what kind of inheritance?
Definition
Autosomal recessive
Term
Why are Phenylketonuria pts retarded?
Definition
They lack Phe which is converted to make tyrosine which is inturn converted to make catecholamines (Dopamine, Epi, NE)
Term
How do Phenylketonuria pts look when they come into your office?
Definition
No tyrosine means no melanin therefore Blond, Blue eyes and fair skin
Term
What does the extra Phenylalanine combine to in Phenylketonuria pts?
Definition
Acetate and pyruvate to form Phenylacetate and Phenylpyruvate => Musty odor PEE
Term
A pregnant mother is adviced by her doctor to stay off of aspartame (Aspartate and Phenylalanine) (nutrisweet - found in diet coke), give me two reasons why?
Definition
She might have PKU or be a carrier and thus child might have it. And since the child is only screened at birth, she needs to have that diet restriction for atleast the first 8 wks - when the brian is developing.
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