Term
What are the Essential A.As? |
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Definition
PVT TIM HALL: Phe, Val, Thr; Trp, Iso, Met; His, Arg, Lys, Leu |
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Term
When in starvation what organs get the most amount of energy? |
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Definition
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Term
Which a.a's becomes essential if one of the 10 normally essential a.a's are lacking? |
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Definition
Tyrosine if Phe is lacking and Cys if Met is lacking |
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Term
Phenylketonuria is deficient in which enzyme? |
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Definition
Phenylalanine hydroxylase |
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Term
Phenylketonuria is what kind of inheritance? |
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Definition
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Term
Why are Phenylketonuria pts retarded? |
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Definition
They lack Phe which is converted to make tyrosine which is inturn converted to make catecholamines (Dopamine, Epi, NE) |
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Term
How do Phenylketonuria pts look when they come into your office? |
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Definition
No tyrosine means no melanin therefore Blond, Blue eyes and fair skin |
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Term
What does the extra Phenylalanine combine to in Phenylketonuria pts? |
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Definition
Acetate and pyruvate to form Phenylacetate and Phenylpyruvate => Musty odor PEE |
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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? |
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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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