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9 Cards in this Set
- Front
- Back
- 3rd side (hint)
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Structure of Amino Acids |
Central Carbon atom 1 Hydrogen atom 1 R group on the opposite
1 COOH group, pH? 1 NH2 group, pH? |
Carboxyl group: Acidic
Amino group: basic |
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Pka of Amino acid side chains |
pH<Pka pH>Pka
Charge? Why? |
1. Net charge: +, More H+ 2. Net charge: -, More OH-, H+ combine with OH to form water |
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PI |
Isoelectric point
Net charge? Formula |
0
Pka 1+ Pka 2/2 |
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PI
pH<Pka pH>Pka
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pH<Pka: protein is acidic pH>Pka: protein is basic |
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Aminoacidopathies
What is it? |
Inherited enzyme defects that inhibit metabolism of certain amino acids
How many are mandated by NYS to test? |
3 |
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Aminoacidopathies
3 tests are mandated by NYS List |
PKU MSUD Homocystinuria |
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Aminoacidopathies
___ MSUD Homocystinuria |
PKU
Metabolic defects and Amino acids Common detection method |
Excessive phenylalanine High in pregnancy
Guthrie test MS/MS: tandem mass pecc |
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Aminoacidopathies
PKU ________ Homocystinuria |
MSUD
Full name Metabolic defects and amino acids Common detection method |
Maple surup urine disease
MS/MS of leucine, isoleucine and valine |
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Aminoacidopathies
PKU MSUD ________________ |
Homocystinuria
Cause Amino acid Test |
Cysthione B-synthase deficiency
Methione is tested because homocysteine is difficult to measure
MS/MS Modified Guthrie |
