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26 Cards in this Set
- Front
- Back
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proline is a precursor of ? |
hydroxyproline |
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-molecular recognition -works with vitamin C to promote healthy connective tissues |
proline |
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-second amino acid that is also an alcohol -myelin sheath component |
serine |
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-important for body's synthesis of pyrimidines, purines, creatinine & porphyrins |
serine |
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production of immunoglobulins & antibodies |
serine |
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-synthesized from phenylalanine -precursor of adrenal hormones |
tyrosine |
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-overall metabolism -mood elevator -suppresses appetite & reduces fats |
tyrosine |
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two new amino acids |
-selenocysteine -pyrrolysine |
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-21st member of amino acids -high conc in different body enzymes |
selenocysteine |
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-not coded for directly in genetic code --UGA (stop codon) |
selenocysteine |
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-selenium analogue of cysteine --same structure of cysteine but sulfur in side chain of cysteine is changed to selenium |
selenocysteine |
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-higher amount in HIV 1 --DEC selenium in blood due to HIV 1 using selenium in blood due to HIV 1 using selenium for selenocysteine formation |
selenocysteine |
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-22nd member of amino acids -same with lysine except that it contains pyrroline |
pyrrolysine |
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-encoded by UAG (stop codon) -used by archea (prokaryotes) |
pyrrolysine |
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classes of inherited errors of amino acid metabolism |
aminoacidopathies |
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-enzymes are defective that inhibits the body's ability to metabolize certain amino acids --due to: metabolic pathway, membrane transport system (ONLY in cystinuria) |
aminoacidopathies |
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genetically-inherited deficiency of phenylalanine hydroxylase (PAH) --converts phenylalanine to tyrosine |
phenylketonuria |
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what is the prime metabolite of phenylketonuria |
phenylpyruvic acid (PPA) |
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reference of serum phenylalanine: adult |
1.2-3.4 mg/dl or 70-200 umol/L |
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reference of serum phenylalanine: baby |
2 mg/dL or 120 umol/L |
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-first aminoacidopathy integrated to the newborn screening -clinical features are: mental retardation and microencephaly |
phenylketonuria |
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-musky odor of urine -more than 1200 umol/L phenylalanine in blood |
phenylketonuria |
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600-1200 umol/L phenylalanine in blood (mild) |
phenylketonuria |
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-180-600 umol/L --hyperphenylalanine ---BH4 deficiency ---aromatic hydroxylation |
phenylketonuria |
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-most well known test for phenylketonuria -B. subtilis is used WITH beta 2 thienylalanine |
guthrie test |
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positive result for guthrie test |
bacterial growth of 4 mg/dL |
