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Amino Acids
Comp Exam Study guide
37
Biochemistry
Graduate
09/06/2012

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Term
Tryptophan
Definition
IF high-supplement B6, niacin, iron,and copper-because of excessive intake or poor metabolic utilization IF low-supplement 5-HTP
Term
alpha-Amino-N-Butyric Acid
Definition
ratio of this amino acid/leucine is a marker for alcoholism. Elevations of this amino acid are seen in alcoholics. also, elevated plasma indicates vitamin B6 insufficient. SO if levels are low- supplement with vitamin B6 and alpha-ketoglutarate
Term
Gamma-Aminobutyric Acid (GABA)
Definition
Inhibitory neurotramsimitter in the CNS. Thought to be involved in epilepsy. It is produced from glutamate in a single decarboxylation reaction Elevated levels-vitamin B6 and alpha-ketoglutarate supplementation
Term
METHIONENE
Definition
Essential precursor in sulfur amino acid metabolism. Low levels indicate poor-quality protein diet. This amino acid's metabolism pathways are dependent on vit B6, B12, and folate. High levels: vit b6, Alpha-ketoglutarate, and magnesium used High levels of this/low homocysteine-SAM supplementation Low levels-balanced free form amino acid mixture
Term
HOMOCYSTEINE
Definition
If high-vit b6, folate, b12, betaine Low levels of this amino acid are due to enzyme deficiency: supplement with vit C Low levels in response to oxidative stress: N-acetylcysteine and taurine
Term
CYSTATHIONE
Definition
high levels: vitamin B6
Term
TAURINE
Definition

Low levels: Vitamin B6

High level: vitamin E, vitamin C, beta-carotene, coQ10, lipoic acid  It is a sulfur-containing beta-amino acid, beta-aminoethylsulfonic acid. It has antioxidant role in various tissues Urinary elevation of this amino acid is seen in zinc deficiency

Term

SARCOSINE

(N-methylglycine)

Definition
Serves to buffer the methyl group or single carbon supply system. Elevations are a sign of exacerbation of methylation difficulties due to blockage in release of homocysteine. If high supplement with B2 (riboflavin) Genetic defects in a key enzyme can prevent the transfer of methyl group resulting in elevated levels of this amino acid
Term
ALANINE
Definition
The major carrier of amino acid nitrogen from muscle to liver wehre its carbon skeleton is converted to glucose.(via gluconeogenesis)Its enzyme ALT is dependent on vit B6 and is one of the most abundant enzymes in hepatic tissue. Plasma levels of this enzyme is the primary marker for liver disease. HIgh levels of this amino acid requre supplment with vit B6. Low levels in plasma can warrant supplementation with the actual amino acid
Term
ETHANOLAMINE
Definition
High due to environmental exposure and physiologicla effects. Supplement with magnesium.
Term
PHOSPHOETHANOLAMINE
Definition
Is high in patients who have breast tumor growth. Supplement with SAMe, vitamin B12, and betaine.
Term
PHOSPHOSERINE
Definition
SUPPLEMENT WITH MAGNESIUM WHEN HIGH. HIGH PLASMA LEVLES ARE SEEN IN PARKINSON'S
Term
PROLINE
Definition
• Major component of collagen and can be metabolized to alpha-KG
• Required for protein synthesis
• Low levels indicate: low tissue supply and should be supplemented with alpha-KG and histidine
• High levels: supplement with niacin and vitamin C
Term
HYDROXYPROLINE
Definition
High levels are characteristic of high bone turnover. It is highly correclated with serum alkaline phosphatase which is also a marker for bone resorption. Sythesized from proline using vitamin C and iron. Supplement with vitamin C and iron when levels are high.
Term
HYDROXYLYSINE
Definition
a marker for bone loss due to the turnover of collagen during bone resorption. Indicator for liver disease. Urine levels provides a marker of response to GH-deficient children. high plasma levels indicate connective tissue breakdown. Supplement with iron, alpha-KG, vit C, sulfate, and manganese
Term
β-ALANINE
Definition
• Vitamin B6 deficiency causes elevated urinary levels
• High levels are due to loss of other amino acids like taurine
• If high supplement with lactobacillus and bifodobacteria sp. And vit B6
• Candida albicans can also elevate β-alanine levels
Term
β-AMINOISOBUTYRIC ACID
Definition
• High in cases of leuekemia, and x-ray radiation-induced destruction of DNA and RNA
• If high supplement with vit B6
Term
GLUTAMIC ACID
Definition
High result supplement with alpha-KG
Term
GLUTAMINE
Definition
High levels supplement with alpha-KG and Vit B6
Term
ARGININE
Definition
If high supplement Mn, l-lysine supplementation and if low supplement with the actual amino acid Has a role in cell regulation via production of nitric oxide and polyamines such as spermine
Term
ASSYMETRIC DIMETHYLARGININE (ADMA)
Definition
is formed by methylation of Arginine residues of nuclear proteins If levels are high supplement with Arginine
Term
CITRULLINE (Cit
Definition
High plasma or urinary levels can indicate a functional block in the argininosuccinate synthetase enzyme, leading to a buildup of excess ammonia in the system.
Supplementation with magnesium and aspartic acid may help to decrease ammonia buildup.

Arginine free diet may be helpful. Arginine loading results in increased citrulline values.
Term
ORNITHINE (Orn)
Definition
Patients with these inherited traits may show elevated plasma glutamine, proline, lysine, valine, and methionine, but usually this particular amino acid is normal. Supplement with the amino acid will help to achieve lower ammonia.
Alpha-ketoglutaric acid, vitamin B6, and magnesium are useful in clearing chronic excess ammonia.
Term
ASPARTIC ACID (Asp)
Definition
aids in the detoxification of ammonia via the urea cycle. Magnesium and zinc are also relevant cofactors If high supplement with Magnesium and Zinc. If low supplement with alpha-KG
Term
ASPARAGINE (Asn)
Definition
needed in all tissues for protein synthesis and it serves a special function for attachment of CHO residues to membrane proteins by which cells are identified. Magnesium used as supplementation when it is low and alpha-KG and vitamin B6 used when it is high.
Term
THREONINE (Thr)
Definition
Dietary supply of protein is critical for protein synthesis and tissue maintenance. If low supplement with vitamin E, C, beta-carotene, coQ10, lipoic acid. If high supplement with B6
Term
ISOLEUCINE
Definition
If high supplement with B6 and check for insulin insenstivity
Term
LEUCINE
Definition
If high supplement with vitamin B6 and if low supplement with vit E,C,beta-carotone, coQ10, lipoic acid
Term
VALINE
Definition
Low levels supplement with vitamin E, C, beta-carotene, coQ10, lipoic acid and high levels supplement with vit b6
Term
HISTIDINE
Definition
if low supplement with folate and high levels supplement with B6
Term
Lysine
Definition
If low supplement with carninite, check for exciessive arg intake. High levels supplement with vit C, niacin, b6, iron, supplementation will decrease arginine excessive amounts of this amino acid will contribute to kidney pathology
Term

Alpha-aminoadipic acid(α-AAA)

Definition
high levels supplement with alpha-KG and vit B6
Term
PHENYLAlANINE
Definition
precursors to DOPA, melanin and thyroid hormones. PKU is a consequence of insufficient activity of phenylalanine hydroxylase (PH) high levels supplement with vit C, niacin, iron, or BH4 and low phenylalanine diet. Low levels supplement with antioxidants like Vit E, coq10, lipoic acid, beta-carotene
Term
Tyrosine
Definition
If high supplement with copper, iron, and vit . If low supplement with iron and the amino acid
Term
what two cofactors are involved in converting dopamine to norepinephrine?
Definition
Copper and Ascorbate (vit C)
Term
WHAT COFACTOR IS NEEDED TO CONVERT NOREPINEPHRINE INTO EPINEPHRINE?
Definition
SAMe
Term
UNDER WHAT CONDITIONS WOULD GLY SUPPLY LIMIT GLUTATHIONE PRODUCTION?
Definition
Individuals exprimentally stressed with benzoate show inhibition of glutathione syntehsis and restriction of dietary protien.
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