Amino Acid Profile, Qn, Plasma (LabCorp)

 

Diagnosis and monitoring of inherited aminoacidurias, organic acidurias, and urea cycle defects. May be used as a follow-up confirmatory test to some abnormal newborn screen results.

False-negative results can occur, especially when a patient is affected with a mild or intermittent variant of a disorder or when clinical condition or dietary treatment causes secondary changes to the profile. Results of amino acid profiling should be interpreted in the context of clinical presentation, as well as other laboratory tests. Frozen serum may be diagnostic, however, certain amino acids may be falsely elevated or low.

Alanine

Optimal range: 209.2 - 515.5 umol/L

Alanine is a non-essential amino acid and helps the body convert the simple sugar glucose into energy and eliminate excess toxins from the liver.

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Alloisoleucine

Optimal range: 0 - 3.2 umol/L

Allo-isoleucine is nearly undetectable in individuals not affected by maple-syrup urine disease (MSUD). Accordingly, its presence is diagnostic for MSUD, and its absence is sufficient to rule-out MSUD.

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Alpha-aminoadipate

Optimal range: 0 - 1.9 umol/L

Alpha-aminoadipic acid (a-Aminoadipic acid) is an intermediary metabolite of lysine (primarily) and of tryptophan.

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Alpha-aminobutyrate

Optimal range: 5.4 - 34.5 umol/L

Alpha amniobutyric acid (AABA), also known as Alpha-amino-N-butyric acid (A-ANB), is an intermediate formed during the catabolism of methionine and threonine. Increases in AABA occur secondary to elevations of either methionine or threonine. AABA becomes propionic acid via alpha-ketobutyric acid in the presence of adequate amounts of thiamin, vitamin B2(as FAD), vitamin B3(as NAD), lipoic acid and magnesium. Deficiencies of any of these, or vitamin B6, could cause increases in AABA. Elevated or decreased levels of the amino acid may indicate a congenital enzyme defect.

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Arginine

Optimal range: 36.3 - 119.2 umol/L

Arginine is a conditionally essential amino acid that is critical for your cardiovascular health and detoxification functions. The amino acid, arginine, is used to make the powerful blood vessel regulator, nitric oxide. Nitric oxide acts to lower blood pressure.

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Argininosuccinate

Optimal range: 0 - 3 umol/L

Argininosuccinate (aka Arginosuccinic acid) is a basic amino acid. Some cells synthesize it from citrulline, aspartic acid and use it as a precursor for arginine in the urea cycle or Citrulline-NO cycle. The enzyme that catalyzes the reaction is argininosuccinate synthetase. Argininosuccinic acid is a precursor to fumarate in the citric acid cycle via argininosuccinate lyase. Defects in the argininosuccinate lyase enzyme can lead to argininosuccinate lyase deficiency, which is an inborn error of metabolism.

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Asparagine

Optimal range: 29.5 - 84.5 umol/L

Asparagine is a protein amino acid. It is non-essential in humans, meaning the body can synthesize it.

Asparagine is synthesized from aspartate and glutamine. Asparagine has three major functions:

  1. incorporation into amino acid sequences of proteins
  2. storage form for aspartate (is a required precursor for synthesis of DNA, RNA and ATP)
  3. source of amino groups for production of other dispensable amino acids via Transaminases.

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Aspartate

Optimal range: 0 - 7.4 umol/L

Beta-alanine

Optimal range: 1.1 - 9 umol/L

Beta-alanine is is a non-essential amino acid.

What are amino acids?

Non-essential amino acids can be made by the body, so they don’t have to be provided by food. Amino acids are the building blocks of proteins.

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Beta-aminoisobutyrate

Optimal range: 0 - 4.3 umol/L

Beta-aminoisobutyric acid (BAIB) is an amino acid end product of the pyrimidine metabolism. It is excreted in small quantities into the urine in almost all human beings. Thymine, released when RNA and DNA are degraded, enters a catabolic pathway that leads to Beta-Aminoisobutyric Acid.

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Citrulline

Optimal range: 15.6 - 46.9 umol/L

The amino acid citrulline gets its name from its high concentration in the watermelon Citrullus vulgaris. In human kidneys, citrulline and aspartic acid are united by argininosuccinate synthetase (ASS) to produce arginosuccinate. The degradation of arginosuccinate to fumarate and arginine is a primary mechanism for sustaining plasma levels of arginine. The same enzyme acts in liver cells to complete the urea cycle.

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Cystathionine

Optimal range: 0 - 0.7 umol/L

Cystathionine is an intermediary metabolite that is formed in the sequential enzymatic conversion of methionine to cysteine. Cystathionine is normally detected at very low levels in plasma. It is found between homocysteine and cysteine and is formed by the enzyme cystathionine beta-synthase (CBS).

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Cystine

Optimal range: 15.8 - 47.3 umol/L

Cystine is the oxidized disulfide form of cysteine (Cys) and is the predominant form of cysteine in the blood due to its greater relative stability. Cystine is derived from dietary protein and, end formed endogenously from cysteine.

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Gamma-aminobutyrate

Optimal range: 0 - 0.6 umol/L

Gamma-aminobutyrate (GABA) is the major inhibitory neurotransmitter found in the CNS and, as such, is important for balancing excitatory action of other neurotransmitters.

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Glutamate

Optimal range: 18.1 - 155.9 umol/L

Glutamate functions as the major excitatory neurotransmitter and metabolic fuel throughout the body. Glutamate is produced in your body, and is also found in many foods.

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Glutamine

Optimal range: 372.8 - 701.4 umol/L

Glutamine is the most abundant amino acid in the blood and is an important source of energy for many tissues in the body. It is derived from the amino acids histidine and glutamic acid.

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Glycine

Optimal range: 144 - 411 umol/L

Glycine is an amino acid with various important functions within your body, including detoxification, DNA formation, the synthesis of hemoglobin, and as a part of brain neurotransmission pathways. Glycine and serine are interchangeable.

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Histidine

Optimal range: 47.2 - 98.5 umol/L

Histidine is the amino acid most necessary during stress. Amino acids are the building blocks of protein in our bodies.

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Homocitrulline

Optimal range: 0 - 1.7 umol/L

The amino acid homocitrulline is a metabolite of ornithine in human metabolism.

The amino acid can be detected in larger amounts in the urine of individuals with urea cycle disorders. Both amino acids can be detected in urine. Amino acid analysis allows for the quantitative analysis of these amino acid metabolites in biological fluids such as urine, blood, plasma or proteins.

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Homocystine

Optimal range: 0 - 0.2 umol/L

Hydroxylysine

Optimal range: 0.1 - 0.8 umol/L

Hydroxylysine is an amino acid related to collagen.

What is collagen?

Collagen is the most abundant protein in your body. It is the major component of connective tissues that make up several body parts, including tendons, ligaments, skin and muscles.

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Hydroxyproline

Optimal range: 4.7 - 35.2 umol/L

Hydroxyproline is a collagen related amino acid. Hydroxyproline is a nonessential amino acid, which means that it is manufactured from other amino acids in the liver. Hydroxyproline is necessary for the construction of the body’s major structural protein, collagen. Hydroxyproline is present in essentially all tissues and all genetic types of collagen.

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Isoleucine

Optimal range: 32.8 - 88.3 umol/L

Isoleucine is one of the three branched-chain amino acids (BCAAs) alongside both leucine and valine.

Isoleucine is a common component of proteins, peptides and hormones. Leucine is catabolized as a source of carbon for energy production during exercise in skeletal muscle.

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Leucine

Optimal range: 66.7 - 165.7 umol/L

Leucine, together with isoleucine and valine, are essential amino acids that are referred to as branched-chain amino acids (BCAAs).

Leucine is nutritionally essential and is required for formation of body proteins, enzymes and some hormones. Leucine itself has a hormone-like activity which is stimulation of pancreatic release of insulin. The branched-chain structure of leucine makes it very important for the formation of flexible collagen tissues, particularly elastin in ligaments. Leucine is relatively abundant in all protein foods.

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Lysine

Optimal range: 94 - 278 umol/L

Lysine is found in great quantities in muscle tissues, stimulates calcium absorption, carnitine synthesis, and growth and repair of muscle tissue.

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Methionine

Optimal range: 14.7 - 35.2 umol/L

Methionine is an essential amino acid, meaning we need to get it from our diet as our body does not produce it. Methionine is a unique sulfur-containing amino acid that can be used to build proteins and produce many molecules in the body.

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Ornithine

Optimal range: 30.1 - 101.3 umol/L

Ornithine is a urea cycle metabolite.

Ornithine can stimulate the release of growth hormone. Growth hormone is necessary for tissue repair and growth. Growth hormone is often low in patients with fibromyalgia.

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Phenylalanine

Optimal range: 35.8 - 76.9 umol/L

Phenylalanine is a precursor for the amino acid tyrosine, which is essential for making neurotransmitters (e.g. epinephrinenorepinephrinedopamine) and thyroid hormone. Neurotransmitters are the chemicals that communicate between nerve cells in the brain. It can relieve pain, alleviate depression, and suppress the appetite. Low levels may indicate a stressful lifestyle, leading to memory loss, fatigue, and depression.

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Proline

Optimal range: 84.8 - 352.5 umol/L

Proline is a nonessential amino acid, which means that it is manufactured from other amino acids in the liver; it does not have to be obtained directly through the diet.

Proline is the precursor to hydroxyproline, which is a major amino acid found in the connective tissue of the body – collagen.

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Sarcosine

Optimal range: 0 - 4 umol/L

Sarcosine is also known as N-methylglycine. It is an intermediate and byproduct in the glycine synthesis and degradation. Sarcosine is metabolized to glycine by the enzyme sarcosine dehydrogenase, while glycine-N-methyl transferase generates sarcosine from glycine.

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Serine

Optimal range: 48.7 - 145.2 umol/L

Serine can be used as an energy source. Formed from threonine and phosphoserine (requiring B6, manganese, and magnesium), serine is necessary for the biosynthesis of acetylcholine, a neurotransmitter used in memory function.

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Taurine

Optimal range: 29.2 - 132.3 umol/L

Taurine is a sulfur-containing amino acid required for bile formation.

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Threonine

Optimal range: 67.8 - 211.6 umol/L

Threonine is an essential amino acid, i.e., it is vital for your health, but it cannot be synthesized by your body and therefore has to be obtained from a diet.

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Tryptophan

Optimal range: 23.5 - 93 umol/L

Tryptophan is an essential amino acid required for the production of the neurotransmitter serotonin.

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Tyrosine

Optimal range: 27.8 - 83.3 umol/L

Tyrosin is the non-essential amino acid precursor for dopamine, norepinephrine and epinephrine. Tyrosine hydroxylase converts tyrosine into the dopamine precursor L-DOPA; BH4, Vitamin D and iron are cofactors for that enzymatic activity.

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Valine

Optimal range: 133 - 317.1 umol/L

Valine, together with Isoleucine and Leucine are essential amino acids and are collectively referred to as branched-chain amino acids (BCAAs).

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