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Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (17)transporters (2) Show 19 proteins XML

enzymes (17)transporters (2) Show 19 proteins

Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2024-05-19 04:28:27 UTC

HMDB ID

HMDB0000159

Secondary Accession Numbers

HMDB0000612
HMDB00159
HMDB00612

Metabolite Identification

Common Name

Phenylalanine

Description

Phenylalanine (Phe), also known as L-phenylalanine is an alpha-amino acid. These are amino acids in which the amino group is attached to the carbon atom immediately adjacent to the carboxylate group (alpha carbon). Amino acids are organic compounds that contain amino (-NH2) and carboxyl (-COOH) functional groups, along with a side chain (R group) specific to each amino acid. L-phenylalanine is one of 20 proteinogenic amino acids, i.e., the amino acids used in the biosynthesis of proteins. Phenylalanine is found in all organisms ranging from bacteria to plants to animals. It is classified as an aromatic, non-polar amino acid. In humans, phenylalanine is an essential amino acid and the precursor of the amino acid tyrosine. Like tyrosine, phenylalanine is also a precursor for catecholamines including tyramine, dopamine, epinephrine, and norepinephrine. Catecholamines are neurotransmitters that act as adrenalin-like substances. Interestingly, several psychotropic drugs (mescaline, morphine, codeine, and papaverine) also have phenylalanine as a constituent. Phenylalanine is highly concentrated in the human brain and plasma. Normal metabolism of phenylalanine requires biopterin, iron, niacin, vitamin B6, copper, and vitamin C. An average adult ingests 5 g of phenylalanine per day and may optimally need up to 8 g daily. Phenylalanine is highly concentrated in a number of high protein foods, such as meat, cottage cheese, and wheat germ. An additional dietary source of phenylalanine is artificial sweeteners containing aspartame (a methyl ester of the aspartic acid/phenylalanine dipeptide). As a general rule, aspartame should be avoided by phenylketonurics and pregnant women. When present in sufficiently high levels (>500 uM), phenylalanine can act as a neurotoxin and a metabotoxin. A neurotoxin is a compound that disrupts or attacks neural cells and neural tissue. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of phenylalanine are associated with at least five inborn errors of metabolism, including Hartnup disorder, hyperphenylalaninemia due to guanosine triphosphate cyclohydrolase deficiency, phenylketonuria (PKU), tyrosinemia type 2 (or Richner-Hanhart syndrome), and tyrosinemia type III (TYRO3). Phenylketonurics have elevated serum plasma levels of phenylalanine up to 400 times normal. High plasma concentrations of phenylalanine influence the blood-brain barrier transport of large neutral amino acids. The high plasma phenylalanine concentrations increase phenylalanine entry into the brain and restrict the entry of other large neutral amino acids (PMID: 19191004 ). Phenylalanine has been found to interfere with different cerebral enzyme systems. Untreated phenylketonuria (PKU) can lead to intellectual disability, seizures, behavioural problems, and mental disorders. It may also result in a musty smell and lighter skin. Classic PKU dramatically affects myelination and white matter tracts in untreated infants; this may be one major cause of neurological disorders associated with phenylketonuria. Mild phenylketonuria can act as an unsuspected cause of hyperactivity, learning problems, and other developmental problems in children. It has been recently suggested that PKU may resemble amyloid diseases, such as Alzheimer's disease and Parkinson's disease, due to the formation of toxic amyloid-like assemblies of phenylalanine (PMID: 22706200 ). Higher serum/plasma levels (>75 µmol/L) of phenylalanine are commonly seen in people suffering from heart failure, chronic inflammation or infections, as well those suffering from sepsis or septic shock (PMID: 31170548 ; PMID: 32618142 ). Normally most amino acid levels drop during inflammation or infection, so a rise in phenylalanine levels is considered unusual. While normal levels of phenylalanine are between 40-75 µmol/L, those in intensive care units with phenylalanine levels >110 µmol/L are at much greater risk of dying (PMID: 32618142 ). These moderately high levels of phenylalanine are not necessarily toxic, they are simply secondary indicators of something else that is seriously wrong. The metabolism of phenylalanine and its level in blood directly relates to the activity of the enzyme phenylalanine hydroxylase, which is functionally attenuated by high levels of inflammation. The attenuation of phenylalanine hydroxylase appears to be associated with insufficient tetrahydrobiopterin (BH4), the co‐factor for phenylalanine hydroxylase. In particular, inflammation‐induced production of reactive oxygen species (ROS) may consume a significant portion of BH4, thereby leaving phenylalanine unmetabolized. Pro‐inflammatory cytokine‐mediated inflammation also increases the production of neopterin at the expense of the production of BH4. Insufficient bioavailability of BH4 also leads to dysfunction in multiple systems, including nitric oxide synthase, tryptophan/kynurenine metabolism, the catecholamine pathway, the neural system, and thyroid hormone production (PMID: 32618142 ). High phenylalanine concentrations in septic patients may also reflect the breakdown of muscle tissues (leading to amino acid release) and the body’s differential metabolic capacity for different amino acids. Muscle tissue is easily able to oxidize branched chain amino acids to support its own energy requirements. Muscle tissue is also able to metabolize alanine, glycine, proline, aspartate, glutamate, histidine, glutamine and serine for gluconeogenesis, but aromatic amino acids such as phenylalanine and tyrosine as well as many cysteine-containing amino acids are not as easily metabolized. This may also account for the increase in the levels of phenylalanine and tyrosine seen during sepsis (PMID: 99098 ). Phenylalanine also has some potential benefits. Phenylalanine can act as an effective pain reliever. Its use in premenstrual syndrome and Parkinson's may enhance the effects of acupuncture and electric transcutaneous nerve stimulation (TENS). Phenylalanine and tyrosine, like L-DOPA, produce a catecholamine-like effect. Phenylalanine is better absorbed than tyrosine and may cause fewer headaches. Low phenylalanine diets have been prescribed for certain cancers with mixed results. For instance, some tumours use more phenylalanine than others (particularly melatonin-producing tumours called melanomas).Phenylalanine is elevated by a factor of two or more in the urine of patients with diet-controlled PKU or phenylketonuria (PMID: 37446577 ).

Structure

MOL 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

HEADER    PROTEIN                                 27-SEP-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   27-SEP-20         0                                  
HETATM    1  C   UNK     0    8666.6948667.435   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0    8668.0288666.666   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0    8669.3598667.435   0.000  0.00  0.00           C+0
HETATM    4  O   UNK     0    8670.6938666.666   0.000  0.00  0.00           O+0
HETATM    5  O   UNK     0    8669.3598668.974   0.000  0.00  0.00           O+0
HETATM    6  N   UNK     0    8668.0288665.130   0.000  0.00  0.00           N+0
HETATM    7  C   UNK     0    8665.3638666.668   0.000  0.00  0.00           C+0
HETATM    8  C   UNK     0    8664.0298667.438   0.000  0.00  0.00           C+0
HETATM    9  C   UNK     0    8662.6968666.668   0.000  0.00  0.00           C+0
HETATM   10  C   UNK     0    8662.6968665.128   0.000  0.00  0.00           C+0
HETATM   11  C   UNK     0    8664.0298664.358   0.000  0.00  0.00           C+0
HETATM   12  C   UNK     0    8665.3638665.128   0.000  0.00  0.00           C+0
CONECT    1    2    7                                                       
CONECT    2    1    3    6                                                  
CONECT    3    2    4    5                                                  
CONECT    4    3                                                            
CONECT    5    3                                                            
CONECT    6    2                                                            
CONECT    7    8   12    1                                                  
CONECT    8    7    9                                                       
CONECT    9    8   10                                                       
CONECT   10    9   11                                                       
CONECT   11   10   12                                                       
CONECT   12   11    7                                                       
MASTER        0    0    0    0    0    0    0    0   12    0   24    0
END

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
(S)-2-Amino-3-phenylpropionic acid	ChEBI
(S)-alpha-Amino-beta-phenylpropionic acid	ChEBI
3-Phenyl-L-alanine	ChEBI
beta-Phenyl-L-alanine	ChEBI
F	ChEBI
Phe	ChEBI
PHENYLALANINE	ChEBI
(S)-2-Amino-3-phenylpropionate	Generator
(S)-a-Amino-b-phenylpropionate	Generator
(S)-a-Amino-b-phenylpropionic acid	Generator
(S)-alpha-Amino-beta-phenylpropionate	Generator
(S)-Α-amino-β-phenylpropionate	Generator
(S)-Α-amino-β-phenylpropionic acid	Generator
b-Phenyl-L-alanine	Generator
Β-phenyl-L-alanine	Generator
(-)-beta-Phenylalanine	HMDB
(L)-Phenylalanine	HMDB
(S)-(-)-Phenylalanine	HMDB
(S)-2-Amino-3-phenylpropanoate	HMDB
(S)-2-Amino-3-phenylpropanoic acid	HMDB
(S)-alpha-Amino-benzenepropanoate	HMDB
(S)-alpha-Amino-benzenepropanoic acid	HMDB
(S)-alpha-Aminobenzenepropanoate	HMDB
(S)-alpha-Aminobenzenepropanoic acid	HMDB
(S)-alpha-Aminohydrocinnamate	HMDB
(S)-alpha-Aminohydrocinnamic acid	HMDB
(S)-Phenylalanine	HMDB
alpha-Aminohydrocinnamate	HMDB
alpha-Aminohydrocinnamic acid	HMDB
beta-Phenyl-alpha-alanine	HMDB
beta-Phenylalanine	HMDB
L-2-Amino-3-phenylpropionate	HMDB
L-2-Amino-3-phenylpropionic acid	HMDB
Phenyl-alanine	HMDB
Phenylalamine	HMDB
L-Isomer phenylalanine	HMDB
Phenylalanine, L isomer	HMDB
Phenylalanine, L-isomer	HMDB
Endorphenyl	HMDB
(6S)-Tetrahydrofolate	HMDB
(6S)-Tetrahydrofolic acid	HMDB
(6S)-THFA	HMDB
5,6,7,8-Tetrahydrofolate	HMDB
Tetrahydrofolate	HMDB
THF	HMDB
5,6,7,8-Tetrahydrofolic acid	HMDB

Chemical Formula

C₉H₁₁NO₂

Average Molecular Weight

165.1891

Monoisotopic Molecular Weight

165.078978601

IUPAC Name

(2S)-2-amino-3-phenylpropanoic acid

Traditional Name

L-phenylalanine

CAS Registry Number

63-91-2

SMILES

N[C@@H](CC1=CC=CC=C1)C(O)=O

InChI Identifier

InChI=1S/C9H11NO2/c10-8(9(11)12)6-7-4-2-1-3-5-7/h1-5,8H,6,10H2,(H,11,12)/t8-/m0/s1

InChI Key

COLNVLDHVKWLRT-QMMMGPOBSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as phenylalanine and derivatives. Phenylalanine and derivatives are compounds containing phenylalanine or a derivative thereof resulting from reaction of phenylalanine at the amino group or the carboxy group, or from the replacement of any hydrogen of glycine by a heteroatom.

Showing metabocard for Phenylalanine (HMDB0000159)

MOL for HMDB0000159 (Phenylalanine)

3D MOL for HMDB0000159 (Phenylalanine)

3D SDF for HMDB0000159 (Phenylalanine)

3D-SDF for HMDB0000159 (Phenylalanine)

PDB for HMDB0000159 (Phenylalanine)

3D PDB for HMDB0000159 (Phenylalanine)

SMILES for HMDB0000159 (Phenylalanine)

INCHI for HMDB0000159 (Phenylalanine)

Structure for HMDB0000159 (Phenylalanine)

3D Structure for HMDB0000159 (Phenylalanine)

Experimental Collision Cross Sections

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra

IR Spectra