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Sodium phenylbutyrate has an aromatic ring attached to butyric acid in its chemical structure. It's a sodium salt of a fragrant fatty acid. The chemical name for the sodium salt of 4-phenylbutyric acid is phenylbutyrate of sodium. It forms water-soluble crystals that are off-white in hue.
The tablet or powder form of sodium phenylbutyrate, which may be administered orally or through nasogastric intubation, has an extremely salty and disagreeable taste. It is used to treat urea cycle disorders, which are hereditary conditions in which ammonia glutamine accumulates in the blood plasma (a state called hyperammonemia). Deficits in the enzymes carbamoyl phosphate synthetase I, ornithine transcarbamylase, or argininosuccinic acid synthetase cause this.
Amenorrhea and other kinds of menstrual disturbance may be harmful to one-fourth of all women. Only 4% of patients report having a loss of appetite. As a consequence of the metabolization of phenylbutyrate, 3% of patients report an unpleasant body odor, and the same number describe an unpleasant taste. In addition to a range of other reported side effects, gastrointestinal issues and mostly mild symptoms of neurotoxicity are seen in fewer than 2% of people.
In cystic fibrosis sufferers, a point mutation in the Cystic Fibrosis Transmembrane Conductance Regulator protein, often known as F508-CFTR, causes the protein to be unstable and misfold. As a consequence of being caught in the endoplasmic reticulum, the protein is unable to reach the cell membrane. The interruption in chloride transport caused by cystic fibrosis and its accompanying symptoms is caused by the lack of CFTR in the cell membrane. Sodium phenylbutyrate has the potential to act as a chemical chaperone. In this function, it may stabilize mutant CFTR in the endoplasmic reticulum and allow it to reach the cell surface.
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The precursor of sodium phenylbutyrate is the aromatic fatty acid 4-phenylbutyrate (4-PBA) or 4-phenylbutyric acid. Because the compound's metabolites give an alternative pathway to the urea cycle, it is utilized to treat urea cycle-related diseases. This permits extra nitrogen to be excreted.
The fact that sodium phenylbutyrate is both an inhibitor of histone deacetylase and a chemical chaperone has motivated research into its usage as an anti-cancer medication and in protein misfolding diseases such as cystic fibrosis.
Structure and possessed qualities
Sodium phenylbutyrate has an aromatic ring attached to butyric acid in its chemical structure. It's a sodium salt of a fragrant fatty acid. The chemical name for the sodium salt of 4-phenylbutyric acid is phenylbutyrate of sodium. It forms water-soluble crystals that are off-white in hue.
Medical applications
The tablet or powder form of sodium phenylbutyrate, which may be administered orally or through nasogastric intubation, has an extremely salty and disagreeable taste. It is used to treat urea cycle disorders, which are hereditary conditions in which ammonia glutamine accumulates in the blood plasma (a state called hyperammonemia). Deficits in the enzymes carbamoyl phosphate synthetase I, ornithine transcarbamylase, or argininosuccinic acid synthetase cause this. If not treated, it will result in mental impairment as well as premature mortality. Children born with urea cycle disorders may typically survive for more than a year with the support of dialysis, amino acid supplements, and a protein-restricted diet. Sodium phenylbutyrate metabolites allow the kidneys to excrete excess nitrogen rather than urea. Patients may need continued treatment throughout the remainder of their lives. Researchers were the first to create the treatment in the 1990s, and the United States finally approved it. The Food and Drug Administration (FDA) was formed in April 1996.
Negative ramifications
Amenorrhea and other kinds of menstrual disturbance may be harmful to one-fourth of all women. Only 4% of patients report having a loss of appetite. As a consequence of the metabolization of phenylbutyrate, 3% of patients report an unpleasant body odor, and the same number describe an unpleasant taste. In addition to a range of other reported side effects, gastrointestinal issues and mostly mild symptoms of neurotoxicity are seen in fewer than 2% of people. Other side effects include: Because sodium phenylbutyrate treatment may mimic maternal phenylketonuria due to phenylalanine production, administration when pregnant is not recommended because it may cause brain damage to the developing fetal brain.
Research
Urea cycle disruptions
Dr. Saul Brusilow, Mark Batshaw, and their colleagues at the Johns Hopkins School of Medicine discovered in the early 1980s that administering sodium phenylbutyrate resulted in an alternate nitrogen disposal mechanism. This finding was made possible by a series of coincidental discoveries. In the late 1970s, they investigated ketoacid therapy for another inborn metabolic mistake, citrullinemia, and discovered that treatment with arginine resulted in an increase in nitrogen in the urine and a decrease in ammonia in the blood. They found this after researching ketoacid treatment for citrullinemia in the late 1970s. When the researchers notified Norman Radin about their results, he remembered reading an article in 1914 about utilizing sodium benzoate to reduce urea excretion. Because sodium phenylacetate was used in another study published in 1919, the researchers decided to employ benzoate and phenylacetate to treat five patients with hyperammonemia and then publish their results in the journal Science. In 1982 and 1984, the researchers published their results on the use of benzoate and arginine for the treatment of urea cycle disorders in the NEJM. Because sodium phenylbutyrate lacks the odor of phenylacetate, its usage became increasingly common in the early 1990s.
Agent of chemical buffering
In cystic fibrosis sufferers, a point mutation in the Cystic Fibrosis Transmembrane Conductance Regulator protein, often known as F508-CFTR, causes the protein to be unstable and misfold. As a consequence of being caught in the endoplasmic reticulum, the protein is unable to reach the cell membrane. The interruption in chloride transport caused by cystic fibrosis and its accompanying symptoms is caused by the lack of CFTR in the cell membrane. Sodium phenylbutyrate has the potential to act as a chemical chaperone. In this function, it may stabilize mutant CFTR in the endoplasmic reticulum and allow it to reach the cell surface.
Inhibitor of the enzyme histone deacetylase
Because of its action as a histone deacetylase inhibitor, sodium phenylbutyrate is being studied as a possible differentiation-inducing drug in malignant glioma and acute myeloid leukemia. Furthermore, sodium phenylbutyrate is being studied as an alternative to hydroxycarbamide for the treatment of some sickle-cell illnesses since it causes the expression of fetal hemoglobin to replace absent adult hemoglobin. To far, no published data supports the use of the chemical in the clinical treatment of cancer, and it is still being studied on a small scale, despite the fact that small-scale research is presently being undertaken. Another prospective medication for Huntington's disease is sodium phenylbutyrate, which is now being studied as a therapeutic drug.
Other
In Drosophila research, phenylbutyrate has been associated to longer lifespans.
Dr. Curt Freed and Wenbo Zhou of the University of Colorado discovered that giving phenylbutyrate to animals stopped the course of Parkinson's disease. They accomplished this by turning on the DJ-1 gene, which has the capacity to prevent the death of dopaminergic neurons in the midbrain. Beginning in July 2011, they intend to begin testing phenylbutyrate on human participants for the treatment of Parkinson's disease.
Pharmacology
Nitrogen elimination through the action of phenylbutyrate metabolites
Phenylbutyrate is an example of a prodrug. It is transformed to phenylbutyryl-CoA in the human body before being metabolized by mitochondrial beta-oxidation, principally in the liver and kidneys, to create the active form, phenylacetate. [18] Phenylacetate then combines with glutamine to form phenylacetylglutamine, which is excreted in the urine. Because it has the same amount of nitrogen as urea, it may be utilized as an alternative to urea for nitrogen removal in the body.
If Pure PBA is administered orally in the form of a 5 gram tablet or powder, the medication may be detected in the blood within 15 minutes and reaches its maximal concentration in the circulation within an hour. The metabolic mechanism converts it to phenylacetate in half an hour.
Because of its action as a histone deacetylase inhibitor, sodium phenylbutyrate is being studied as a possible differentiation-inducing drug in malignant glioma and acute myeloid leukemia. Furthermore, sodium phenylbutyrate is being studied as an alternative to hydroxycarbamide for the treatment of some sickle-cell illnesses since it causes the expression of fetal hemoglobin to replace absent adult hemoglobin.
Dr. Curt Freed and Wenbo Zhou of the University of Colorado discovered that giving phenylbutyrate to animals stopped the course of Parkinson's disease. They accomplished this by turning on the DJ-1 gene, which has the capacity to prevent the death of dopaminergic neurons in the midbrain. Beginning in July 2011, they intend to begin testing phenylbutyrate on human participants for the treatment of Parkinson's disease.
Phenylbutyrate is an example of a prodrug. It is transformed to phenylbutyryl-CoA in the human body before being metabolized by mitochondrial beta-oxidation, principally in the liver and kidneys, to create the active form, phenylacetate. [18] Phenylacetate then combines with glutamine to form phenylacetylglutamine, which is excreted in the urine. Because it has the same amount of nitrogen as urea, it may be utilized as an alternative to urea for nitrogen removal in the body.
Sodium phenylbutyrate has an aromatic ring attached to butyric acid in its chemical structure. It's a sodium salt of a fragrant fatty acid. The chemical name for the sodium salt of 4-phenylbutyric acid is phenylbutyrate of sodium. It forms water-soluble crystals that are off-white in hue.
The tablet or powder form of sodium phenylbutyrate, which may be administered orally or through nasogastric intubation, has an extremely salty and disagreeable taste. It is used to treat urea cycle disorders, which are hereditary conditions in which ammonia glutamine accumulates in the blood plasma (a state called hyperammonemia). Deficits in the enzymes carbamoyl phosphate synthetase I, ornithine transcarbamylase, or argininosuccinic acid synthetase cause this. If not treated, it will result in mental impairment as well as premature mortality. Children born with urea cycle disorders may typically survive for more than a year with the support of dialysis, amino acid supplements, and a protein-restricted diet. Sodium phenylbutyrate metabolites allow the kidneys to excrete excess nitrogen rather than urea. Patients may need continued treatment throughout the remainder of their lives. Researchers were the first to create the treatment in the 1990s, and the United States finally approved it. The Food and Drug Administration (FDA) was formed in April 1996.
Agent of chemical buffering
In cystic fibrosis sufferers, a point mutation in the Cystic Fibrosis Transmembrane Conductance Regulator protein, often known as F508-CFTR, causes the protein to be unstable and misfold. As a consequence of being caught in the endoplasmic reticulum, the protein is unable to reach the cell membrane. The interruption in chloride transport caused by cystic fibrosis and its accompanying symptoms is caused by the lack of CFTR in the cell membrane. Sodium phenylbutyrate has the potential to act as a chemical chaperone. In this function, it may stabilize mutant CFTR in the endoplasmic reticulum and allow it to reach the cell surface.
Inhibitor of the enzyme histone deacetylase
Because of its action as a histone deacetylase inhibitor, sodium phenylbutyrate is being studied as a possible differentiation-inducing drug in malignant glioma and acute myeloid leukemia. Furthermore, sodium phenylbutyrate is being studied as an alternative to hydroxycarbamide for the treatment of some sickle-cell illnesses since it causes the expression of fetal hemoglobin to replace absent adult hemoglobin. To far, no published data supports the use of the chemical in the clinical treatment of cancer, and it is still being studied on a small scale, despite the fact that small-scale research is presently being undertaken. Another prospective medication for Huntington's disease is sodium phenylbutyrate, which is now being studied as a therapeutic drug.
Other
In Drosophila research, phenylbutyrate has been associated to longer lifespans.
Dr. Curt Freed and Wenbo Zhou of the University of Colorado discovered that giving phenylbutyrate to animals stopped the course of Parkinson's disease. They accomplished this by turning on the DJ-1 gene, which has the capacity to prevent the death of dopaminergic neurons in the midbrain. Beginning in July 2011, they intend to begin testing phenylbutyrate on human participants for the treatment of Parkinson's disease.
Pharmacology
Nitrogen elimination through the action of phenylbutyrate metabolites
Phenylbutyrate is an example of a prodrug. It is transformed to phenylbutyryl-CoA in the human body before being metabolized by mitochondrial beta-oxidation, principally in the liver and kidneys, to create the active form, phenylacetate. [18] Phenylacetate then combines with glutamine to form phenylacetylglutamine, which is excreted in the urine. Because it has the same amount of nitrogen as urea, it may be utilized as an alternative to urea for nitrogen removal in the body.
If Pure PBA is administered orally in the form of a 5 gram tablet or powder, the medication may be detected in the blood within 15 minutes and reaches its maximal concentration in the circulation within an hour. The metabolic mechanism converts it to phenylacetate in half an hour.