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  2. Sodium phenylbutyrate - Wikipedia

    en.wikipedia.org/wiki/Sodium_phenylbutyrate

    Sodium phenylbutyrate is taken orally or by nasogastric intubation as a tablet or powder, and tastes very salty and bitter. It treats urea cycle disorders, genetic diseases in which nitrogen waste builds up in the blood plasma as ammonia glutamine (a state called hyperammonemia) due to deficiences in the enzymes carbamoyl phosphate synthetase I, ornithine transcarbamylase, or argininosuccinic ...

  3. Sodium phenylbutyrate/ursodoxicoltaurine - Wikipedia

    en.wikipedia.org/wiki/Sodium_phenylbutyrate/...

    Sodium phenylbutyrate is a chemical chaperone that helps proteins maintain their normal conformation, preventing aggregation that may lead to cell death. [6] Ursodoxicoltaurine improves mitochondrial energy production.

  4. Phenylacetylglutamine - Wikipedia

    en.wikipedia.org/wiki/Phenylacetylglutamine

    Phenylacetylglutamine is the primary metabolite of the degradation of phenylacetate when in the presence of glutamine in the liver. It is also produced in higher concentrations in the body through the metabolic degradation pathway of the pharmaceutical compounds sodium phenylbutyrate, glycerol phenylbutyrate, and sodium phenylacetate, considered more toxic, that are used as treatments for the ...

  5. Ursodoxicoltaurine - Wikipedia

    en.wikipedia.org/wiki/Ursodoxicoltaurine

    In Canada and the United States, ursodoxicoltaurine, in combination with sodium phenylbutyrate, was indicated for the treatment of amyotrophic lateral sclerosis (ALS). [5] [6] Following failed results from the phase 3 PHOENIX trial (NCT05021536) It has been removed from the market in April 2024. Amylyx Pharmaceuticals has announced that ...

  6. Sodium butyrate - Wikipedia

    en.wikipedia.org/wiki/Sodium_butyrate

    Sodium butyrate is a compound with formula Na(C 3 H 7 COO). It is the sodium salt of butyric acid . It has various effects on cultured mammalian cells including inhibition of proliferation , induction of differentiation and induction or repression of gene expression. [ 1 ]

  7. Histone deacetylase inhibitor - Wikipedia

    en.wikipedia.org/wiki/Histone_deacetylase_inhibitor

    the aliphatic acid compounds such as Sodium phenylbutyrate and valproic acid. As of 2007, "second-generation" HDIs included the hydroxamic acids : trichostatin A , vorinostat (SAHA), belinostat (PXD101), resminostat , abexinostat , givinostat , LAQ824 , ivaltinostat , nanatinostat and panobinostat (LBH589); and the benzamides : entinostat (MS ...

  8. Hyperammonemia - Wikipedia

    en.wikipedia.org/wiki/Hyperammonemia

    Phenylbutyrate, which is the product of phenylacetate, conjugates with glutamine to form phenylacetylglutamine, which is excreted by the kidneys. Similarly, sodium benzoate reduces ammonia content in the blood by conjugating with glycine to form hippuric acid, which is rapidly excreted by the kidneys. [14]

  9. Sodium phenylacetate/sodium benzoate - Wikipedia

    en.wikipedia.org/wiki/Sodium_phenylacetate/...

    Sodium phenylacetate/sodium benzoate, sold under the brand name Ammonul among others, is a fixed-dose combination medication used to treat high blood ammonia. [1] It is a combination of sodium phenylacetate and sodium benzoate .

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