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  2. Branched-chain amino acid - Wikipedia

    en.wikipedia.org/wiki/Branched-chain_amino_acid

    A deficiency of this complex leads to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products in the blood and urine, giving the condition the name maple syrup urine disease. On the other hand, unchecked activity of this complex causes branched-chain keto acid dehydrogenase kinase deficiency.

  3. BCKDK - Wikipedia

    en.wikipedia.org/wiki/BCKDK

    Deficiency of BCKDK, first described in 2012, [11] is a disorder that could be considered as the "opposite" of maple syrup disease, because patients have decreased levels of branched-chain amino acids, instead of increased levels. The condition may present as autism with epileptiform abnormalities on EEG and seizures.

  4. Cirrhosis - Wikipedia

    en.wikipedia.org/wiki/Cirrhosis

    Generally, liver damage from cirrhosis cannot be reversed, but treatment can stop or delay further progression and reduce complications. A healthy diet is encouraged, as cirrhosis may be an energy-consuming process. A recommended diet consists of high-protein, high-fiber diet plus supplementation with branched-chain amino acids. [111]

  5. Branched-chain alpha-keto acid dehydrogenase complex

    en.wikipedia.org/wiki/Branched-chain_alpha-keto...

    A deficiency in any of the enzymes of this complex as well as an inhibition of the complex as a whole leads to a buildup of branched-chain amino acids and their harmful derivatives in the body. These accumulations lend a sweet smell to bodily excretions (such as ear wax and urine), leading to a pathology known as maple syrup urine disease. [19]

  6. BCKDHA - Wikipedia

    en.wikipedia.org/wiki/BCKDHA

    The second major step in the catabolism of the branched-chain amino acids (isoleucine, leucine, and valine) is catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD; EC 1.2.4.4), an inner-mitochondrial enzyme complex that consists of 3 catalytic components: a heterotetrameric (alpha2, beta2) branched-chain alpha-keto acid decarboxylase (E1), a homo-24-meric dihydrolipoyl ...

  7. α-Ketoisocaproic acid - Wikipedia

    en.wikipedia.org/wiki/Α-Ketoisocaproic_acid

    This is disease is caused by a partial branched-chain alpha-keto acid dehydrogenase deficiency, which leads to a buildup of branched-chain alpha-keto acids, including α-KIC and HMB. [13] These keto-acids build up in the liver , [ 2 ] [ 4 ] [ 5 ] and since limited isovaleryl-CoA can be produced, these keto-acids must be excreted in the urine as ...

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