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109 results
  1. Nonketotic Hyperglycinemia - GeneReviews® - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK1357
    ... several other amino acids in the CSF (particularly branched-chain amino acids). Note that on brain MRI the diffusion restriction ... Tada K. Effects of the metabolites of the branched-chain amino acids and cysteamine on the glycine cleavage system. Biochem ...
  2. Isolated Methylmalonic Acidemia - GeneReviews® - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK1231
    ... reflect low natural protein intake, imbalanced intake of branched chain amino acid from use of metabolic formulas, or effects of chronic acidosis on branched chain amino acid metabolism [ Manoli et al 2016b ]. Surveillance During the ...
  3. Episodic Ataxia Type 2 - GeneReviews® - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK1501
    ... physical developmental delay, feeding problems, and elevation of branched-chain amino acids and keto acids in the urine as well ... This condition is treated by the elimination of branched-chain amino acids (leucine, isoleucine, valine) from the diet. A variant ...
  4. pdfNLM Technical Bulletin, no.105-116 
    https://www.nlm.nih.gov/.../nlmarchives/techbull/105-116-1978.pdf
    ... CHAIN is a new major descriptor, with the branched-chain amino acids collected under this umbrella. Another term which has ... INHIB PTEROYLPOLYGLUTAMIC ACIDS* THIAMINE MONOPHOSPHATE* THIAMINE TRIPHOSPHATE* D12 AMINO ACIDS, BRANCHED-CHAIN 3-AMINOPROPIONIC ACID* AMYLOID PROTEIN AA* AMYLOID PROTEIN ...
  5. Nuclear Gene-Encoded Leigh Syndrome Spectrum Overview - GeneReviews® - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK320989
    ... KGDH = alpha-ketoglutarate dehydrogenase; AR = autosomal recessive ; BCAA = branched-chain amino acid; BCKDH = branched-chain ketoacid dehydrogenase; CC = corpus callosum; ...
  6. Mitochondrial Membrane Protein-Associated Neurodegeneration - GeneReviews® - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK185329
    ... regulation with proteins of fatty acid biogenesis and branched chain amino acid degradation expression profiles suggest similarities to other proteins ...
  7. Hepatocerebral degeneration 
    https://medlineplus.gov/ency/article/001393.htm
    ... of ammonia in the blood. A treatment called branched-chain amino acid therapy may also: Improve symptoms Reverse brain damage ...
  8. Excluded Studies - Short- and Long-Term Outcomes after Bariatric Surgery in the Medicare Population - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK544886
    ... Effects of Sleeve Gastrectomy and Gastric Bypass on Branched-Chain Amino Acid Metabolism 1Â Year After Bariatric Surgery Obesity Surgery ...
  9. Mitochondrial Short-Chain Enoyl-CoA Hydratase 1 Deficiency - GeneReviews® - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK542806
    ... However, the same enzyme also is involved in branched-chain amino acid metabolism, particularly valine metabolism [ Peters et al 2014 , ...
  10. Coenzyme Q10 (PDQ®) - PDQ Cancer Information Summaries - NCBI Bookshelf 
    https://www.ncbi.nlm.nih.gov/books/NBK65890
    ... contained coenzyme Q 10 (30 mg) along with branched-chain amino acids (2,500 mg) and L-carnitine (50 mg). ...
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