Isocaproic add

All this reasoning appeared very plausible until, in 1900, Walden recorded a grave objection. Following up an old observation of Biot (1835) he found that natural petroleums possess a very obvious rotatory power, while the products of synthesis are inactive. The hypothesis of Engler thus appeared fallacious. It, at least, had need of revision, and that is what Neuberg did some years later. Neuberg had the idea that perhaps the rotatory power of natural petroleums was due to the presence, in the fatty materials from whence they are derived, of optically active fatty acids, such as isovaleric acid and isocaproic add. 

Of these, leucine and its corresponding keto acid, 2-oxo-isocaproic add, are the primary toxic compounds in this disorder (Box 16.1). Maple syrap urine disease is so named because patients with this disorder have a characteristic sweet odor detectable in the urine or cerumen. 

Monotetpene, linalool, limonene, a-pinene, dtronellal, dtral, geranial Pyruvic acid, acetaldehyde, ethanol Isopropanal, isopropanol, a-keto-isobutyric add 3-Methylbutanal, 3-methylbutanol, a-keto-isocaproic add 2-Methylbutanal, 2-methylbutanol Benzaldehyde, phenylacetaldehyde, dnnamaldehyde Hydrocinnamaldehyde, p-hydroxybenzaldehyde p-Hydroxy phenylacetaldehyde, p-hydroxy dnnamaldehyde, p-hydroxy cinnamaldehyde... 

Toxic metabolite Leucine and its keto add, 2-oxo-isocaproic acid Trial of 100-1,000 mg thiamine is completed by some metabolic centers for patients with variant forms of MSUD [1,2],... 

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