2-Isovaleryl-4-

Exceptions formyl, acetyl, propionyl, butyryl, isobutyryl, valeryl, isovaleryl, oxalyl, malonyl, succinyl, glutaryl, furoyl, and thenoyl. 

Reaction 3 is analogous to the dehydrogenation of fatty acyl-CoA thioesters (see Figure 22—3). In isovaleric acidemia, ingestion of protein-rich foods elevates isovalerate, the deacylation product of isovaleryl-CoA. Figures 30-20, 30-21, and 30-22 illustrate the subsequent reactions unique to each amino acid skeleton. 

Mutation of the dihydrolipoate reductase component impairs decarboxylation of branched-chain a-keto acids, of pyruvate, and of a-ketoglutarate. In intermittent branched-chain ketonuria, the a-keto acid decarboxylase retains some activity, and symptoms occur later in life. The impaired enzyme in isovaleric acidemia is isovaleryl-CoA dehydrogenase (reaction 3, Figure 30-19). Vomiting, acidosis, and coma follow ingestion of excess protein. Accumulated... 

XVIII has now been excluded,42 since the methyl glycoside of the sugar reacts rather rapidly with one mole of periodate per mole furthermore, in contrast to the behavior of mycaminose (XXI), no moiety with one carbon atom less has been isolated from the products of periodate oxidation. Oxidation of mycarose with hypoiodite affords a crystalline lactone with the empirical formula C7H12O4 this observation eliminates the possibility of the keto structure XX. Thus, mycarose appears to be a 2,6-dideoxy-3-C -methylhexose (XIX). In the original compound, the isovaleryl group must be esterified to the alcohol function at C4, since the methyl glycoside isovalerate obtained from carbomycin is only attacked by periodate after alkaline hydrolysis. 

Isopropylidone acetone, m3 70 Isopropyl myristate, il31 Isovaleryl chloride, ml86... 

Glycine to promote acylation of isovaleryl-CoA Infancy vomiting, metabolic acidosis, hyperlactatemia, convulsions, coma... 

A few interesting glyceride prodrugs of peptides can be found in the literature. For example, the pentapeptide renin inhibitor isovaleryl-Phe-Nle-Sta-Ala-Sta was derivatized to the l,3-dipalmitoyl-2-glyceryl ester (8.40) in the hope of improving oral bioavailability [55]. The in vitro results were... 

M. Shameem, T. Imai, Y. Yoshigae, A. Sparreboom, M. Otagiri, Stereoselective Hydrolysis of O-Isovaleryl Propranolol and Its Influence on the Clearance of Propranolol after Oral Administration , J. Pharm. Sci. 1994, 83, 1754-1757. 

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