83972-60-5Relevant articles and documents
Identification of 5-hydroxyhexanoic acid in the urine of twin siblings with a Reye's-like syndrome associated with dicarboxylic aciduria and hypoglycaemia and with similarities to Jamaican vomiting sickness
Chalmers,Lawson
, p. 444 - 446 (1979)
Twin male infant siblings who presented in Harrow, UK, with a Reye's-like syndrome associated with profound hypoglycaemia, vomiting, diarrhoea, coma and death in one child, with dicarboxylic aciduria, and similarities to Jamaican vomiting sickness (hypoglycin toxicity) have been shown to excrete large amounts of a previously unrecorded urinary organic acid. This has been identified as 5-hydroxyhexanoic acid by gas chromatography mass spectrometry using a synthesized standard. Concentrations observed were 340 and 330 mg g-1 creatinine in the two patients. The metabolic precursor of the urinary acid is suggested to be hex-4-enoic acid, a probable chemical toxin closely related to the active organic acid metabolite of hypoglycin. The possibility of ω-1 oxidation of hexanoic acid to 5-hydroxyhexanoic acid in these and other patients with dicarboxylic acidurias is also discussed.
PHA E and PHA C components of poly(hydroxy fatty acid) synthase from thiocapsa pfennigii
-
, (2008/06/13)
PCT No. PCT/DE95/01279 Sec. 371 Date Jul. 3, 1997 Sec. 102(e) Date Jul. 3, 1997 PCT Filed Sep. 15, 1995 PCT Pub. No. WO96/08566 PCT Pub. Date Mar. 21, 1996The present invention relates to a process for the production of poly (hydroxy fatty acids) as well as recombinant bacterial strains for carrying out the process. In addition, new poly(hydroxy fatty acids) and new substrates for the production of conventional and new poly(hydroxy fatty acids) are described. Moreover, the invention also relates to a DNA fragment, which codes for a PhaE and a PhaC component of the poly(hydroxy fatty acid) synthase from Thiocapsa pfennigii, as well as the corresponding poly (hydroxy fatty acid) synthase protein.