Reference

Ornithine transcarbamylase deficiency

Ornithine transcarbamylase deficiency. Yeh, Shu-Jen; Hwu, Wuh-Liang; Tsai, Wen-Shiung; Wu, Tzong-Jin; Tuchman, Mendel; Wang, Tso-Ren (Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan). Journal of the Formosan Medical Association, 96(1), 43-45 (English) 1997 Formosan Medical Association. CODEN: JFASEO. ISSN: 0929-6646. DOCUMENT TYPE: Journal CA Section: 14 (Mammalian Pathological Biochemistry) Section cross-reference(s): 1 Two infants, one male and one female, with elevated serum ammonia levels, were shown, based on urine org. acid anal. and DNA studies, to have ornithine transcarbamylase (OTC) deficiency. OTC deficiency is one of the most common urea cycle disorders. Hyperammonemia occurred at 3 days of age in the male infant, and at approx. 7 days of age in the female infant. Administration of sodium benzoate and sodium phenylacetate lowered the serum ammonia level effectively in both cases. Other modalities, including peritoneal dialysis and protein restriction, were also important in the control of the serum ammonia level.There are some commonly used reagents with their cas registry numbers 7664-41-7 and 114-70-5 in this article. The mother of the male infant was shown to be a carrier of the OTC gene mutation by allopurinol loading test. The mutation site of the OTC gene for the female infant was identified, but her mother did not have the mutation. OTC deficiency, an incompletely dominant X-linked disorder, is a severe disease even for females and prompt treatment and precise genetic counseling are mandatory. .

Anti-inflammatory effects of short chain fatty acids in IFN-g-stimulated RAW 264

All Rights Reserved. Anti-inflammatory effects of short chain fatty acids in IFN-g-stimulated RAW 264.7 murine macrophage cells: Involvement of NF-kB and ERK signaling pathways. Park, Jin-Sun; Lee, Eun-Jung; Lee, Jae-Chul; Kim, Won-Ki; Kim, Hee-Sun (Department of Neuroscience and Medical Research Institute, College of Medicine, Ewha Womans University, Seoul 158-710, S. Korea). International Immunopharmacology, Volume Date 2007, 7(1), 70-77 (English) 2006 Elsevier B.V. CODEN: IINMBA. ISSN: 1567-5769. DOCUMENT TYPE: Journal CA Section: 1 (Pharmacology) The overactivation of macrophages causes abnormal cell death and chronic inflammatory diseases. Therefore, the modulation of macrophage-mediated cytotoxicity is expected to become a new therapeutic strategy for various inflammatory diseases. In this study, three types of short chain fatty acids (sodium butyrate (NaB), sodium phenylbutyrate (NaPB), sodium phenylacetate (NaPA)) were found to have anti-inflammatory effects in IFN-g-stimulated RAW 264.7 cells. They inhibited the expression of iNOS, TNF-a, and IL-6 induced by IFN-g, while they enhanced the expression of the anti-inflammatory cytokine, IL-10. Their potency as anti-inflammatory agents was in the order of NaB > NaPB > NaPA. Further mechanistic studies revealed these three agents to repress the DNA binding and transcriptional activities of NF-kB, which is an important modulator of inflammation. In addn., these agents repressed the IFN-g-induced ERK1/2 phosphorylation without affecting the Jak/STAT activities. The potency of NF-kB and ERK inhibition was also in the order of NaB > NaPB > NaPA. The results suggest that the NF-kB and ERK signaling pathways are at least in part involved in the anti-inflammatory activities of these SCFAs. Considering that SCFAs are normally present in the body and have few side effects, they might be promising agents for the prevention and/or treatment of various inflammatory diseases.Except for chemicals metioned above, 114-70-5 and 501433-35-8 are also used. .