PubMed · 826666
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1976. Lithogenic bile.. https://pubmed.ncbi.nlm.nih.gov/826666/
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Helicobacter pylori is one of the most prevalent human pathogens in the world and is the aetiological agent of gastritis, peptic ulcer disease and gastric malignancies. In addition H. pylori and other novel members of the genus are capable of successfully colonising the bile-rich niche of the upper intestine and are associated with a diverse range of intestinal pathologies. Surface-enhanced laser desorption/ionisation-time of flight mass spectrometry was used to analyse surface extracts from H. pylori, Helicobacter bilis, Helicobacter pullorum and "Helicobacter sp. flexispira" to characterise cell surface changes following bile stress. The system detected two distinct response patterns to bile stress on the cell surface of Helicobacter spp. in vitro. The first involved the increase under bile stress of peaks at 7.6 and 7.9 kDa for H. billis and H. pullorum, respectively. In contrast both "Helicobacter sp. flexispira" and a clinical isolate of H. pylori had similar response profiles to bile stress. Both strains had at least three low mass peaks decreased under bile stress and a single peak induced by bile stress. The present study has established the use of ProteinChip(R) technology to analyse helicobacter-related proteomics. Specifically this study has established that different patterns are generated in response to bile stress among various pathogenic Helicobacter spp. which may give insights into the ability of these strains to colonise different niches.
BACKGROUND & AIMS: Inborn errors of bile acid metabolism may present as neonatal cholestasis and fat-soluble vitamin malabsorption or as late onset chronic liver disease. Our aim was to fully characterize a defect in bile acid synthesis in a 2-week-old African-American girl presenting with coagulopathy, vitamin D and E deficiencies, and mild cholestasis and in her sibling, whose liver had been used for orthotopic liver transplantation (OLT). METHODS: Bile acids were measured by mass spectrometry in urine, bile, serum, and feces of the patient and in urine from the unrelated recipient. RESULTS: Liver biopsy specimens showed neonatal hepatitis with giant cell transformation and hepatocyte necrosis; peroxisomes were reduced in number. High concentrations of (25R)3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoic acid in the urine, bile, and serum established a pattern similar to that of Zellweger syndrome and identical to the Alligator mississippiensis. Serum phytanic acid was normal, whereas pristanic acid was markedly elevated. Biochemical, MRI, and neurologic findings were inconsistent with a generalized defect of peroxisomal function and were unique. Analysis of the urine from the recipient of the deceased sibling's liver confirmed the same bile acid synthetic defect. A deficiency in 2-methylacyl-CoA racemase, which is essential for conversion of (25R)THCA to its 25S-isomer, the substrate to initiate peroxisomal beta-oxidation to primary bile acids, was confirmed by DNA analysis revealing a missense mutation (S52P) in the gene encoding this enzyme. Long-term treatment with cholic acid normalized liver enzymes and prevented progression of symptoms. CONCLUSIONS: This genetic defect further highlights bile acid synthetic defects as a cause of neonatal cholestasis.
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