Zobrazeno 1 - 10
of 58
pro vyhledávání: '"C R Roe"'
Autor:
C R Roe, F. Demaugre, Audrey Boutron, A. Legrand, J. M. Saudubray, Michèle Brivet, F. Poggi, David S. Millington, Abdelhamid Slama
Publikováno v:
Journal of Inherited Metabolic Disease. 19:181-184
Four steps are required for the transport of long-chain fatty acids (LCFA) into the mitochondrial matrix : a plasma membrane carnitine transporter maintains the intracellular supply of carnitine ; an outer mitochondrial membrane carnitine palmitoyltr
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8ca06fa1450d3e1c9b6867f44ae52495
https://doi.org/10.1007/bf01799424
https://doi.org/10.1007/bf01799424
Publikováno v:
Clinical Chemistry. 39:66-71
A new method for quantifying specific amino acids in small volumes of plasma and whole blood has been developed. Based on isotope-dilution tandem mass spectrometry, the method takes only a few minutes to perform and requires minimal sample preparatio
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::23f430bc641aaf5b389d28071cf58255
https://doi.org/10.1093/clinchem/39.1.66
https://doi.org/10.1093/clinchem/39.1.66
Publikováno v:
Journal of lipid research. 39(1)
The relationship between peroxisomal and mitochondrial oxidation of the methyl branched fatty acids, phytanic acid and pristanic acid, was studied in normal and mutant human skin fibroblasts with established enzyme deficiencies. Tandem mass spectrome
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid________::ff09df55df517970375856c16f03b708
https://pubmed.ncbi.nlm.nih.gov/9469587
https://pubmed.ncbi.nlm.nih.gov/9469587
Autor:
B S, Andresen, P, Bross, C, Vianey-Saban, P, Divry, M T, Zabot, C R, Roe, M A, Nada, A, Byskov, T A, Kruse, S, Neve, K, Kristiansen, I, Knudsen, M J, Corydon, N, Gregersen
Publikováno v:
Human molecular genetics. 5(4)
Very-long-chain acyl-CoA dehydrogenase (VLCAD) is one of four straight-chain acyl-CoA dehydrogenase (ACD) enzymes, which are all nuclear encoded mitochondrial flavoproteins catalyzing the initial step in fatty acid beta-oxidation. We have used the ve
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid________::fb7c51773a58e1e98c055f5c2e3a5409
https://pubmed.ncbi.nlm.nih.gov/8845838
https://pubmed.ncbi.nlm.nih.gov/8845838
Autor:
M A, Nada, C, Vianey-Saban, C R, Roe, J H, Ding, M, Mathieu, R S, Wappner, M G, Bialer, J A, McGlynn, G, Mandon
Publikováno v:
Prenatal diagnosis. 16(2)
Amniocytes isolated from two pregnancies at risk for fatty acid oxidation defects were incubated with stable isotopically labelled palmitate, in the presence of L-carnitine, to probe that pathway. The labelled acylcarnitines were then quantitated usi
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid________::da66c8e56300a6a742afefb68eaa0734
https://pubmed.ncbi.nlm.nih.gov/8650121
https://pubmed.ncbi.nlm.nih.gov/8650121
Publikováno v:
Clinical chemistry. 41(1)
We report a new method for the diagnosis of maple syrup urine disease (MSUD) from dried blood spots on newborn screening cards based on tandem mass spectrometry (MS-MS). The mean +/- SD concentration of Leu plus Ile in normal newborns was 151 +/- 47
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid________::9e2d65ea7e5f78307421422c2d182116
https://pubmed.ncbi.nlm.nih.gov/7813082
https://pubmed.ncbi.nlm.nih.gov/7813082
Autor:
Stephen G. Kahler, Diane S. Roe, D H Chace, David S. Millington, J. L. K. Van Hove, C R Roe, S J R Heales
Publikováno v:
Pediatric research. 35(1)
The purpose of this study was to determine whether treatment with L-carnitine or acetyl-L-carnitine enhances the turnover of lipid or branched-chain amino acid oxidation in patients with inborn errors of metabolism. Increasing i.v. doses of L-carniti
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::92354ed8f82ee7fa0a5b779e4ff28167
https://pubmed.ncbi.nlm.nih.gov/8134205
https://pubmed.ncbi.nlm.nih.gov/8134205
Publikováno v:
World review of nutrition and dietetics. 75
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid________::a61750eb54b39f246a03e4efc8eaa142
https://pubmed.ncbi.nlm.nih.gov/7871828
https://pubmed.ncbi.nlm.nih.gov/7871828
Publikováno v:
Clinical chemistry. 39(1)
A new method for quantifying specific amino acids in small volumes of plasma and whole blood has been developed. Based on isotope-dilution tandem mass spectrometry, the method takes only a few minutes to perform and requires minimal sample preparatio
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid________::fafd8f3cbd910f75cce4c7ac55c6495b
https://pubmed.ncbi.nlm.nih.gov/8419060
https://pubmed.ncbi.nlm.nih.gov/8419060
Autor:
D R, Groothuis, B E, Lippitz, I, Fekete, K E, Schlageter, P, Molnar, O M, Colvin, C R, Roe, D D, Bigner, H S, Friedman
Publikováno v:
Cancer research. 52(20)
Melphalan (L-phenylalanine mustard, L-PAM, alkeran; molecular weight, 305,000) is transported across tumor cell membranes and the blood-brain barrier by the large neutral amino acid (LNAA) transport system. Normally, plasma LNAA levels are high enoug
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid________::377006971d80a8dd05edc6e7102f48cf
https://pubmed.ncbi.nlm.nih.gov/1394182
https://pubmed.ncbi.nlm.nih.gov/1394182