Zobrazeno 1 - 10
of 46
pro vyhledávání: '"P. G. Pentchev"'
Autor:
R D Shamburek, P G Pentchev, L A Zech, J Blanchette-Mackie, E D Carstea, J M VandenBroek, P S Cooper, E B Neufeld, R D Phair, H B Brewer, Jr, R O Brady, C C Schwartz
Publikováno v:
Journal of Lipid Research, Vol 38, Iss 12, Pp 2422-2435 (1997)
Niemann-Pick C disease (NP-C) is a rare inborn error of metabolism with hepatic involvement and neurological sequelae that usually manifest in childhood. Although in vitro studies have shown that the lysosomal distribution of LDL-derived cholesterol
Externí odkaz:
https://doaj.org/article/55306360d4814767a73496af294e7219
Autor:
P G Pentchev, J A Barranger
Publikováno v:
Journal of Lipid Research, Vol 19, Iss 4, Pp 401-409 (1978)
Externí odkaz:
https://doaj.org/article/d9acb335febb416a90f01c8d6bbae594
Autor:
J F, Goodrum, P G, Pentchev
Publikováno v:
Journal of neuroscience research. 49(3)
Niemann-Pick C (NPC) disease is an autosomal recessive lipidosis characterized by lysosomal accumulations of unesterified cholesterol. Cultured NPC cells exhibit a defect in the intracellular trafficking of LDL-derived cholesterol that leads to lysos
Publikováno v:
Sub-cellular biochemistry. 28
Autor:
C F, Roff, A, Pastuszyn, J F, Strauss, J T, Billheimer, M T, Vanier, R O, Brady, T J, Scallen, P G, Pentchev
Publikováno v:
The Journal of biological chemistry. 267(22)
Hepatic sterol carrier protein-2 (SCP2) and sterol carrier protein-X (SCPx) levels in normal and in mutant Niemann-Pick Type C mice were determined by immunoblotting with antiserum against rat SCP2. A 14-kDa protein (SCP2) was detected in the cytosol
Autor:
M T, Vanier, C, Rodriguez-Lafrasse, R, Rousson, G, Mandon, J, Boué, A, Choiset, M F, Peyrat, C, Dumontel, M C, Juge, P G, Pentchev
Publikováno v:
American journal of human genetics. 51(1)
Thirty-seven pregnancies at risk for Niemann-Pick type C disease were monitored by study of cultured amniotic fluid cells (8 cases) or chorionic villus cells (29 cases) in 23 couples over the period 1984–91. An early protocol combined determination
Autor:
J A Barranger, P G Pentchev
Publikováno v:
Journal of Lipid Research, Vol 19, Iss 4, Pp 401-409 (1978)
Publikováno v:
Biochemistry. 12:2490-2495
Autor:
P. G. Pentchev, J. Martyn Bailey
Publikováno v:
Biochemical and Biophysical Research Communications. 14:161-164
Publikováno v:
Biochimica et Biophysica Acta (BBA) - Biophysics including Photosynthesis. 94:124-129
Homogenates of rat kidney, liver, intestine, stomach, lung, spleen, heart, brain, diaphragm and skeletal muscle were prepared both in EDTA and phosphate buffers. “Mutarotase” (EC 5.1.3.3) and lactate dehydrogenase (EC 1.1.1.27) activity were meas