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Fitting a Mathematical Model to Biological Data: Intracellular Trafficking in Niemann-Pick C Disease

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Mathematical Modeling in Nutrition and the Health Sciences

Part of the book series: Advances in Experimental Medicine and Biology ((AEMB,volume 537))

Abstract

Mathematical models are powerful and heuristic aids for investigating complex systems. Once a model has been developed, it encompasses a large body of knowledge, and it can be used to make predictions about how a system would react under various conditions. This approach applies whether the system being modeled is physical (e.g., an automobile) or biological.

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References

  • Blanchette-Mackie, E.J., Dwyer, N.K., Amende, L.M., Kruth, H.S., Butler, J.D., Sokol, J., Comley, M.E., Vanier, M.T., August, J.T., Brady, R.O., and Pentchev, P.G., 1988, Type-C Niemann-Pick disease: low-density lipoprotein uptake is associated with premature cholesterol accumulation in the Golgi complex and excessive cholesterol storage in lysosomes, Proc. Natl. Acad. Sci. USA 85:8022–8026.

    Article  Google Scholar 

  • Blomhoff, R., Nenseter, M.S., Green, M.H., and Berg, T.A., 1989, Multicompartmental model of fluid-phase endocytosis in rabbit liver parenchymal cells, Biochem. J. 262:605–610.

    Google Scholar 

  • Boston, R.C., Greif, P., Wastney, M., and Linares, O., 1998, Balancing needs, efficiency and functionality in the provision of modeling software: a perspective of the NIH WinSAAM project, Adv. Exp. Med. Biol. 445:3–20.

    Google Scholar 

  • Carstea, E.D., Morris, J.A, Coleman, K.G., Loftus, S.K., Zhang, D., Cummings, C, Gu, J.Z., Rosenfeld, M.A., Pavan, W.J., Krizman, D.B., Nagle, J., Polymeropoulos, M.H., Sturley, S.L., Ioannou, Y.A., Higgins, M.E., Comly, M., Cooney, A., Brown, A., Kaneski, C.R., Blanchette-Mackie, E.J., Dwyer, N.K., Neufeld, E.B., Chang, T.-Y., Lisum, L., Strauss III, J.F., Ohno, K., Zeigler, M., Carmi, R., Sokol, J., Markie, D., O’Neill, R.R., van Diggelen, O.P., Elleder, M., Patterson, M.C., Brady, R.O., Vanier, M.T., Pentchev, P.G., and Tagle, D.A., 1997, Neimann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis, Science 277:228–231.

    Article  Google Scholar 

  • Mukherjee, S., Ghosh, R.N., and Maxfield, F.R., 1997, Endocytosis, Physiol. Rev. 77:759–803.

    Google Scholar 

  • Neufeld, E.B., 1998, What the Niemann-Pick Type C gene has taught us about cholesterol transport, in: Intracellular Cholesterol Trafficking T.Y. Chan and D.A. Freeman, Kluwer Acad. Pub., Norwell.

    Google Scholar 

  • Neufeld, E.B., Wastney, M., Patel, S., Suresh, S., Cooney, A.M., Dwyer, N.K., Roff, CF., Ohno, K., Morris, J.A., Carstea, E.D., Incardona, J.P., Strauss III, J.F., Vanier, M.T., Patterson, M.C., Brady, R.O., Pentchev, P.G., and Blanchette-Mackie, E.J., 1999, The Niemann-Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo, J. Biol Chem. 274:9627–9635.

    Article  Google Scholar 

  • Patterson, M.C., Vanier, M.T., Suzuki, K., Morris, J.A., Carstea, E., Neufeld, E.B., Blanchette - Mackie, E.J., and Pentchev, P.G., 2001, Niemann-Pick disease, type C: a lipid trafficking disorder, in: The Metabolic and Molecular Bases of Inherited Disease, 8th ed.C.R. Scriver, K.W.Kinzler, D.Valle, B. Childs, B. Vogelstein, A.L. Beaudet, and W.S. Sly, eds., McGraw-Hill, New York.

    Google Scholar 

  • Pentchev, P.G., Comly, M.E., Tokoro, T., Butler, J., Sokol, J., Filling-Katz, M., Quirk, J.M., Marshall, D.C., Patel, S., and Vanier, M.T., 1987, Group C Niemann-Pick disease: faulty regulation of low-density lipoprotein uptake and cholesterol storage in cultured fibroblasts, FASEB J. 1:40–45.

    Google Scholar 

  • Shamburek, R.D., Pentchev, P.G., Zech, L.A., Blanchette-Mackie, E.J., Carstea, E.D., VandenBroek, J.M., Cooper, P.S., Neufeld, E.B., Phair, R.D., Brewer Jr., H.B., Brady, R.O., and Schwartz, C.C., 1997, Intracellular trafficking of the free cholesterol derived from LDL cholesteryl ester is defective in vivo in Niemann-Pick C disease: insights on normal metabolism of HDL and LDL gained from the NP-C mutation, J. Lipid Res. 38:2422–2435.

    Google Scholar 

  • Sokol, J., Blanchette-Mackie, E.J., Kruth, H.S., Dwyer, N.K., Amende, L.M., Butler, J.D., Robinson, E., Patel, S., Brady, R.O., Comley, M.E., Vanier, M.T., and Pentchev, P.G., 1988, Type C Niemann-Pick disease. Lysosomal accumulation and defective intracellular mobilization of low-density lipoprotein cholesterol, J. Biol. Chem. 263:3411–3417.

    Google Scholar 

  • Wastney, M.E., Patterson, B.H., Linares, O.A., Greif, P.C., and Boston, R.C., 1999, Investigating Biological- Systems using Modeling: Strategies and Software, Academic Press, San Diego.

    Google Scholar 

  • Yano, T., Taniguchi, M., Akaboshi, S., Vanier, M.T., Tai, T., Sakuraba, H., and Ohno,K., 1996, Accumulation of GM2 ganglioside in Niemann-Pick disease type C fibroblasts, Proc. Japan Acad. 72:214–219.

    Article  Google Scholar 

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Wastney, M.E., Pentchev, P.G., Neufeld, E.B. (2003). Fitting a Mathematical Model to Biological Data: Intracellular Trafficking in Niemann-Pick C Disease. In: Novotny, J.A., Green, M.H., Boston, R.C. (eds) Mathematical Modeling in Nutrition and the Health Sciences. Advances in Experimental Medicine and Biology, vol 537. Springer, Boston, MA. https://doi.org/10.1007/978-1-4419-9019-8_4

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  • DOI: https://doi.org/10.1007/978-1-4419-9019-8_4

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4613-4759-0

  • Online ISBN: 978-1-4419-9019-8

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