Photo of Samuel E. Lux,  MD

Samuel E. Lux, MD

Boston Children's Hospital

Boston Children's Hospital
Phone: (617) 919-2093
Fax: (617) 730-0469


lux@enders.tch.harvard.edu

Samuel E. Lux, MD

Boston Children's Hospital

EDUCATIONAL TITLES

  • Robert A. Stranahan Professor of Pediatrics, Pediatrics, Harvard Medical School
  • Director, Internship Selection and Vice-Chair for Research, Boston Children's Hospital

Research Abstract

We work on membrane cytoskeletons and are interested in the structure of the skeleton and its roles in the biology of cells, particularly in vesicle trafficking, in nuclear organization and in regulation of cell growth and adhesion. We are also interested in the mechanism of terminal erythroid differentiation.

Publications

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  • Korsgren C, Lux SE. The carboxyterminal EF domain of erythroid alpha-spectrin is necessary for optimal spectrin-actin binding. Blood 2010; 116:2600-7. PubMed
  • Williams DA, Porter ES, Lux SE, Grier HE, Mack JW, Orkin SH. Training program in cancer and blood diseases: Pediatric Hematology/Oncology Fellowship Program, Children's Hospital Boston/Dana-Farber Cancer Institute. Am J Hematol 2010; 85:793-4. PubMed
  • Tse WT, Tang J, Jin O, Korsgren C, John KM, Kung AL, Gwynn B, Peters LL, Lux SE. A new spectrin, beta IV, has a major truncated isoform that associates with promyelocytic leukemia protein nuclear bodies and the nuclear matrix. J Biol Chem 2001; 276:23974-85. PubMed
  • Gallagher PG, Romana M, Tse WT, Lux SE, Forget BG. The human ankyrin-1 gene is selectively transcribed in erythroid cell lines despite the presence of a housekeeping-like promoter. Blood 2000; 96:1136-43. PubMed
  • Donovan A, Brownlie A, Zhou Y, Shepard J, Pratt SJ, Moynihan J, Paw BH, Drejer A, Barut B, Zapata A, Law TC, Brugnara C, Lux SE, Pinkus GS, Pinkus JL, Kingsley PD, Palis J, Fleming MD, Andrews NC, Zon LI. Positional cloning of zebrafish ferroportin1 identifies a conserved vertebrate iron exporter. Nature 2000; 403:776-81. PubMed
  • Jensen LJ, Stuart-Tilley AK, Peters LL, Lux SE, Alper SL, Breton S. Immunolocalization of AE2 anion exchanger in rat and mouse epididymis. Biol Reprod 1999; 61:973-80. PubMed
  • Rasband MN, Peles E, Trimmer JS, Levinson SR, Lux SE, Shrager P. Dependence of nodal sodium channel clustering on paranodal axoglial contact in the developing CNS. J Neurosci 1999; 19:7516-28. PubMed
  • Berezovska O, Frosch M, McLean P, Knowles R, Koo E, Kang D, Shen J, Lu FM, Lux SE, Tonegawa S, Hyman BT. The Alzheimer-related gene presenilin 1 facilitates notch 1 in primary mammalian neurons. Brain Res Mol Brain Res 1999; 69:273-80. PubMed
  • Peters LL, Jindel HK, Gwynn B, Korsgren C, John KM, Lux SE, Mohandas N, Cohen CM, Cho MR, Golan DE, Brugnara C. Mild spherocytosis and altered red cell ion transport in protein 4. 2-null mice. J Clin Invest 1999; 103:1527-37. PubMed
  • Tse WT, Lux SE. Red blood cell membrane disorders. Br J Haematol 1999; 104:2-13. PubMed
  • Berezovska O, McLean P, Knowles R, Frosh M, Lu FM, Lux SE, Hyman BT. Notch1 inhibits neurite outgrowth in postmitotic primary neurons. Neuroscience 1999; 93:433-9. PubMed
  • Cho MR, Eber SW, Liu SC, Lux SE, Golan DE. Regulation of band 3 rotational mobility by ankyrin in intact human red cells. Biochemistry 1999; 37:17828-35. PubMed
  • Stankewich MC, Tse WT, Peters LL, Ch'ng Y, John KM, Stabach PR, Devarajan P, Morrow JS, Lux SE. A widely expressed betaIII spectrin associated with Golgi and cytoplasmic vesicles. Proc Natl Acad Sci U S A 1998; 95:14158-63. PubMed
  • Doctor RB, Chen J, Peters LL, Lux SE, Mandel LJ. Distribution of epithelial ankyrin (Ank3) spliceoforms in renal proximal and distal tubules. Am J Physiol 1998; 274:F129-38. PubMed
  • Gallagher PG, Tse WT, Scarpa AL, Lux SE, Forget BG. Structure and organization of the human ankyrin-1 gene. Basis for complexity of pre-mRNA processing. J Biol Chem 1997; 272:19220-8. PubMed
  • Hoock TC, Peters LL, Lux SE. Isoforms of ankyrin-3 that lack the NH2-terminal repeats associate with mouse macrophage lysosomes. J Cell Biol 1997; 136:1059-70. PubMed
  • Peters LL, Shivdasani RA, Liu SC, Hanspal M, John KM, Gonzalez JM, Brugnara C, Gwynn B, Mohandas N, Alper SL, Orkin SH, Lux SE. Anion exchanger 1 (band 3) is required to prevent erythrocyte membrane surface loss but not to form the membrane skeleton. Cell 1996; 86:917-27. PubMed
  • Eber SW, Gonzalez JM, Lux ML, Scarpa AL, Tse WT, Dornwell M, Herbers J, Kugler W, Ozcan R, Pekrun A, Gallagher PG, Schr. Ankyrin-1 mutations are a major cause of dominant and recessive hereditary spherocytosis. Nat Genet 1996; 13:214-8. PubMed
  • Lu FM, Lux SE. Constitutively active human Notch1 binds to the transcription factor CBF1 and stimulates transcription through a promoter containing a CBF1-responsive element. Proc Natl Acad Sci U S A 1996; 93:5663-7. PubMed
  • Jenkins PB, Abou-Alfa GK, Dhermy D, Bursaux E, F. A nonsense mutation in the erythrocyte band 3 gene associated with decreased mRNA accumulation in a kindred with dominant hereditary spherocytosis. J Clin Invest 1996; 97:373-80. PubMed
  • Joiner CH, Franco RS, Jiang M, Franco MS, Barker JE, Lux SE. Increased cation permeability in mutant mouse red blood cells with defective membrane skeletons. Blood 1995; 86:4307-14. PubMed
  • Piepenhagen PA, Peters LL, Lux SE, Nelson WJ. Differential expression of Na(+)-K(+)-ATPase, ankyrin, fodrin, and E-cadherin along the kidney nephron. Am J Physiol 1995; 269:C1417-32. PubMed
  • Morris MB, Lux SE. Characterization of the binary interaction between human erythrocyte protein 4.1 and actin. Eur J Biochem 1995; 231:644-50. PubMed
  • Peters LL, John KM, Lu FM, Eicher EM, Higgins A, Yialamas M, Turtzo LC, Otsuka AJ, Lux SE. Ank3 (epithelial ankyrin), a widely distributed new member of the ankyrin gene family and the major ankyrin in kidney, is expressed in alternatively spliced forms, including forms that lack the repeat domain. J Cell Biol 1995; 130:313-30. PubMed
  • White RA, Geissler EN, Adkison LR, Dowler LL, Alper SL, Lux SE. Chromosomal location of the murine anion exchanger genes encoding AE2 and AE3. Mamm Genome 1994; 5:827-9. PubMed
  • Platt OS, Lux SE, Falcone JF. A highly conserved region of human erythrocyte ankyrin contains the capacity to bind spectrin. J Biol Chem 1993; 268:24421-6. PubMed
  • Savvides P, Shalev O, John KM, Lux SE. Combined spectrin and ankyrin deficiency is common in autosomal dominant hereditary spherocytosis. Blood 1993; 82:2953-60. PubMed
  • Becker PS, Tse WT, Lux SE, Forget BG. Beta spectrin kissimmee: a spectrin variant associated with autosomal dominant hereditary spherocytosis and defective binding to protein 4.1. J Clin Invest 1993; 92:612-6. PubMed
  • Birkenmeier CS, White RA, Peters LL, Hall EJ, Lux SE, Barker JE. Complex patterns of sequence variation and multiple 5' and 3' ends are found among transcripts of the erythroid ankyrin gene. J Biol Chem 1993; 268:9533-40. PubMed
  • Wang CC, Badylak JA, Lux SE, Moriyama R, Dixon JE, Low PS. Expression, purification, and characterization of the functional dimeric cytoplasmic domain of human erythrocyte band 3 in Escherichia coli. Protein Sci 1992; 1:1206-14. PubMed
  • Peters LL, White RA, Birkenmeier CS, Bloom ML, Lux SE, Barker JE. Changing patterns in cytoskeletal mRNA expression and protein synthesis during murine erythropoiesis in vivo. Proc Natl Acad Sci U S A 1992; 89:5749-53. PubMed
  • Peters LL, Birkenmeier CS, Bronson RT, White RA, Lux SE, Otto E, Bennett V, Higgins A, Barker JE. Purkinje cell degeneration associated with erythroid ankyrin deficiency in nb/nb mice. J Cell Biol 1991; 114:1233-41. PubMed
  • Tse WT, Menninger JC, Yang-Feng TL, Francke U, Sahr KE, Lux SE, Ward DC, Forget BG. Isolation and chromosomal localization of a novel nonerythroid ankyrin gene. Genomics 1991; 10:858-66. PubMed
  • Becker PS, Schwartz MA, Morrow JS, Lux SE. Radiolabel-transfer cross-linking demonstrates that protein 4.1 binds to the N-terminal region of beta spectrin and to actin in binary interactions. Eur J Biochem 1990; 193:827-36. PubMed
  • Costa FF, Agre P, Watkins PC, Winkelmann JC, Tang TK, John KM, Lux SE, Forget BG. Linkage of dominant hereditary spherocytosis to the gene for the erythrocyte membrane-skeleton protein ankyrin. N Engl J Med 1990; 323:1046-50. PubMed
  • Lux SE, Tse WT, Menninger JC, John KM, Harris P, Shalev O, Chilcote RR, Marchesi SL, Watkins PC, Bennett V. Hereditary spherocytosis associated with deletion of human erythrocyte ankyrin gene on chromosome 8. Nature 1990; 345:736-9. PubMed
  • White RA, Birkenmeier CS, Lux SE, Barker JE. Ankyrin and the hemolytic anemia mutation, nb, map to mouse chromosome 8: presence of the nb allele is associated with a truncated erythrocyte ankyrin. Proc Natl Acad Sci U S A 1990; 87:3117-21. PubMed
  • Lux SE, John KM, Bennett V. Analysis of cDNA for human erythrocyte ankyrin indicates a repeated structure with homology to tissue-differentiation and cell-cycle control proteins. Nature 1990; 344:36-42. PubMed
  • Lux SE, John KM, Kopito RR, Lodish HF. Cloning and characterization of band 3, the human erythrocyte anion-exchange protein (AE1). Proc Natl Acad Sci U S A 1989; 86:9089-93. PubMed
  • Davies KA, Lux SE. Hereditary disorders of the red cell membrane skeleton. Trends Genet 1989; 5:222-7. PubMed
  • Davis L, Lux SE, Bennett V. Mapping the ankyrin-binding site of the human erythrocyte anion exchanger. J Biol Chem 1989; 264:9665-72. PubMed
  • Becker PS, Morrow JS, Lux SE. Abnormal oxidant sensitivity and beta-chain structure of spectrin in hereditary spherocytosis associated with defective spectrin-protein 4.1 binding. J Clin Invest 1987; 80:557-65. PubMed
  • Becker PS, Cohen CM, Lux SE. The effect of mild diamide oxidation on the structure and function of human erythrocyte spectrin. J Biol Chem 1986; 261:4620-8. PubMed
  • Beaven GH, Jean-Baptiste L, Ungewickell E, Baines AJ, Shahbakhti F, Pinder JC, Lux SE, Gratzer WB. An examination of the soluble oligomeric complexes extracted from the red cell membrane and their relation to the membrane cytoskeleton. Eur J Cell Biol 1985; 36:299-306. PubMed
  • Platt OS, Falcone JF, Lux SE. Molecular defect in the sickle erythrocyte skeleton. Abnormal spectrin binding to sickle inside-our vesicles. J Clin Invest 1984; 75:266-71. PubMed
  • Ohanian V, Wolfe LC, John KM, Pinder JC, Lux SE, Gratzer WB. Analysis of the ternary interaction of the red cell membrane skeletal proteins spectrin, actin, and 4.1. Biochemistry 1984; 23:4416-20. PubMed
  • Spiegel JE, Beardsley DS, Southwick FS, Lux SE. An analogue of the erythroid membrane skeletal protein 4.1 in nonerythroid cells. J Cell Biol 1984; 99:886-93. PubMed
  • Wolfe LC, John KM, Falcone JC, Byrne AM, Lux SE. A genetic defect in the binding of protein 4.1 to spectrin in a kindred with hereditary spherocytosis. N Engl J Med 1982; 307:1367-74. PubMed
  • Mohandas N, Clark MR, Health BP, Rossi M, Wolfe LC, Lux SE, Shohet SB. A technique to detect reduced mechanical stability of red cell membranes: relevance to elliptocytic disorders. Blood 1982; 59:768-74. PubMed
  • Platt OS, Lux SE, Nathan DG. Exercise-induced hemolysis in xerocytosis. Erythrocyte dehydration and shear sensitivity. J Clin Invest 1981; 68:631-8. PubMed
  • Tomaselli MB, John KM, Lux SE. Elliptical erythrocyte membrane skeletons and heat-sensitive spectrin in hereditary elliptocytosis. Proc Natl Acad Sci U S A 1981; 78:1911-5. PubMed
  • Harris HW, Lux SE. Structural characterization of the phosphorylation sites of human erythrocyte spectrin. J Biol Chem 1980; 255:11512-20. PubMed
  • Harris HW, Levin N, Lux SE. Comparison of the phosphorylation of human erythrocyte spectrin in the intact red cell and in various cell-free systems. J Biol Chem 1980; 255:11521-5. PubMed
  • Lux SE. Dissecting the red cell membrane skeleton. Nature 1979; 281:426-9. PubMed
  • Glader BE, Lux SE, Muller-Soyano A, Platt OS, Propper RD, Nathan DG. Energy reserve and cation composition of irreversibly sickled cells in vivo. Br J Haematol 1978; 40:527-32. PubMed
  • Lux SE, John KM. Isolation and partial characterization of a high molecular weight red cell membrane protein complex normally removed by the spleen. Blood 1977; 50:625-41. PubMed
  • Lux SE, John KM. Evidence that spectrin is a determinant of shape and deformability in the human erythrocyte. Prog Clin Biol Res 1976; 17:481-91. PubMed
  • Lux SE, John KM, Karnovsky MJ. Irreversible deformation of the spectrin-actin lattice in irreversibly sickled cells. J Clin Invest 1976; 58:955-63. PubMed
  • Breslow JL, Spaulding DR, Lux SE, Levy RI, Lees RS. Homozygous familial hypercholesterolemia. A possible biochemical explanation of clinical heterogeneity. N Engl J Med 1975; 293:900-3. PubMed
  • Levy RI, Fredrickson DS, Stone NJ, Bilheimer DW, Brown WV, Glueck CJ, Gotto AM, Herbert PN, Kwiterovich PO, Langer T, LaRosa J, Lux SE, Rider AK, Shulman RS, Sloan HR. Cholestyramine in type II hyperlipoproteinemia. A double-blind trial. Ann Intern Med 1973; 79:51-8. PubMed
  • Gotto AM, Levy RI, Lux SE, Birnbaumer ME, Fredrickson DS. A comparaive study of the effects of chemical modification on the immunochemical and optical properties of human plasma low-density lipoprotein(s) and apoproteins. Biochem J 1973; 133:369-82. PubMed
  • Lux SE, John KM, Ronan R, Brewer HB. Isolation and characterization of the tryptic and cyanogen bromide peptides of apoLp-Gln-II (apoA-II), plasma high density apolipoprotein. J Biol Chem 1972; 247:7519-27. PubMed
  • Lux SE, Hirz R, Shrager RI, Gotto AM. The influence of lipid on the conformation of human plasma high density apolipoproteins. J Biol Chem 1972; 247:2598-606. PubMed
  • LaRosa JC, Levy RI, Herbert P, Lux SE, Fredrickson DS. A specific apoprotein activator for lipoprotein lipase. Biochem Biophys Res Commun 1970; 41:57-62. PubMed
  • Lux SE, Johnston RB, August CS, Say B, Penchaszadeh VB, Rosen FS, McKusick VA. Chronic neutropenia and abnormal cellular immunity in cartilage-hair hypoplasia. N Engl J Med 1970; 282:231-6. PubMed
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