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Original Articles

(Most recent publications listed first)

Brigham & Women’s Hospital Publications (Faculty Member Work):

84.  Seguin A, Takahashi-Makise N, Yien YY, Huston NC, Whitman JC, Musso G, Wallace JA, Bradley T, Bergonia H, Kafina MD, Matsumoto M, Igarashi K, Phillips JD, Paw BH, Kaplan J, Ward DM.  Reductions in the mitochondrial ABC transporter Abcb10 affect the transcriptional profile of heme biosynthesis genes.  J. Biol. Chem., 2017, in press.  (doi: 10.1074/jbc.M117.806901).  

83.  Yien YY, Ducamp S, van der Vorm LN, Kardon JR, Manceau H, Kannengieser C, Bergonia HA, Kafina MD, Karim Z, Gouya L, Baker TA*, Puy H*, Phillips JD*, Nicolas G, Paw BH*.  A mutation in human CLPX elevates levels of δ-aminolevulinate synthase and protoporphyrin IX to promote erythropoietic protoporphyria.  Proc. Natl. Acad. Sci. USA 2017; 114:E8045-E8052.  (doi:  10.1074/pnas.1700632114).  [*co-corresponding senior authors].

82.  Chung J, Wittig JG, Ghamari A, Maeda M, Dailey TA, Bergonia H, Kafina MD, Coughlin EE, Minogue CE, Hebert AS, Li L, Kaplan J, Lodish HF, Bauer DE, Orkin SH, Cantor AB, Maeda T, Phillips JD, Coon JJ, Pagliarini DJ, Dailey HA, Paw BH. Erythropoietin signaling regulates heme biosynthesis. eLife 2017; 6:e24767. (doi: 10.7554/eLife.24767).

81.  Grillo AS, SantaMaria AM, Kafina MD, Cioffi AG, Huston NC, Han M, Seo YA, Yien YY, Nardone C, Menon AV, Fan J, Svoboda DC, Anderson JB, Hong JD, Nicolau BG, Subedi K, Gewirth AA, Wessling-Resnick M*, Kim J*, Paw BH*, Burke MD*.  Restored iron transport by a small molecule promotes absorption and hemoglobinization in animals.  Science 2017; 356:608-616.  [*co-corresponding senior authors].

  • Issue Commentary:  Service RF.  Iron Man molecule restores balance to cells.  Science 2017 (doi: 10.1126/science.aal6678).
  • Issue Commentary:  Dahlmann HA.  Small molecule shuttles iron across cellular membranes.  ACS Chem. Biol. 2017; 12:1452-1454..

80.   Lichtenstein DA, Crispin AW, Sendamarai AK, Campagna DR, Schmitz-Abe K, Sousa CM, Kafina MD, Schmidt PJ, Niemeyer CM, Porter J, May A, Patnaik MM, Heeney MM, Kimmelman A, Bottomley SS, Paw BH, Markianos K, Fleming MD.  A recurring mutation in the respiratory complex 1 protein NDUFB11 is responsible for a novel form of x-linked sideroblastic anemia.  Blood 2016; 128:1913-1917.  

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  • Chosen by Editors of Blood as one of the top 10 papers published in 2016.

79.  Yien YY, Paw BH.  A role for iron deficiency in dopaminergic neurodegeneration.  Proc. Natl. Acad. Sci. USA 2016; 113:3417-3418.

78.  Anderson H, Patch TC, Reddy PNG, Hagedron EJ, Kim PG, Soltis KA, Chen MJ, Tamplin OJ, Frye M, MacLean GA, Hübner K, Bauer DE, Kanki JP, Vogin G, Huston NC, Nguyen M, Fujiwara Y, Paw BH, Vestweber D, Zon LI, Orkin SH, Daley GQ, Shah DI.  Hematopoietic stem cells develop in the absence of endothelial cadherin 5 expression.  Blood 2015; 126:2811-2820.  

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77.  Shmukler BE, Huston NC, Thon JN, Chih WN, Kourkoulis G, Lawson ND, Paw BH*, Alper SL*.  Homozygous knockout of the piezo1 gene in the zebrafish is not associated with anemia.  Haematologica 2015; 100(12):e483-485.  [*co-corresponding senior authors].

76.  Kardon JR, Yien YY, Huston NC, Branco DS, Hildick-Smith GJ, Rhee KY, Paw BH, Baker TA.  Mitochondrial ClpX unfoldase activates a key enzyme for heme biosynthesis and erythropoiesis.  Cell 2015; 161:858-867.

75.  Chung J, Bauer DE, Ghamari A, Nizzi CP, Deck KM, Kingsley PD, Yien YY, Huston NC, Chen C, Schultz IJ, Dalton AJ, Wittig JG, Palis J, Orkin SH, Lodish HF, Eisenstein RS, Cantor AB, Paw BH.  The mTORC1/4E-BP pathway coordinates hemoglobin production with L-leucine availabiltiy.  Science Signaling 2015; 8:ra35.  

  • Issue Commentaries:  Nathan DG (2015) Sci. Signal. 8, fs9;  Wong W, Science (2015) 348(6232), 300-C.
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74.  Yien YY, Robledo RF, Schultz IJ, Takahashi-Makise N, Gwynn B, Bauer DE, Dass A, Yi G, Li L, Hildick-Smith GJ, Cooney JD, Pierce EL, Mohler K, Dailey TA, Miyata N, Kingsley PD, Garone C, Hattangadi SM, Huang H, Chen W, Keenan EM, Shah DI, Schlaeger TM, DiMauro S, Orkin SH, Cantor AB, Palis J, Koehler CM, Lodish HF, Kaplan J, Ward DM, Dailey HA, Phillips JD, Peters LL, Paw BH.  TMEM14C is is required for erythroid mitochondrial heme metabolism.  J. Clin. Invest. 2014; 124:4294-4304.

  • “NIDDK February Document” featuring the paper as one of the key publications supported by the NIDDK, February 2015, page 114:http://www.niddk.nih.gov/about-niddk/strategic-plans-reports/Documents/Feb%20Doc%202015/NIDDK2015_RecentAdvances_508c.pdf

73.  Canver MC, Bauer DE, Dass A, Yien YY, Chung J, Masuda T, Maeda T, Paw BH, Orkin SH.  Characterization of genomic deletion efficiency mediated by CRISPR/Cas9 in mammalian cells.  J. Biol. Chem. 2014; 289:21312-21324.

  • Chosen by the Editors of JBC as one the best of papers published in 2014 (>21/4000 published papers).

72.  Shmukler BE, Reimold FR, Heneghan JF, Chen C, Zhao T, Paw BH, Alper SL.  Molecular cloning and functional characterization of zebrafish Slc4a3/Ae3 anion exchanger.  Pflügers Arch. 2014; 466:1605-1618.

71.  Chung J, Anderson SA, Gwynn B, Deck KM, Chen MJ, Langer NB, Shaw GC, Huston NC, Boyer LF, Datta S, Paradkar PN, Li L, Wei Z, Lambert AJ, Sahr K, Wittig JG, Chen W, Lu W, Galy B, Schlaeger TM, Hentze MW, Ward DM, Kaplan J, Eisenstein RS, Peters LL, Paw BH.  Iron response protein-1 protects against Mitoferrin1-deficient porphyria.  J. Biol. Chem. 2014; 289:7835-7843.

70.  Hildick-Smith GJ, Cooney JD, Garone C, Kremer L, Haack TB, Thon JN, Miyata N, Lieber DS, Calvo SE, Akman HO, Yien YY, Huston NC, Branco DS, Shah DI, Freedman ML, Koehler CM, Italiano JE, Merkenschlager A, Beblo S, Strom TM, Meitinger T, Freisinger P, Donati MA, Prokisch H, Mootha VK, DiMauro S, Paw BH.  Macrocytic anemia and mitochondriopathy resulting from a defect in sideroflexin 4.  Am. J. Hum. Genet. 2013; 93:906-914.

69.  Bayeva M, Khechaduri A, Wu R, Burke MA, Wasserstrom JA, Singh N, Liesa M, Shirihai OS, Langer NB, Paw BH, Ardehali H.  ABCB10 regulates early steps of heme synthesis.  Circ. Res. 2013; 113:279-287.

68.  Pei D, Luther W, Wang W, Paw BH, Stewart RA, George R.  Neuroblastoma-associated PHOX2B mutations impair sympathetic neuronal differentiation.  PLos Genet. 2013; 9(6):e1003533.

67.  Chen C, Garcia-Santos D, Li L, Ishikawa Y, Seguin A, Li L, Hattangadi SM, Fegan KH, Hildick-Smith GJ, Cooney JD, Chen W, King MJ, Schultz IJ, Dalton AJ, Kingsley PD, Palis J, Lodish HF, Ward DM, Kaplan J, Maeda T, Ponka P, Paw BH.  Snx3 regulates recycling of the transferrin receptor and iron assimilation.  Cell Metabolism 2013; 17:343-352.  

  • Issue Highlights

66.  Harris JM, Esain V, Frechette GM, Harris LJ, Cox AG, Cortes M, Garnaas MK, Carroll KJ, Cutting CC, Khan T, Elks PM, Renshaw SA, Dickinson BC, Chang CJ, Murphy MP, Paw BH, Vander Heiden MG, Goessling W, North TE.  Glucose metabolism impacts the spatio-temporal onset and magnitude of HSC induction in vivo.  Blood 2013; 121:2483-2493.

65.  Cooney JD, Hildick-Smith GJ, Shafizadeh E, McBride PF, Carroll KJ, Anderson H, Shaw GC, Tamplin OJ, Shah DI, Dalton AJ, Wong C, Gallagher PG, Zon LI, North TE, Paw BH.  Teleost Growth Factor Independence (gfi) genes differentially regulate successive waves of hematopoiesis.  Dev. Biol. 2013; 373:431-441.

64.  Shah DI, Takahashi-Makise N, Cooney JD, Li L, Schultz IJ, Pierce EL, Narla A, Seguin A, Hattangadi SM, Medlock AE, Langer NB, Dailey TA, Hurst SN, Faccenda D, Wiwczar J, Heggers SK, Vogin G, Chen W, Chen C, Campagna DR, Brugnara C, Zhou Y, Ebert BL, Danial NN, Fleming MD, Ward DM, Campanella M, Dailey HA, Kaplan J, Paw BH.  Mitochondrial Atpif1 regulates heme synthesis in developing erythroblasts.  Nature 2012; 491:608-612.

63.  Blanc L, Ciciotte SL, Gwynn B, Hildick-Smith GJ, Pierce EL, Soltis KA, Cooney JD, Paw BH, Peters LL.  Critical function of Rasa3 in vertebrate erythropoiesis and megakaryopoiesis.  Proc. Natl. Acad. Sci. USA 2012; 109:12099-12104.

62.  Payne EM, Virgilio M, Narla A, Sun H, Levine M, Paw BH, Berliner N, Look AT, Ebert EL, Khanna-Gupta A.  L-leucine improves anemia and developmental defects associated with Diamond-Blackfan anemia and del(5q) MDS by activating mTOR pathway.  Blood 2012; 120:2214-2224.

61.  Leshchiner I, Alexa K, Kelsey PJ, Adzhubey I, Austin CA, Cooney JD, Anderson H, King MJ, Stottman R, Ha S, Drummond IA, Paw BH, North TE, Beier DR, Goessling W, Sunyae S.  Mutation mapping and identification by whole genome sequencing.  Genome Research 2012; 22:1541-1548.

60.  Wang L, He F, Bu J, Liu X, Du W, Dong J, Cooney JD, Dubey SK, Shi Y, Gong B, Li J, McBride PF, Jia Y, Lu F, Soltis KA, Lin Y, Namburi P, Liang C, Sundaresan P, Paw BH, Li DY, Phillips JD, Yang Z.  ABCB6 mutations cause ocular colobama.  Am. J. Hum. Genet. 2012; 90:40-48.

59.  Wang Y, Langer NB, Shaw GC, Yang G, Li L, Kaplan J, Paw BH*, Bloomer JR*.  Abnormal Mitoferrin-1 expression in patients with erythropoietic protoporphyria.  Exp. Hematol. 2011; 39:784-794.  [*co-corresponding senior authors].

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58.  Troadec MB, Warner D, Wallace J, Thomas K, Spangrude G, Phillips J, Khalimonchuk O, Paw BH, Ward DM, Kaplan J.  Targeted deletion of the mouse mitoferrin 1 gene:  from anemia to protoporphyria.  Blood 2011; 117:5494-5502.  

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57.  Amigo JD, Ming Y, Troadec MB, Gwynn B, Cooney JD, Lambert AJ, Chi NC, Weiss MJ, Peters LL, Kaplan J, Cantor AB, Paw BH.  Identification of distal cis-regulatory elements at the mouse mitoferrin loci using zebrafish transgenesis.  Mol. Cell. Biol. 2011; 31:1344-1356.  

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56.  Saslowsky DE, Cho JA, Chinnapen H, Massol RH, Chinnapen DJF, Wagner JS, DeLuca HE, Kam W, Paw BH, Lencer WI.  Intoxication of zebrafish and mammalian cells by cholera toxin depends on the flotillin/reggie proteins, but not Derlin-1 or -2.  J. Clin. Invest. 2010; 120:4399-4409.

55.  Mardini L, Gasiorek J, Derjuga A, Carriere L, Paw BH, Schranzhofer M, Ponka P, Blank V.  Antagonistic roles of ERK and p38 MAP kinases in globin expression, heme biosynthesis and iron uptake in erythroid cells.  Biochem. J. 2010; 432:145-151.

54.  Yu D, dos Santos CO, Zhao G, Jiang J, Amigo JD, Khandros E, Dore LC, Yao Y, D’Souza J, Zhang Z, Ghaffari S, Choi J, Friend S, Wei T, Orange JS, Paw BH, Weiss MJ.  miR-451 protects against erythroid oxidant stress by repressing 14-3-3ζ.  Genes Dev. 2010; 24:1620-1633.

53.  Chen W, Dailey HA, Paw BH.  Ferrochelatase forms an oligomeric complex with mitoferrin1 and Abcb10 for erythroid heme biosynthesis.  Blood 2010; 11:628-630.

52.  Sun X, Hoage T, Bai P, Ding Y, Chen Z, Zhang R, Jahangir J, Paw BH, Li Y, Xu X.  Cardic remodeling involves both cardiomyocyte hypertrophy and hyperplasia in anemic zebrafish.  PLoS One 2009; 4(8):e6596.

51.  Amigo JD, Ackermann GE, Cope JJ, Yu M, Ma DD, Cooney J, Langer NB, Shafizadeh E, Shaw GC, Horsely W, Trede NS, Davidson AJ, Barut BA, Wojiski SA, Traver D, Moran TB, Kourkoulis G, Hsu K, Kanki J, Shah DI, Lin HF, Handin RI, Cantor AB, Paw BH.  The role and regulation of Friend of GATA-1 (FOG-1) in hematopoietic development of the zebrafish.  Blood 2009; 114:4654-4663.  

  • Cover Illustration.

50.  Chen W, Paradkar PN, Li L, Pierce EL, Langer NB, Takahashi-Makise N, Hyde BB, Shirihai OS, Ward DM, Kaplan J*, Paw BH*.  Abcb10 physically interacts with mitoferrin1 (slc25a37) to enhance its stability and function in the erythroid mitochondria. Proc. Natl. Acad. Sci. USA 2009; 106:16263-16268. [*co-corresponding senior authors].  

  • Issue Commentary:  Andrews NC.  PNAS 2009; 106:16263-16268.

49.  Schwarz K, Iolascon A, Verissimo F, Trede NS, Horsley W, Chen W, Paw BH, Hopfner KP, Holzmann K, Russo R, Esposito MR, Spano D, De Falco L, Heinrich K, Joggerst B, Rojewski MT, Perotta S, Denecke J, Pannicke U, Delaunay J, Pepperkok R, Heimpel H.  Mutations affecting the secretory COPII coat component SEC23B cause congenital dyserytrhopoietic anemia type II.  Nat. Genet. 2009; 41:936-940.

48.  Nilsson R, Schultz IJ, Pierce EL, Soltis KA, Naranuntarat A, Ward DM, Baughman J, Paradkar PN, Kingsley PD, Culotta VC, Kaplan J, Palis J, Paw BH*, Mootha VK*.  Discovery of genes essential for heme biosynthesis through large-scale gene expression analysis. Cell Metabolism 2009; 10:119-130.  *[co-corresponding senior authors]

  • Issue Commentary:  Rouault TA, Tong WH.  Cell Metab. 2009; 10:80-81.

47.  Johnson HJ, Gandhi MJ, Shafizadeh E, Langer LB, Pierce EL, Paw BH, Gilligan DM, Drachman JG.  In vivo inactivation of MASTL kinase results in thrombocytopenia.  Exp. Hematol. 2009; 37:901-908.

46.  Stachura DL, Reyes JR, Bartunek P, Paw BH, Zon LI, Traver D.  Zebrafish kidney stromal cell lines support multilineage hematopoiesis.  Blood 2009; 114:279-289.

45.  Paradkar PN, Zumbrennen KB, Paw BH, Ward DM, Kaplan J. Regulation of mitochondrial iron import through differential turnover of mitoferrin1 and mitoferrin2.  Mol. Cell. Biol. 2009; 29:1007-1016.

44.  Freeman JL, Ceol C, Feng H, Langenau DM, Belair C, Stern HM, Song A, Paw BH, Look AT, Zhou Y, Zon LI, Lee C.  Construction and application of a zebrafish array CGH platform.  Genes Chromosomes Cancer 2009; 48:155-170.

43.  Dobson JT, Seibert J, Teh EM, Daas S, Fraser RB, Paw BH, Lin TJ, Berman JN.  Carboxypeptidase A5 identifies a novel mast cell lineage in the zebrafish providing new insight into mass cell fate determination.  Blood 2008; 112:2969-2972.

42.  Trede NS, Ota T, Kawasaki H, Paw BH, Katz T, Demarest B, Hutchinson S, Zhou Y, Hersey C, Zapata A, Amemiya CT, Zon LI.  Zebrafish mutants with disrupted early T cell and thymus development identified in early pressure screen.  Dev. Dynamics 2008; 237:2575-2584.

41.  Dooley KA, Fraenkel P, Langer NB, Schmid B, Davidson AJ, Weber G, Chiang K, Foot H, Dwyer C, Wingert RA. Zhou Y, Paw BH, Zon LI; Tübingen 2000 Screen Consortium.  montalcino, a zebrafish model for variegate porphyria.  Exp. Hematol. 2008; 36:1132-1142.

40.  Rajagopal A, Rao AU, Amigo J, Tian M, Upadhyay SK, Hall C, Uhm S, Mathew MK, Fleming MD, Paw BH, Krause M, Hamza I.  Haem homeostasis is regulated by the conserved and concerted functions of the HRG-1 proteins.  Nature 2008; 453:1127-1131.

39.  Woo A, Moran TB, Schindler Y, Choe SK, Langer NB, Sullivan MR, Fujiwara Y, Paw BH, Cantor AB.   Identification of ZBP-89 as a novel GATA-1 associated transcription factor involved in megakaryocytic and erythroid development.  Mol. Cell. Biol. 2008; 28:2675-2689.

38.  Dore LC, Amigo JD, dos Santos CO, Zhang Z, Gai X, Tobias JW, Yu D, Dorman C, Wu W, Hardison RC, Paw BH*, Weiss MJ*.  A gata-1 regulated microRNA locus essential for erythropoiesis. Proc. Natl. Acad. Sci. USA 2008; 105:3333-3338.  [*co-corresponding senior authors].

37.  Shmukler BE, Clark JS, Hsu A, Vandorpe DH, Stewart AK, Kurschat CE, Choe SK, Zhou Y, Amigo J, Paw BH, Alper SL.  Zebrafish Ae2.1 encodes a second slc4a2 anion exchanger.  Am. J. Physiol. Regul. Integrat. Comp. Physiol. 2008; 294:1081-1091.

36.  Trede NS, Medenbach J, Damianov A, Hung LH, Weber GJ, Paw BH, Zhou Y, Hersey C, Zapata A, Keefe M, Barut BA, Stuart AB, Katz T, Amemiya CT, Zon LI, Bindereif A.  Network of coregulated spliceosome components revealed by zebrafish mutant in recycling factor p110.  Proc. Natl. Acad. Sci. USA  2007; 104:6608-6613.

35.  Paffett-Lugassy N, Hsia N, Fraenkel PF, Paw BH, Leshinksy I, Barut BA, Bahary N, Caro J, Handin RI, Zon LI.  Functional conservation of erythropoietin signaling in zebrafish.  Blood 2007; 110:2718-1726.

34.  Shaw GC, Cope JJ, Li L, Corson K, Hersey C, Wingert R, Ackermann GE, Gwynn B, Lambert AJ, Traver D, Trede NS, Barut BA, Minet E, Zhou Y, Donovan A, Brownlie A, Balzan R, Weiss MJ, Peters LL, Kaplan J, Zon LI, Paw BH.  Mitoferrin is essential for erythroid iron assimilation. Nature 2006; 440:96-100.

33.  Lee YM, Cope JJ, Ackermann GE, Goishi K, Armstrong EJ, Paw BH, Bischoff J.  Vascular endothelial growth factor receptor signaling is required for cardiac valve formation in zebrafish.  Dev. Dynamics 2006; 235:29-37.

32.  Lin HF, Traver D, Zhu H, Dooley K, Paw BH, Zon LI, Handin RI.  Analysis of thrombocyte development in CD41-GFP transgenic zebrafish.  Blood 2005; 106:3803-3810.

31.  Shmukler BE, Kurschat Ce, Ackermann GE, Jiang L, Zhou Y, Barut B, Stuart-Tilley AK, Drummond IA, Vandorpe DH, Paw BH, Alper SE.   Zebrafish slc4a2/ae2 anion exchanger: cDNA cloning, mapping, functional characterization and localization.  Am. J. Physiol. Renal Physiol. 2005; 289:F835-F849.

30.  Wingert RA, Galloway JL, Barut BA, Foot H, Fraenkel P, Axe JL, Weber G, Dooley K, Davidson AJ, Paw BH, Shaw GC, Kingsley P, Palis J, Schubert H, Chen O, Kaplan J, Tübingen 2000 Screen Consortium, Zon LI.  Deficiency of glutaredoxin 5 reveals Fe/S clusters are required for vertebrate heme synthesis.  Nature 2005; 436:1035-1039.

Boston Children’s Hospital Publications (Post-doctoral Work):

29.  Hsu K, Traver D, Kutok JL, Hagen A, Liu TX, Paw BH, Rhodes J, Berman J, Zon LI, Look AT.  The pu.1 promoter drives myeloid gene expression in zebrafish.  Blood 2004; 104:1291-1297.

28.  Altmann SM, Mellon MT, Johnson MC, Paw BH, Trede NS, Zon L.I, Kim CH.  Cloning and Characterization of an Mx Gene and its Corresponding Promoter from the Zebrafish, Danio rerio.  Dev. Comp. Immunol.  2003; 28:295-306.

27.  Traver D, Paw BH, Poss KD, Penberthy WT, Lin S, Zon LI.  Transplatation and in vivo Imaging of Multilineage Engraftment in Zebrafish Bloodless Mutants.  Nat. Immunol. 2003; 4:1238-1246.

26.  Ward AC, McPhee DO, Condron MM, Varma S, Cody SH, Onnebo SM, Paw BH, Zon LI, Lieschke GJ.  The Zebrafish spi1 Promoter Drives Myeloid-specific Expression in Stable Transgenic Fish.  Blood  2003; 102:3238-3240.

25.  Paw BH, Davidson AJ, Zhou Y, Li R, Pratt SJ, Lee C, Trede NS, Brownlie A, Donovan A, Liao EC, Ziai JM, Drejer A, Guo W, Kim C, Gwynn B, Peters L, Chernova M, Alper S, Zapata A, Wickramasinghe SN, Lee MJ, Lux SE, Fritz A, Postlethwait JH, Zon LI.  Cell-specific Mitotic Defect and Dyserythropoiesis Associated with Erythroid Band 3 Deficiency.  Nat. Genet. 2003; 34:59-64.

24.  Brownlie A, Hersey C, Oates AC, Paw BH, Falick AM, Witkowska HE, Flint J, Higgs D, Jessen J, Bahary N, Zhu H, Lin S, Zon LI.  Characterization of Embryonic Globin Genes of the Zebrafish, Dev. Biol. 2003; 255:48-61.

23.  Donovan A, Brownlie A, Dorschner MO, Zhou Y, Pratt SJ, Paw BH, Phllips RB, Thisse C, Thisse B, Zon LI.  Identification of a Zebrafish Mutant in the Iron Transporter Divalent Metal Transporter 1 (DMT1).  Blood 2002; 100:4655-4659.

22.  Shafizadeh E, Paw BH, Foott H, Liao EC, Barut BA, Cope JJ, Zon LI, Lin S.  Characterization of zebrafish merlot/chablis as a non-mammalian vertebrate model for  severe hereditary elliptocytosis due to Protein 4.1 deficiency.  Development  2002; 129:4359-4370.

21.  LieschkeGJ, Oates AC, Paw BH, Thompson MA, Hall NE, Ward AC, Ho RK, Zon LI, Layton JE.  A Zebrafish spi-1 (pu.1)  Orthologue Marks a Rostral Site of Early Myeloid Commitment Independent of Ventro-caudal Determinants of Erythroid Fate.  Dev. Biol. 2002; 264:274-295.

20.  Bennett CM, Kanki JP, Rhodes J, Liu TX, Paw BH, Kieran MW, Langenau D, Delahaye-Brown A, Zon LI, Fleming MD, Look TA.  Myelopoiesis in the Zebrafish, Danio rerio.  Blood 2001; 98: 643-651.

19.  Liao EC, Paw BH, Peters LL, Zapata A, Pratt SJ, Do CP, Lieschke G, Zon LI.  Hereditary Spherocytosis in Zebrafish riesling Illustrates Evolution of Erythroid β-spectrin Structure, and Function in Red Cell Morphogenesis and Membrane Stability.  Development  2000; 127:5123-5132.

18.  Parichy DM, Ransom D, Paw BH, Zon LI, Johnson SL.  An Ortholog of the kit-related Gene fms  is Require for Development of Neural Crest-derived Xanthophores and a Subpopulation of Adult Melanocytes in the Zebrafish, Danio rerio.  Development 2000; 127:3031-3044.

17.  Donovan A, Brownlie A, Zhou Y, Shepard J, Pratt SJ, Moynihan J, Paw BH, Drejer A, Barut B, Law T, Brugnara C, Lux SE, Pinkus GS, Pinkus JL, Kingsley PD, Palis J, Fleming M, Andrews NC, Zon LI.  Positional Cloning of Zebrafish ferroportin 1 Identifies a Conserved Vertebrate Iron Exporter.  Nature  2000; 403:776-781.

16.  Hukriede NA, Joly L, Tsang M, Miles J, Tellis P, Epstein JA, Barbazuk WB, Li F, Paw BH, Postlethwait JH, Hudson TJ, Zon LI, McPherson JD, Chevrette M, Dawid IB, Johnson SL, Ekker M.  Radiation Hybrid Mapping of the Zebrafish Genome.  Proc. Natl. Acad. Sci. USA 1999; 96:9745-9750.

15.  Oates AC, Wollberg P, Pratt SJ, Paw BH, Johnson SL, Ho RH, Postlethwait JH, Zon LI, Wilks A.  Zebrafish Stat3 is Expressed in Restricted Tissues During Embryogenesis and Stat1 Rescues Cytokine Signaling in a STAT1 Deficient Human Cell Line.  Dev. Dyn. 1999; 215:352-370.

14.  Oates AC, Brownlie A, Pratt SJ, Irvine DV, Liao EC, Paw BH, Dorian K, Johnson SL, Postlethwait JH, Zon LI, Wilks AF.  Gene Duplication of Zebrafish JAK2 Homologs is Accompanied by Divergent Embryonic Expression Patterns; Only Jak2A is Expressed during Erythropoiesis.  Blood  1999; 94:2622-2636.

13.  Brownlie A, Donovan A, Pratt S, Paw BH, Oates AC, Brugnara C, Witkowska E, Sassa S, Zon LI.  Positional Cloning of the Zebrafish sauternes Gene: A Model of Congenital Sideroblastic Anemia.  Nat. Genet.  1998; 20:244-250.

12.  Kwok C, Korn RM, Davis ME, Burt DW, Critcher R, McCarthy L, Paw BH, Zon LI, Goodfellow PN, Schmitt K.  Characterization of Whole Genome Radiation Hybrid Mapping Resources for Non-mammalian Vertebrates.  Nucleic Acid Res. 1998; 26:3562-3566.

11.  Thompson MA, Ransom D, Pratt SJ, MacLennan H, Kieran MW, Detrich HW, Vail B, Huber TL, Paw BH, Brownlie AJ, Oates AC, Fritz A, Gates M, Amores A, Bahary N, Talbot W, Her H, Beier DC, Postlethwait JH, Zon LI.  The cloche  and spadetail  Genes Differentially Affect Hematopoiesis and Vasculogenesis.  Dev. Biol.  1998; 197:248-269.

10.  Jessen JR, Meng A, McFarlane RJ, Paw BH, Zon LI, Lin S.  Modification of Bacterial Artificial Chromosomes Through Chi-Stimulated Homologous Recombination and its Application in Zebrafish Transgenesis.  Proc. Natl. Acad. Sci. USA  1998; 95:5121-5126.

9.   Liao EC, Paw BH, Oates AC, Pratt SJ, Postlethwait JH, Zon LI.  SCL/tal-1 Transcription Factor Acts Downstream of cloche to Specify Hematopoietic and Vascular Progenitors in Zebrafish.  Genes Dev.  1998; 12:621-626.

8.   Postlethwait JH, Yan Y-L, Gates M, Horne S, Amores A, Brownlie A, Donovan A, Egan E, Force A, Gong Z, Goutel C, Fritz A, Kelsh R, Knapik E, Liao E, Paw BH, Ransom D, Singer A, Thompson M, Beier D, Joly J, Larhammar D, Rosa F, Westerfield M, Zon LI, Johnson S, Talbot W.  Vertebrate Genome Evolution and the Zebrafish Gene Map.  Nat. Genet.  1998; 18:345-349.

UCLA Publications (Graduate Work):

7.  Paw BH, Wood LC, Neufeld EF.  A Third Mutation in the CpG Dinucleotide of Codon 504 and a Silent Mutation in Codon 506 of the HEX A Gene.  Am. J. Hum. Genet.  1991; 48:1139-1146.

6.  Paw BH, Tieu PT, Kaback MM, Lim J, Neufeld EF.  Frequency of Three HEX A Alleles Among Jewish and non-Jewish Carriers Identified in a Tay-Sachs Screening Program.  Am. J. Hum. Genet.  1990; 47:698-705.

5.  Paw BH, Moskowitz SM, Uhrhammer N, Wright N, Kaback MM, Neufeld EF.  Juvenile GM2 Gangliosidosis Caused by Substitution of Histidine for Arginine at Position 499 or 504 of the α-Subunit of β-Hexosaminidase.  J. Biol. Chem. 1990; 265:9452-9457.

4.  Paw BH, Kaback MM, Neufeld EF.  Molecular Basis of Adult-onset and Chronic GM2 Gangliosidoses in Patients of Ashkenazi Jewish Origin: Substitution of Serine for Glycine in Position 269 of the α-Subunit of β-Hexosaminidase.  Proc. Natl. Acad. Sci. USA  1989; 86: 2413-2417.

3.  Paw BH, Neufeld EF.  Normal Transcription of the α-Hexosaminidase β-Chain Gene in the Ashkenazi Tay-Sachs Mutation.  J. Biol. Chem. 1988; 263:3012-3015.

UC Berkeley Publications (Undergraduate Work):

2.  Coy M, Paw BH, Neilands JB.  Isolation and Properties of N-Hydroxylysine: Acetyl  Coenzyme-A Transacetylase from E. coli (pABN12).  Biochemistry 1986; 25:2485-2489.

 1.  DeLorenzo V, Bindereif A, Paw BH, Neilands JB.  Expression & Mapping of the  Aerobactin Biosynthesis & Transport Genes of Plasmid ColV-K30 in E. coli K12. J.  Bacteriol. 1986; 165:570-578.

 

 

Reviews:

  1. Neilands JB, Konopka K, Schwyn B, Coy M, Francis R, Paw BH, Bagg A.  In Winkelmann G, Van der Helm D, Neilands JB, eds. Iron Transport Microbes, Plants and Animals. New York: VCH Press, 1987:1-33.
  2. Zon LI, Kieran MW, Paw BH, Thompson MA, Ransom D, Brownlie A, Chan FY, Barone L, MacLennan H, Zieglar S, Pratt SJ, Detrich HW.  The Zebrafish: a New Model for Studying Embryonic Hematopoiesis.  In Gluckman E, Coulombel L, eds. Ontogeny of Hematopoiesis.  Aplastic Anemia. Colloque INSERM/John Libbey Eurotext, 1995;235:17-22.
  3. Paw BH, Zon LI.  Primary Fibroblast Cell Culture.  In Detrich W, Zon LI, Westerfield M, eds. Methods in Cell Biology.  San Diego:  Academic Press, 1999;59:39-43.
  4. Paw BH, Zon LI.  Zebrafish: a Genetic Approach in Studying Hematopoiesis.  Curr Opinion Hematol.  2000; 7:79-84.
  5. Paw BH.  Cloning of the Zebrafish retsina Blood Mutation:  A Genetic Model for Dyserythropoiesis and Erythroid Cytokinesis.  Blood Cells Mol Dis 2001;27,62-64. (Meeting Abstract).
  6. Ackermann GE, Paw BH.  Zebrafish:  Genetic Model for Studying Organogenesis and  Human Disorders, Frontiers Bioscience, 2003;8,d1227-1253.
  7. Shafizadeh E, Paw BH.  Zebrafish as a model of human hematologic disorders.  Curr Opinion Hematol.2004;11,255-261.
  8. Paw BH, Choe SK, Costa FC, Sundar SV, Peterson K.  Vertebrate Models for Sickle Cell Disease Research.  In Renaissance of Sickle Cell Disease Research in the Genomic Era. (Betty Pace, ed.)  World Scientific & Imperial College Press, London, UK, 2006:309-336.
  9. Paw BH, Zon LI.  Primary Fibroblast Cell Culture.  In Detrich W, Zon LI, Westerfield M, eds.  Methods in Cell Biology (2nd Edition).  San Diego:  Academic Press.  2009;1,27-32.
  10. Schultz IJ, Chen C, Paw BH*, Hamza I*.  Iron and porphyrin trafficking in heme biogenesis. J. Biol. Chem. 2010;285,26753-9.  [*co-corresponding senior authors].
  11. Chen C, Paw BH.   Cellular and mitochondrial iron homeostasis in vertebrates.  Biochim. Biophys. Acta – Molecular Cell Research 2012;1823,1459-1467.
  12. Chung J, Chen C, Paw BH.  Heme metabolism and erythropoiesis.  Curr. Opinion Hematol., 2012;19,156-162.
  13. Peters LL, Paw BH, Blanc L.  The scat mouse model highlights RASA3, a GTPase activating protein, as a key regulator of vertebrate erythropoiesis and megakaryopoiesis.  Small GTPases 2012;4(1),1-4.
  14. Yien YY, Paw BH.  Iron and heme transport and trafficking.  In Metals in Cells (Culotta VC, Scott RA. eds).  Chichester, UK: John Wiley & Sons, Ltd., 2013; 113-129.
  15. Shah DI, Paw BH.  Zebrafish as a genetic model of hematopoiesis and human red blood cell diseases.  In Handbook of Porphyrin Science (Ferreira GC, Kadish KM, Smith KM, Guilard R, eds.)  Singapore:  World Scientific Press, 2013; 27:85-115.
  16. Yien YY, Ringel AR, Paw BH.  Mitochondrial transport of protoporphyrinogen IX in erythroid cells.  Oncotarget 2015; 6:20742-20743.
  17. van der Vorm L, Paw BH.  Studying disorders of vertebrate iron and heme metabolism using zebrafish.  In The Zebrafish: Disease Models and Chemical Screens (4th Edition), (Detrich HW, Westerfield M, Zon LI, eds).  Methods Cell Biology 2017; 138:193-220.
  18. Kafina MD, Paw BH.  Using the zebrafish model system to study erythropoiesis.  In Methods in Molecular Biology: Erythropoiesis – Methods and Protocols.  (Lloyd JA, ed).  Springer, New York, NY, 2016; in press.
  19. Kafina MD, Paw BH.  Intracellular iron and heme trafficking and metabolism in developing erythroblasts.  Metallomics 2017; 9:1193-1203.
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