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Items: 1 to 50 of 95

1.

FAM210B is an erythropoietin target and regulates erythroid heme synthesis by controlling mitochondrial iron import and ferrochelatase activity.

Yien YY, Shi J, Chen C, Cheung JTM, Grillo AS, Shrestha R, Li L, Zhang X, Kafina MD, Kingsley PD, King MJ, Ablain J, Li H, Zon LI, Palis J, Burke MD, Bauer DE, Orkin SH, Koehler CM, Phillips JD, Kaplan J, Ward DM, Lodish HF, Paw BH.

J Biol Chem. 2018 Dec 21;293(51):19797-19811. doi: 10.1074/jbc.RA118.002742. Epub 2018 Oct 26.

PMID:
30366982
2.

The role of ClpX in erythropoietic protoporphyria.

Whitman JC, Paw BH, Chung J.

Hematol Transfus Cell Ther. 2018 Apr-Jun;40(2):182-188. doi: 10.1016/j.htct.2018.03.001. Epub 2018 Mar 28. Review.

3.

Using the Zebrafish as an Approach to Examine the Mechanisms of Vertebrate Erythropoiesis.

Kafina MD, Paw BH.

Methods Mol Biol. 2018;1698:11-36. doi: 10.1007/978-1-4939-7428-3_2.

PMID:
29076082
4.

Mutation in human CLPX elevates levels of δ-aminolevulinate synthase and protoporphyrin IX to promote erythropoietic protoporphyria.

Yien YY, Ducamp S, van der Vorm LN, Kardon JR, Manceau H, Kannengiesser C, Bergonia HA, Kafina MD, Karim Z, Gouya L, Baker TA, Puy H, Phillips JD, Nicolas G, Paw BH.

Proc Natl Acad Sci U S A. 2017 Sep 19;114(38):E8045-E8052. doi: 10.1073/pnas.1700632114. Epub 2017 Sep 5.

5.

Reductions in the mitochondrial ABC transporter Abcb10 affect the transcriptional profile of heme biosynthesis genes.

Seguin A, Takahashi-Makise N, Yien YY, Huston NC, Whitman JC, Musso G, Wallace JA, Bradley T, Bergonia HA, Kafina MD, Matsumoto M, Igarashi K, Phillips JD, Paw BH, Kaplan J, Ward DM.

J Biol Chem. 2017 Sep 29;292(39):16284-16299. doi: 10.1074/jbc.M117.797415. Epub 2017 Aug 14.

6.

Intracellular iron and heme trafficking and metabolism in developing erythroblasts.

Kafina MD, Paw BH.

Metallomics. 2017 Sep 20;9(9):1193-1203. doi: 10.1039/c7mt00103g. Review.

7.

Erythropoietin signaling regulates heme biosynthesis.

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.

Elife. 2017 May 29;6. pii: e24767. doi: 10.7554/eLife.24767.

8.

Restored iron transport by a small molecule promotes absorption and hemoglobinization in animals.

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.

Science. 2017 May 12;356(6338):608-616. doi: 10.1126/science.aah3862.

9.

Characterization of genomic deletion efficiency mediated by clustered regularly interspaced short palindromic repeats (CRISPR)/Cas9 nuclease system in mammalian cells.

Canver MC, Bauer DE, Dass A, Yien YY, Chung J, Masuda T, Maeda T, Paw BH, Orkin SH.

J Biol Chem. 2017 Feb 10;292(6):2556. doi: 10.1074/jbc.A114.564625. No abstract available.

10.

Studying disorders of vertebrate iron and heme metabolism using zebrafish.

van der Vorm LN, Paw BH.

Methods Cell Biol. 2017;138:193-220. doi: 10.1016/bs.mcb.2016.10.008. Epub 2016 Dec 9. Review.

11.

A recurring mutation in the respiratory complex 1 protein NDUFB11 is responsible for a novel form of X-linked sideroblastic anemia.

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.

Blood. 2016 Oct 13;128(15):1913-1917. Epub 2016 Aug 3.

12.

A role for iron deficiency in dopaminergic neurodegeneration.

Yien YY, Paw BH.

Proc Natl Acad Sci U S A. 2016 Mar 29;113(13):3417-8. doi: 10.1073/pnas.1601976113. Epub 2016 Mar 16. No abstract available.

13.

Authors' response to "Comment on: 'Homozygous knockout of the piezo1 gene in the zebrafish is not associated with anemia'".

Shmukler BE, Lawson ND, Paw BH, Alper SL.

Haematologica. 2016 Jan;101(1):e39. doi: 10.3324/haematol.2015.137810. No abstract available.

14.

Hematopoietic stem cells develop in the absence of endothelial cadherin 5 expression.

Anderson H, Patch TC, Reddy PN, Hagedorn 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.

Blood. 2015 Dec 24;126(26):2811-20. doi: 10.1182/blood-2015-07-659276. Epub 2015 Sep 18.

15.

Mitochondrial transport of protoporphyrinogen IX in erythroid cells.

Yien YY, Ringel AR, Paw BH.

Oncotarget. 2015 Aug 28;6(25):20742-3.

16.

Homozygous knockout of the piezo1 gene in the zebrafish is not associated with anemia.

Shmukler BE, Huston NC, Thon JN, Ni CW, Kourkoulis G, Lawson ND, Paw BH, Alper SL.

Haematologica. 2015 Dec;100(12):e483-5. doi: 10.3324/haematol.2015.132449. Epub 2015 Aug 20. No abstract available.

17.

Mitochondrial ClpX Activates a Key Enzyme for Heme Biosynthesis and Erythropoiesis.

Kardon JR, Yien YY, Huston NC, Branco DS, Hildick-Smith GJ, Rhee KY, Paw BH, Baker TA.

Cell. 2015 May 7;161(4):858-67. doi: 10.1016/j.cell.2015.04.017.

18.

The mTORC1/4E-BP pathway coordinates hemoglobin production with L-leucine availability.

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.

Sci Signal. 2015 Apr 14;8(372):ra34. doi: 10.1126/scisignal.aaa5903.

19.

TMEM14C is required for erythroid mitochondrial heme metabolism.

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.

J Clin Invest. 2014 Oct;124(10):4294-304. doi: 10.1172/JCI76979. Epub 2014 Aug 26.

20.

Characterization of genomic deletion efficiency mediated by clustered regularly interspaced short palindromic repeats (CRISPR)/Cas9 nuclease system in mammalian cells.

Canver MC, Bauer DE, Dass A, Yien YY, Chung J, Masuda T, Maeda T, Paw BH, Orkin SH.

J Biol Chem. 2014 Aug 1;289(31):21312-24. doi: 10.1074/jbc.M114.564625. Epub 2014 Jun 6. Erratum in: J Biol Chem. 2017 Feb 10;292(6):2556.

21.

Molecular cloning and functional characterization of zebrafish Slc4a3/Ae3 anion exchanger.

Shmukler BE, Reimold FR, Heneghan JF, Chen C, Zhao T, Paw BH, Alper SL.

Pflugers Arch. 2014 Aug;466(8):1605-18. doi: 10.1007/s00424-014-1494-2. Epub 2014 Mar 26.

22.

Iron regulatory protein-1 protects against mitoferrin-1-deficient porphyria.

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.

J Biol Chem. 2014 Mar 14;289(11):7835-43. doi: 10.1074/jbc.M114.547778. Epub 2014 Feb 7. Erratum in: J Biol Chem. 2014 May 16;289(20):13707.

23.

Macrocytic anemia and mitochondriopathy resulting from a defect in sideroflexin 4.

Hildick-Smith GJ, Cooney JD, Garone C, Kremer LS, 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 Jr, Merkenschlager A, Beblo S, Strom TM, Meitinger T, Freisinger P, Donati MA, Prokisch H, Mootha VK, DiMauro S, Paw BH.

Am J Hum Genet. 2013 Nov 7;93(5):906-14. doi: 10.1016/j.ajhg.2013.09.011. Epub 2013 Oct 10.

24.

Distinct neuroblastoma-associated alterations of PHOX2B impair sympathetic neuronal differentiation in zebrafish models.

Pei D, Luther W, Wang W, Paw BH, Stewart RA, George RE.

PLoS Genet. 2013 Jun;9(6):e1003533. doi: 10.1371/journal.pgen.1003533. Epub 2013 Jun 6.

25.

ATP-binding cassette B10 regulates early steps of heme synthesis.

Bayeva M, Khechaduri A, Wu R, Burke MA, Wasserstrom JA, Singh N, Liesa M, Shirihai OS, Langer NB, Paw BH, Ardehali H.

Circ Res. 2013 Jul 19;113(3):279-87. doi: 10.1161/CIRCRESAHA.113.301552. Epub 2013 May 29.

26.

Snx3 regulates recycling of the transferrin receptor and iron assimilation.

Chen C, Garcia-Santos D, Ishikawa Y, Seguin A, Li L, Fegan KH, Hildick-Smith GJ, Shah DI, Cooney JD, Chen W, King MJ, Yien YY, Schultz IJ, Anderson H, Dalton AJ, Freedman ML, Kingsley PD, Palis J, Hattangadi SM, Lodish HF, Ward DM, Kaplan J, Maeda T, Ponka P, Paw BH.

Cell Metab. 2013 Mar 5;17(3):343-52. doi: 10.1016/j.cmet.2013.01.013. Epub 2013 Feb 14.

27.

Glucose metabolism impacts the spatiotemporal onset and magnitude of HSC induction in vivo.

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.

Blood. 2013 Mar 28;121(13):2483-93. doi: 10.1182/blood-2012-12-471201. Epub 2013 Jan 22.

28.

The scat mouse model highlights RASA3, a GTPase activating protein, as a key regulator of vertebrate erythropoiesis and megakaryopoiesis.

Peters LL, Paw BH, Blanc L.

Small GTPases. 2013 Jan-Mar;4(1):47-50. doi: 10.4161/sgtp.23013. Epub 2012 Dec 6.

29.

Mitochondrial Atpif1 regulates haem synthesis in developing erythroblasts.

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 JM, 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.

Nature. 2012 Nov 22;491(7425):608-12. doi: 10.1038/nature11536. Epub 2012 Nov 7. Erratum in: Nature. 2013 Apr 18;496(7445):386.

30.

Teleost growth factor independence (gfi) genes differentially regulate successive waves of hematopoiesis.

Cooney JD, Hildick-Smith GJ, Shafizadeh E, McBride PF, Carroll KJ, Anderson H, Shaw GC, Tamplin OJ, Branco DS, Dalton AJ, Shah DI, Wong C, Gallagher PG, Zon LI, North TE, Paw BH.

Dev Biol. 2013 Jan 15;373(2):431-41. doi: 10.1016/j.ydbio.2012.08.015. Epub 2012 Aug 29.

31.

Critical function for the Ras-GTPase activating protein RASA3 in vertebrate erythropoiesis and megakaryopoiesis.

Blanc L, Ciciotte SL, Gwynn B, Hildick-Smith GJ, Pierce EL, Soltis KA, Cooney JD, Paw BH, Peters LL.

Proc Natl Acad Sci U S A. 2012 Jul 24;109(30):12099-104. doi: 10.1073/pnas.1204948109. Epub 2012 Jul 6.

32.

L-Leucine improves the anemia and developmental defects associated with Diamond-Blackfan anemia and del(5q) MDS by activating the mTOR pathway.

Payne EM, Virgilio M, Narla A, Sun H, Levine M, Paw BH, Berliner N, Look AT, Ebert BL, Khanna-Gupta A.

Blood. 2012 Sep 13;120(11):2214-24. doi: 10.1182/blood-2011-10-382986. Epub 2012 Jun 25.

33.

Mutation mapping and identification by whole-genome sequencing.

Leshchiner I, Alexa K, Kelsey P, Adzhubei I, Austin-Tse CA, Cooney JD, Anderson H, King MJ, Stottmann RW, Garnaas MK, Ha S, Drummond IA, Paw BH, North TE, Beier DR, Goessling W, Sunyaev SR.

Genome Res. 2012 Aug;22(8):1541-8. doi: 10.1101/gr.135541.111. Epub 2012 May 3.

34.

Heme metabolism and erythropoiesis.

Chung J, Chen C, Paw BH.

Curr Opin Hematol. 2012 May;19(3):156-62. doi: 10.1097/MOH.0b013e328351c48b. Review.

35.

Cellular and mitochondrial iron homeostasis in vertebrates.

Chen C, Paw BH.

Biochim Biophys Acta. 2012 Sep;1823(9):1459-67. doi: 10.1016/j.bbamcr.2012.01.003. Epub 2012 Jan 18. Review.

36.

ABCB6 mutations cause ocular coloboma.

Wang L, He F, Bu J, Zhen Y, 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 W, Li DY, Phillips JD, Yang Z.

Am J Hum Genet. 2012 Jan 13;90(1):40-8. doi: 10.1016/j.ajhg.2011.11.026. Epub 2012 Jan 5. Erratum in: Am J Hum Genet. 2012 Aug 10;91(2):397. Zhen, Yuanli [added]; Li, Wei [added]; Wang, Lejing [corrected to Wang, Lejin]; Liu, Xiaqi [corrected to Liu, Xiaoqui].

37.

Abnormal mitoferrin-1 expression in patients with erythropoietic protoporphyria.

Wang Y, Langer NB, Shaw GC, Yang G, Li L, Kaplan J, Paw BH, Bloomer JR.

Exp Hematol. 2011 Jul;39(7):784-94. doi: 10.1016/j.exphem.2011.05.003. Epub 2011 May 11.

38.

Targeted deletion of the mouse Mitoferrin1 gene: from anemia to protoporphyria.

Troadec MB, Warner D, Wallace J, Thomas K, Spangrude GJ, Phillips J, Khalimonchuk O, Paw BH, Ward DM, Kaplan J.

Blood. 2011 May 19;117(20):5494-502. doi: 10.1182/blood-2010-11-319483. Epub 2011 Feb 10.

39.

Identification of distal cis-regulatory elements at mouse mitoferrin loci using zebrafish transgenesis.

Amigo JD, Yu M, Troadec MB, Gwynn B, Cooney JD, Lambert AJ, Chi NC, Weiss MJ, Peters LL, Kaplan J, Cantor AB, Paw BH.

Mol Cell Biol. 2011 Apr;31(7):1344-56. doi: 10.1128/MCB.01010-10. Epub 2011 Jan 19.

40.

Intoxication of zebrafish and mammalian cells by cholera toxin depends on the flotillin/reggie proteins but not Derlin-1 or -2.

Saslowsky DE, Cho JA, Chinnapen H, Massol RH, Chinnapen DJ, Wagner JS, De Luca HE, Kam W, Paw BH, Lencer WI.

J Clin Invest. 2010 Dec;120(12):4399-4409. doi: 10.1172/JCI42958.

41.

Antagonistic roles of the ERK and p38 MAPK signalling pathways in globin expression, haem biosynthesis and iron uptake.

Mardini L, Gasiorek J, Derjuga A, Carrière L, Schranzhofer M, Paw BH, Ponka P, Blank V.

Biochem J. 2010 Nov 15;432(1):145-51. doi: 10.1042/BJ20100541.

PMID:
20738258
42.

miR-451 protects against erythroid oxidant stress by repressing 14-3-3zeta.

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, Tong W, Orange JS, Paw BH, Weiss MJ.

Genes Dev. 2010 Aug 1;24(15):1620-33. doi: 10.1101/gad.1942110.

43.

Iron and porphyrin trafficking in heme biogenesis.

Schultz IJ, Chen C, Paw BH, Hamza I.

J Biol Chem. 2010 Aug 27;285(35):26753-9. doi: 10.1074/jbc.R110.119503. Epub 2010 Jun 3. Review.

44.

Ferrochelatase forms an oligomeric complex with mitoferrin-1 and Abcb10 for erythroid heme biosynthesis.

Chen W, Dailey HA, Paw BH.

Blood. 2010 Jul 29;116(4):628-30. doi: 10.1182/blood-2009-12-259614. Epub 2010 Apr 28.

45.

Abcb10 physically interacts with mitoferrin-1 (Slc25a37) to enhance its stability and function in the erythroid mitochondria.

Chen W, Paradkar PN, Li L, Pierce EL, Langer NB, Takahashi-Makise N, Hyde BB, Shirihai OS, Ward DM, Kaplan J, Paw BH.

Proc Natl Acad Sci U S A. 2009 Sep 22;106(38):16263-8. doi: 10.1073/pnas.0904519106. Epub 2009 Sep 4.

46.

The role and regulation of friend of GATA-1 (FOG-1) during blood development in the zebrafish.

Amigo JD, Ackermann GE, Cope JJ, Yu M, Cooney JD, Ma D, Langer NB, Shafizadeh E, Shaw GC, Horsely W, Trede NS, Davidson AJ, Barut BA, Zhou Y, Wojiski SA, Traver D, Moran TB, Kourkoulis G, Hsu K, Kanki JP, Shah DI, Lin HF, Handin RI, Cantor AB, Paw BH.

Blood. 2009 Nov 19;114(21):4654-63. doi: 10.1182/blood-2008-12-189910. Epub 2009 Sep 3.

47.

Discovery of genes essential for heme biosynthesis through large-scale gene expression analysis.

Nilsson R, Schultz IJ, Pierce EL, Soltis KA, Naranuntarat A, Ward DM, Baughman JM, Paradkar PN, Kingsley PD, Culotta VC, Kaplan J, Palis J, Paw BH, Mootha VK.

Cell Metab. 2009 Aug;10(2):119-30. doi: 10.1016/j.cmet.2009.06.012.

48.

Mutations affecting the secretory COPII coat component SEC23B cause congenital dyserythropoietic anemia type II.

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, Perrotta S, Denecke J, Pannicke U, Delaunay J, Pepperkok R, Heimpel H.

Nat Genet. 2009 Aug;41(8):936-40. doi: 10.1038/ng.405. Epub 2009 Jun 28.

PMID:
19561605
49.

In vivo inactivation of MASTL kinase results in thrombocytopenia.

Johnson HJ, Gandhi MJ, Shafizadeh E, Langer NB, Pierce EL, Paw BH, Gilligan DM, Drachman JG.

Exp Hematol. 2009 Aug;37(8):901-8. doi: 10.1016/j.exphem.2009.05.005. Epub 2009 May 19.

50.

Zebrafish kidney stromal cell lines support multilineage hematopoiesis.

Stachura DL, Reyes JR, Bartunek P, Paw BH, Zon LI, Traver D.

Blood. 2009 Jul 9;114(2):279-89. doi: 10.1182/blood-2009-02-203638. Epub 2009 May 11.

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