Browsing by Author "Paw, Barry Htin"

Now showing items 1-13 of 13

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

      Chen, Wen; Paradkar, P. N.; Li, L.; Pierce, E. L.; Langer, N. B.; Takahashi-Makise, N.; Hyde, B. B.; Shirihai, O. S.; Ward, D. M.; Kaplan, J.; Paw, Barry Htin (Proceedings of the National Academy of Sciences, 2009)
      Mitoferrin-1 (Mfrn1; Slc25a37), a member of the solute carrier family localized in the mitochondrial inner membrane, functions as an essential iron importer for the synthesis of mitochondrial heme and iron–sulfur clusters ...

    • Abnormal mitoferrin-1 expression in patients with erythropoietic protoporphyria 

      Wang, Yongming; Langer, Nathaniel B.; Shaw, George C.; Yang, Guang; Li, Liangtao; Kaplan, Jerry; Paw, Barry Htin; Bloomer, Joseph R. (Elsevier BV, 2011)

    • Cardiac Hypertrophy Involves both Myocyte Hypertrophy and Hyperplasia in Anemic Zebrafish 

      Sun, Xiaojing; Hoage, Tiffany; Ding, Yonghe; Chen, Zhenyue; Zhang, Ruilin; Jahangir, Ashad; Xu, Xiaolei; Paw, Barry Htin; Bai, Ping; Huang, Wei; Li, Yi-Gang (Public Library of Science, 2009)
      Background: An adult zebrafish heart possesses a high capacity of regeneration. However, it has been unclear whether and how myocyte hyperplasia contributes to cardiac remodeling in response to biomechanical stress and ...

    • Discovery of Genes Essential for Heme Biosynthesis through Large-Scale Gene Expression Analysis 

      Nilsson, Roland; Schultz, Iman J.; Pierce, Eric Adam; Soltis, Kathleen A.; Naranuntarat, Amornrat; Ward, Diane M.; Baughman, Joshua M.; Paradkar, Prasad N.; Kingsley, Paul D.; Culotta, Valeria C.; Kaplan, Jerry B.; Palis, James; Paw, Barry Htin; Mootha, Vamsi Krishna (Elsevier BV, 2009)
      Heme biosynthesis consists of a series of eight enzymatic reactions that originate in mitochondria and continue in the cytosol before returning to mitochondria. Although these core enzymes are well studied, additional ...

    • Distinct Neuroblastoma-associated Alterations of PHOX2B Impair Sympathetic Neuronal Differentiation in Zebrafish Models 

      Pei, Desheng; Luther, William; Wang, Wenchao; Paw, Barry H.; Stewart, Rodney A.; George, Rani E. (Public Library of Science, 2013)
      Heterozygous germline mutations and deletions in PHOX2B, a key regulator of autonomic neuron development, predispose to neuroblastoma, a tumor of the peripheral sympathetic nervous system. To gain insight into the oncogenic ...

    • A GATA-1-regulated microRNA locus essential for erythropoiesis 

      Dore, L. C.; Amigo, J. D.; Dos Santos, Carolina; Zhang, Z.; Gai, Xiaowu; Tobias, J. W.; Yu, D.; Klein, A. M.; Dorman, C.; Wu, W.; Hardison, R. C.; Paw, Barry Htin; Weiss, M. J. (Proceedings of the National Academy of Sciences, 2008)
      MicroRNAs (miRNAs) control tissue development, but their mechanism of regulation is not well understood. We used a gene complementation strategy combined with microarray screening to identify miRNAs involved in the formation ...

    • Identification of Distal cis-Regulatory Elements at Mouse Mitoferrin Loci Using Zebrafish Transgenesis 

      Amigo, J. D.; Yu, M.; Troadec, M.-B.; Gwynn, B.; Cooney, J. D.; Lambert, A. J.; Chi, N. C.; Weiss, M. J.; Peters, L. L.; Kaplan, J.; Cantor, Alan B.; Paw, Barry Htin (American Society for Microbiology, 2011)
      Mitoferrin 1 (Mfrn1; Slc25a37) and mitoferrin 2 (Mfrn2; Slc25a28) function as essential mitochondrial iron importers for heme and Fe/S cluster biogenesis. A genetic deficiency of Mfrn1 results in a profound hypochromic ...

    • Mitochondrial Atpif1 regulates heme synthesis in developing erythroblasts 

      Shah, Dhvanit I; Takahashi-Makise, Naoko; Cooney, Jeffrey D.; Li, Liangtao; Schultz, Iman J.; Pierce, Eric L.; Narla, Anupama; Seguin, Alexandra; Hattangadi, Shilpa M.; Medlock, Amy E.; Langer, Nathaniel B.; Dailey, Tamara A.; Hurst, Slater N.; Faccenda, Danilo; Wiwczar, Jessica M.; Heggers, Spencer K.; Vogin, Guillaume; Chen, Wen; Chen, Caiyong; Campagna, Dean R.; Brugnara, Carlo; Zhou, Yi; Ebert, Benjamin L.; Danial, Nika; Fleming, Mark Daniel; Ward, Diane M.; Campanella, Michelangelo; Dailey, Harry A.; Kaplan, Jerry; Paw, Barry Htin (2012)
      SUMMARY Defects in the availability of heme substrates or the catalytic activity of the terminal enzyme in heme biosynthesis, ferrochelatase (Fech), impair heme synthesis, and thus cause human congenital anemias1,2. The ...

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

      Yien, Yvette Yee; Huston, Nicholas C.; Branco, Diana S.; Hildick-Smith, Gordon J.; Rhee, Kevin; Paw, Barry Htin; Baker, Tania A. (Elsevier BV, 2015)
      The mitochondrion maintains and regulates its proteome with chaperones primarily inherited from its bacterial endosymbiont ancestor. Among these chaperones is the AAA+ unfoldase ClpX, an important regulator of prokaryotic ...

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

      Chung, Jacky; Bauer, Daniel Evan; Ghamari, Alireza; Nizzi, C. P.; Deck, K. M.; Kingsley, P. D.; Yien, Yvette Yee; Huston, N. C.; Chen, C.; Schultz, I. J.; Dalton, Arthur J.; Wittig, Johannes; Palis, J.; Orkin, Stuart Holland; Lodish, H. F.; Eisenstein, R. S.; Cantor, Alan B.; Paw, Barry Htin (American Association for the Advancement of Science (AAAS), 2015)
      In multicellular organisms, the mechanisms by which diverse cell types acquire distinct amino acids and how cellular function adapts to their availability are fundamental questions in biology. We found that increased neutral ...

    • Snx3 Regulates Recycling of the Transferrin Receptor and Iron Assimilation 

      Chen, Caiyong; Garcia-Santos, Daniel; Ishikawa, Yuichi; Seguin, Alexandra; Li, Liangtao; Fegan, Katherine H.; Hildick-Smith, Gordon J.; Shah, Darshan Anil; Cooney, James A.; Chen, Wen; King, Michael; Yien, Yvette Yee; Schultz, Iman J.; Anderson, Heidi; Dalton, Abigail Goodnow; Freedman, Matthew Lawrence; Kingsley, Paul D.; Palis, James; Hattangadi, Shilpa M.; Lodish, Harvey F.; Ward, Daniel Alexander; Kaplan, Jerry B.; Maeda, Takahiro; Ponka, Prem; Paw, Barry Htin (Elsevier BV, 2013)
      Sorting of endocytic ligands and receptors is critical for diverse cellular processes. The physiological significance of endosomal sorting proteins in vertebrates, however, remains largely unknown. Here we report that ...

    • Teleost Growth Factor Independence (Gfi) Genes Differentially Regulate Successive Waves of Hematopoiesis 

      Cooney, Jeffrey D.; Hildick-Smith, Gordon J.; Shafizadeh, Ebrahim; McBride, Paul F.; Carroll, Kelli Jane; Anderson, Heidi; Shaw, George C.; Tamplin, Owen J.; Branco, Diana S.; Dalton, Arthur J.; Shah, Dhvanit I; Wong, Clara; Gallagher, Patrick G.; Zon, Leonard Ira; North, Trista Elizabeth; Paw, Barry Htin (Elsevier BV, 2013)
      Growth Factor Independence (Gfi) transcription factors play essential roles in hematopoiesis, differentially activating and repressing transcriptional programs required for hematopoietic stem/progenitor cell (HSPC) development ...

    • TMEM14C is required for erythroid mitochondrial heme metabolism 

      Yien, Yvette Yee; Robledo, Raymond F.; Schultz, Iman J.; Takahashi-Makise, Naoko; Gwynn, Babette; Bauer, Daniel Evan; Dass, Abhishek; Yi, Gloria; Li, Liangtao; Hildick-Smith, Gordon J.; Cooney, Jeffrey D.; Pierce, Eric Adam; Mohler, Kyla; Dailey, Tamara A.; Miyata, Non; Kingsley, Paul D.; Garone, Caterina; Hattangadi, Shilpa M.; Huang, Hui; Chen, Wen; Keenan, Ellen M.; Shah, Dhvanit I; Schlaeger, Thorsten M.; DiMauro, Salvatore; Orkin, Stuart Holland; Cantor, Alan B.; Palis, James; Koehler, Carla M.; Lodish, Harvey F.; Kaplan, Jerry B.; Ward, Diane M.; Dailey, Harry A.; Phillips, John Gary; Peters, Luanne L.; Paw, Barry Htin (American Society for Clinical Investigation, 2014)
      The transport and intracellular trafficking of heme biosynthesis intermediates are crucial for hemoglobin production, which is a critical process in developing red cells. Here, we profiled gene expression in terminally ...