Recent Publications
July 2020
Hepatocellular Carcinoma in Acute Hepatic Porphyrias: Results from the Longitudinal Study of the U.S. Porphyrias Consortium. Saberi B, Naik H, Overbey JR, Erwin AL, Anderson KE, Bissell DM, Bonkovsky HL, Phillips JD, Wang B, K Singal A, M McGuire B, Desnick RJ, Balwani M. Hepatology. 2020 Jul 18. doi: 10.1002/hep.31460. Online ahead of print. PMID: 32681675
Exchange transfusion for hemolytic hyperbilirubinemia: could some be averted by emergent administration of an inhibitor of bilirubin production? Wiedmeier SE, Bahr TM, Ohls RK, Christensen TR, Baer VL, Ilstrup SJ, Cail K, Christensen RD. J Perinatol. 2020 Jul 15. doi: 10.1038/s41372-020-0736-8. Online ahead of print. PMID: 32669646
Peripheral blood mononuclear cells show prominent gene expression by erythroid progenitors in diseases characterized by heightened erythropoiesis. Zhang X, Song J, Shah BN, Nekhai S, Miasnikova G, Sergueeva A, Prchal JT, Gordeuk VR. Br J Haematol. 2020 Jul;190(1):e42-e45. doi: 10.1111/bjh.16696. Epub 2020 May 12. PMID: 32399971
Cutting in-line with iron: ribosomal function and non-oxidative RNA cleavage. Guth-Metzler R, Bray MS, Frenkel-Pinter M, Suttapitugsakul S, Montllor-Albalate C, Bowman JC, Wu R, Reddi AR, Okafor CD, Glass JB, Williams LD. Nucleic Acids Res. 2020 Jul 14:gkaa586. doi: 10.1093/nar/gkaa586. Online ahead of print. PMID: 32663277
June 2020
Dizygotic twins with prolonged jaundice and microcytic, hypochromic, hemolytic anemia with pyropoikilocytosis. Bahr TM, Lozano-Chinga M, Agarwal AM, Meznarich JA, Gerday E, Smoot JL, Taylor A, Christensen RD. Blood Cells Mol Dis. 2020 Jun 25;85:102462. doi: 10.1016/j.bcmd.2020.102462. Online ahead of print. PMID: 32623341
Pediatric Hematology Normal Ranges Derived From Pediatric Primary Care Patients. Staffa SJ, Joerger JD, Henry E, Christensen RD, Brugnara C, Zurakowski D. Am J Hematol. 2020 Jun 12. doi: 10.1002/ajh.25904. Online ahead of print. PMID: 32530536
QSRR Automator: A Tool for Automating Retention Time Prediction in Lipidomics and Metabolomics. Naylor BC, Catrow JL, Maschek JA, Cox JE. Metabolites. 2020 Jun 9;10(6):237. doi: 10.3390/metabo10060237. PMID: 32526851
Human aminolevulinate synthase structure reveals a eukaryotic-specific autoinhibitory loop regulating substrate binding and product release. Bailey HJ, Bezerra GA, Marcero JR, Padhi S, Foster WR, Rembeza E, Roy A, Bishop DF, Desnick RJ, Bulusu G, Dailey HA Jr, Yue WW. Nat Commun. 2020 Jun 4;11(1):2813. doi: 10.1038/s41467-020-16586-x.
Macrophage HIF-1α is an independent prognostic indicator in kidney cancer. Cowman S, Fuja DG, Liu XD, Tidwell RSS, Kandula N, Sirohi D, Agarwal AM, Emerson LL, Tripp SR, Mohlman JS, Stonhill M, Garcia G, Conley CJ, Olalde AA, Sargis T, Ramirez-Torres A, Karam JA, Wood CG, Sircar K, Tamboli P, Boucher KM, Maughan BL, Spike BT, Ho TH, Agarwal N, Jonasch E, Koh MY. Clin Cancer Res. 2020 Jun 25:clincanres.3890.2019. doi: 10.1158/1078-0432.CCR-19-3890. Online ahead of print. PMID: 32586940
Iron overload causes a mild and transient increase in acute lung injury. Zhang V, Ganz T, Nemeth E, Kim A. Physiol Rep. 2020 Jun;8(12):e14470. doi: 10.14814/phy2.14470. PMID: 32596989
Maternal Hepcidin Determines Embryo Iron Homeostasis. Sangkhae V, Fisher AL, Chua KJ, Ruchala P, Ganz T, Nemeth E. Blood. 2020 Jun 25:blood.2020005745. doi: 10.1182/blood.2020005745. Online ahead of print. PMID: 32584957
The mitochondrial metal transporters mitoferrin1 and mitoferrin2 are required for liver regeneration and cell proliferation in mice. Seguin A, Jia X, Earl AM, Li L, Wallace J, Qiu A, Bradley T, Shrestha R, Troadec MB, Hockin M, Titen S, Warner DE, Dowdle PT, Wohlfahrt ME, Hillas E, Firpo MA, Phillips JD, Kaplan J, Paw BH, Barasch J, Ward DM. J Biol Chem. 2020 Jun 9:jbc.RA120.013229. doi: 10.1074/jbc.RA120.013229. Online ahead of print. PMID: 32518166
May 2020
Iron chaperone PCBP1 protects murine liver from lipid peroxidation and steatosis. Protchenko O, Baratz E, Jadhav S, Li F, Shakoury-Elizeh M, Gavrilova O, Ghosh MC, Cox JE, Maschek JA, Tyurin VA, Tyurina YY, Bayir H, Aron AT, Chang CJ, Kagan VE, Philpott CC. Hepatology. 2020 May 21. doi: 10.1002/hep.31328. Online ahead of print. PMID: 32438524
Toxoplasma gondii requires its plant-like heme biosynthesis pathway for infection. Bergmann A, Floyd K, Key M, Dameron C, Rees KC, Thornton LB, Whitehead DC, Hamza I, Dou Z.PLoS Pathog. 2020 May 14;16(5):e1008499. doi: 10.1371/journal.ppat.1008499. eCollection 2020 May. PMID: 32407406Free PMC article.
Increased iron stores influence glucose metabolism in sickle cell anaemia. Shah BN, Hassan TO, Zhang X, McClain DA, Gordeuk VR. Br J Haematol. 2020 May;189(4):e184-e187. doi: 10.1111/bjh.16596. Epub 2020 Mar 30. PMID: 32232834
2020 Apr
Dendritic cell-derived hepcidin sequesters iron from the microbiota to promote mucosal healing. Bessman NJ, Mathieu JRR, Renassia C, Zhou L, Fung TC, Fernandez KC, Austin C, Moeller JB, Zumerle S, Louis S, Vaulont S, Ajami NJ, Sokol H, Putzel GG, Arvedson T, Sockolow RE, Lakhal-Littleton S, Cloonan SM, Arora M, Peyssonnaux C, Sonnenberg GF. Science. 2020 Apr 10;368(6487):186-189. doi: 10.1126/science.aau6481.PMID:32273468
Mitochondrial-nuclear heme trafficking is regulated by GTPases in control of mitochondrial dynamics and ER contact sites. Martinez-Guzman O, Willoughby MM, Saini A, Dietz JV, Bohovych I, Medlock AE, Khalimonchuk O, Reddi AR. J Cell Sci. 2020 Apr 7. pii: jcs.237917. doi: 10.1242/jcs.237917. [Epub ahead of print] PMID:32265272
2020 Mar
Exploiting species specificity to understand the tropism of a human-specific toxin. Boguslawski KM, McKeown AN, Day CJ, Lacey KA, Tam K, Vozhilla N, Kim SY, Jennings MP, Koralov SB, Elde NC, Torres VJ. Sci Adv. 2020 Mar 11;6(11):eaax7515. doi: 10.1126/sciadv.aax7515. eCollection 2020 Mar. PMID:32195339
2020 Feb
Genome-wide CRISPR-Cas9 screening reveals ubiquitous T cell cancer targeting via the monomorphic MHC class I-related protein MR1. Crowther MD, Dolton G, Legut M, Caillaud ME, Lloyd A, Attaf M, Galloway SAE, Rius C, Farrell CP, Szomolay B, Ager A, Parker AL, Fuller A, Donia M, McCluskey J, Rossjohn J, Svane IM, Phillips JD, Sewell AK. Nat Immunol. 2020 Feb;21(2):178-185. doi: 10.1038/s41590-019-0578-8. Epub 2020 Jan 20. PMID:31959982
Dexmedetomidine pretreatment improves lipopolysaccharide-induced iron homeostasis disorder in aged mice. Guo F, Kang J, Tan J, Wang Y, Jia L, Xu H. Curr Neurovasc Res. 2020 Feb 16. doi: 10.2174/1567202617666200217105109. [Epub ahead of print]
PMID:32065090
2020 Jan
Alternative splicing of UCP1 by non-cell-autonomous action of PEMT. Johnson JM, Verkerke ARP, Maschek JA, Ferrara PJ, Lin CT, Kew KA, Neufer PD, Lodhi IJ, Cox JE, Funai K. Mol Metab. 2020 Jan;31:55-66. doi: 10.1016/j.molmet.2019.10.007. Epub 2019 Nov 8.PMID:31918922
Iron and Heme Metabolism at the Leishmania-Host Interface. Laranjeira-Silva MF, Hamza I, Pérez-Victoria JM. Trends Parasitol. 2020 Mar;36(3):279-289. doi: 10.1016/j.pt.2019.12.010. Epub 2020 Jan 28. PMID: 32005611
Cysteine Toxicity Drives Age-Related Mitochondrial Decline by Altering Iron Homeostasis. Hughes C.E. Coody T.K. , Jeong M.-Y. ,Berg J.A. , Winge D.R., Hughes A.L. Cell. 2020; 180: 296-310
Genetic deletion of gpr27 alters acylcarnitine metabolism, insulin sensitivity, and glucose homeostasis in zebrafish. Nath AK, Ma J, Chen ZZ, Li Z, Vitery MDC, Kelley ML, Peterson RT, Gerszten RE, Yeh JJ. FASEB J. 2020 Jan;34(1):1546-1557. doi: 10.1096/fj.201901466R. Epub 2019 Dec 2. PMID:31914600
XPRESSyourself: Enhancing, standardizing, and automating ribosome profiling computational analyses yields improved insight into data. Berg JA, Belyeu JR, Morgan JT, Ouyang Y, Bott AJ, Quinlan AR, Gertz J, Rutter J. PLoS Comput Biol. 2020 Jan 31;16(1):e1007625. doi: 10.1371/journal.pcbi.1007625. [Epub ahead of print] PMID:32004313
Machine learning reveals serum sphingolipids as cholesterol-independent biomarkers of coronary artery disease. Poss AM, Maschek JA, Cox JE, Hauner BJ, Hopkins PN, Hunt SC, Holland WL, Summers SA, Playdon MC. J Clin Invest. 2020 Jan 27. pii: 131838. doi: 10.1172/JCI131838. [Epub ahead of print] PMID:31743112
PCBP2 post-transcriptionally regulates sortilin expression by binding to a C-rich element in its 3′ UTR. Yabe-Wada T, Philpott CC, Onai N. FEBS Open Bio. 2020 Jan 21. doi: 10.1002/2211-5463.12794. [Epub ahead of print] PMID:3196107
Irp2 regulates insulin production through iron-mediated Cdkal1-catalyzed tRNA modification. Santos MCFD, Anderson CP, Neschen S, Zumbrennen-Bullough KB, Romney SJ, Kahle-Stephan M, Rathkolb B, Gailus-Durner V, Fuchs H, Wolf E, Rozman J, de Angelis MH, Cai WM, Rajan M, Hu J, Dedon PC, Leibold EA. Nat Commun. 2020 Jan 15;11(1):296. doi: 10.1038/s41467-019-14004-5. PMID:31941883
Editorial: Special Issue on mitochondrial fidelity. Khalimonchuk O. Mitochondrion. 2020 Jan 7;51:104. doi: 10.1016/j.mito.2019.12.007. [Epub ahead of print] No abstract available. PMID:31917323
Maternal Tobacco Smoke Exposure Causes Sex-Divergent Changes in Placental Lipid Metabolism in the Rat. Weinheimer C, Wang H, Comstock JM, Singh P, Wang Z, Locklear BA, Goodwin KL, Maschek JA, Cox JE, Baack ML, Joss-Moore LA. Reprod Sci. 2020 Jan 6. doi: 10.1007/s43032-019-00065-w. [Epub ahead of print] PMID:32046449
ASA Suppresses PGE2 in Plasma and Melanocytic Nevi of Human Subjects at Increased Risk for Melanoma. Varedi A, Rahman H, Kumar D, Catrow JL, Cox JE, Liu T, Florell SR, Boucher KM, Okwundu N, Burnett WJ, VanBrocklin MW, Grossman D. Pharmaceuticals (Basel). 2020 Jan 2;13(1). pii: E7. doi: 10.3390/ph13010007. PMID:31906519
The mitochondrial iron exporter genes MMT1 and MMT2 in yeast are transcriptionally regulated by Aft1 and Yap1. Li L, Bertram S, Kaplan J, Jia X, Ward DM.J Biol Chem. 2020 Jan 2. pii: jbc.RA119.011154. doi: 10.1074/jbc.RA119.011154. [Epub ahead of print] PMID:31896574
Compartment and hub definitions tune metabolic networks for metabolomic interpretations. Waller TC, Berg JA, Lex A, Chapman BE, Rutter J. Gigascience. 2020 Jan 1;9(1). pii: giz137. doi: 10.1093/gigascience/giz137. PMID:31972021
2019 Dec
Parallel Reaction Monitoring reveals structure-specific ceramide alterations in the zebrafish. Zhang T, Trauger SA, Vidoudez C, Doane KP, Pluimer BR, Peterson RT. Sci Rep. 2019 Dec 27;9(1):19939. doi: 10.1038/s41598-019-56466-z.
A prospective evaluation of pegylated interferon alfa-2a therapy in patients with polycythemia vera and essential thrombocythemia with a prior splanchnic vein thrombosis. Mascarenhas J, Kosiorek H, Prchal J, Yacoub A, Berenzon D, Baer MR, Ritchie E, Silver RT, Kessler C, Winton E, Finazzi MC, Rambaldi A, Vannucchi AM, Leibowitz D, Rondelli D, Arcasoy MO, Catchatourian R, Vadakara J, Rosti V, Hexner E, Kremyanskaya M, Sandy L, Tripodi J, Najfeld V, Farnoud N, Salama ME, Weinberg RS, Rampal R, Goldberg JD, Mesa R, Dueck AC, Hoffman R. Leukemia. 2019 Dec;33(12):2974-2978. doi: 10.1038/s41375-019-0524-7. Epub 2019 Jul 30. No abstract available. PMID:31363161
JAK2 ex13InDel drives oncogenic transformation and is associated with chronic eosinophilic leukemia and polycythemia vera. Patel AB, Franzini A, Leroy E, Kim SJ, Pomicter AD, Genet L, Xiao M, Yan D, Ahmann JM, Agarwal AM, Clair P, Addada J, Lambert J, Salmon M, Gleich GJ, Cross NCP, Constantinescu SN, O’Hare T, Prchal JT, Deininger MW. Blood. 2019 Dec 26;134(26):2388-2398. doi: 10.1182/blood.2019001385. PMID:31697804
Universal Principled Review: A Community-Driven Method to Improve Peer Review. Krummel M, Blish C, Kuhns M, Cadwell K, Oberst A, Goldrath A, Ansel KM, Chi H, O’Connell R, Wherry EJ, Pepper M; Future Immunology Consortium. Cell. 2019 Dec 12;179(7):1441-1445. doi: 10.1016/j.cell.2019.11.029. PMID:31835023
Regulation of Tumor Initiation by the Mitochondrial Pyruvate Carrier. Bensard CL, Wisidagama DR, Olson KA, Berg JA, Krah NM, Schell JC, Nowinski SM, Fogarty S, Bott AJ, Wei P, Dove KK, Tanner JM, Panic V, Cluntun A, Lettlova S, Earl CS, Namnath DF, Vázquez-Arreguín K, Villanueva CJ, Tantin D, Murtaugh LC, Evason KJ, Ducker GS, Thummel CS, Rutter J. Cell Metab. 2019 Dec 5. pii: S1550-4131(19)30609-6. doi: 10.1016/j.cmet.2019.11.002. [Epub ahead of print] PMID:31813825
Erythroferrone lowers hepcidin by sequestering BMP2/6 heterodimer from binding to the BMP type I receptor ALK3. Wang CY, Xu Y, Traeger L, Dogan DY, Xiao X, Steinbicker AU, Babitt JL. Blood. 2019 Dec 4. pii: blood.2019002620. doi: 10.1182/blood.2019002620. [Epub ahead of print] No abstract available. PMID:31800957
Absence of severe neonatal ABO hemolytic disease at Intermountain Healthcare. Why? Baer VL, Hulse W, Bahr TM, Ilstrup SJ, Christensen RD. J Perinatol. 2019 Dec 3. doi: 10.1038/s41372-019-0553-0. [Epub ahead of print] No abstract available. PMID:31796840
Overexpression of the peroxin Pex34p suppresses impaired acetate utilization in yeast lacking the mitochondrial aspartate/glutamate carrier Agc1p. Chalermwat C, Thosapornvichai T, Wongkittichote P, Phillips JD, Cox JE, Jensen AN, Wattanasirichaigoon D, Jensen LT. FEMS Yeast Res. 2019 Dec 1;19(8). pii: foz078. doi: 10.1093/femsyr/foz078.PMID:31711143
2019 Nov
Heme biosynthesis and the porphyrias. Phillips JD. Mol Genet Metab. 2019 Nov;128(3):164-177. doi: 10.1016/j.ymgme.2019.04.008. Epub 2019 Apr 22. Review.PMID:31326287
Endothelial Bmp2 knockout exacerbates hemochromatosis in Hfe knockout mice but not Bmp6 knockout mice. Xiao X, Dev S, Canali S, Bayer A, Xu Y, Agarwal A, Wang CY, Babitt JL. Hepatology. 2019 Nov 28. doi: 10.1002/hep.31048. [Epub ahead of print] PMID:31778583
Iron chelation and 2-oxoglutarate-dependent dioxygenase inhibition suppress mantle cell lymphoma’s cyclin D1. Babosova O, Kapralova K, Raskova Kafkova L, Korinek V, Divoky V, Prchal JT, Lanikova L. J Cell Mol Med. 2019 Nov;23(11):7785-7795. doi: 10.1111/jcmm.14655. Epub 2019 Sep 13. PMID:31517438
Neonates with suspected microangiopathic disorders: performance of standard manual schistocyte enumeration vs. the automated fragmented red cell count. Bahr TM, Judkins AJ, Christensen RD, Baer VL, Henry E, Minton SD, Gerday E, Bennett ST. J Perinatol. 2019 Nov;39(11):1555-1561. doi: 10.1038/s41372-019-0482-y. Epub 2019 Aug 28. PMID:31462723
Results of a pilot study of isoniazid in patients with erythropoietic protoporphyria.Parker CJ, Desnick RJ, Bissel MD, Bloomer JR, Singal A, Gouya L, Puy H, Anderson KE, Balwani M, Phillips JD. Mol Genet Metab. 2019 Nov;128(3):309-313. doi: 10.1016/j.ymgme.2019.07.017. Epub 2019 Jul 31.PMID:31395332
Mitoferrin-1 is required for brain energy metabolism and hippocampus-dependent memory. Baldauf L, Endres T, Scholz J, Kirches E, Ward DM, Lessmann V, Borucki K, Mawrin C.Neurosci Lett. 2019 Nov 20;713:134521. doi: 10.1016/j.neulet.2019.134521. Epub 2019 Sep 26. PMID:31563673
Absence of HIF1A Leads to Glycogen Accumulation and an Inflammatory Response That Enables Pancreatic Tumor Growth. Maruggi M, Layng FI, Lemos R Jr, Garcia G, James BP, Sevilla M, Soldevilla F, Baaten BJ, de Jong PR, Koh MY, Powis G. Cancer Res. 2019 Nov 15;79(22):5839-5848. doi: 10.1158/0008-5472.CAN-18-2994. Epub 2019 Oct 4. PMID:31585939
Structural insights into heme binding to IL-36α proinflammatory cytokine. Wißbrock A, Goradia NB, Kumar A, Paul George AA, Kühl T, Bellstedt P, Ramachandran R, Hoffmann P, Galler K, Popp J, Neugebauer U, Hampel K, Zimmermann B, Adam S, Wiendl M, Krönke G, Hamza I, Heinemann SH, Frey S, Hueber AJ, Ohlenschläger O, Imhof D. Sci Rep. 2019 Nov 15;9(1):16893. doi: 10.1038/s41598-019-53231-0. PMID:31729440
The Human-Specific BOLA2 Duplication Modifies Iron Homeostasis and Anemia Predisposition in Chromosome 16p11.2 Autism Individuals. Giannuzzi G, Schmidt PJ, Porcu E, Willemin G, Munson KM, Nuttle X, Earl R, Chrast J, Hoekzema K, Risso D, Männik K, De Nittis P, Baratz ED; 16p11.2 Consortium, Herault Y, Gao X, Philpott CC, Bernier RA, Kutalik Z, Fleming MD, Eichler EE, Reymond A.Am J Hum Genet. 2019 Nov 7;105(5):947-958. doi: 10.1016/j.ajhg.2019.09.023. Epub 2019 Oct 24. PMID:31668704
Evaluating the Patient-Reported Outcomes Measurement Information System scales in acute intermittent porphyria. Naik H, Overbey JR, Montgomery GH, Winkel G, Balwani M, Anderson KE, Bissell DM, Bonkovsky HL, Phillips JD, Wang B, McGuire B, Keel S, Levy C, Erwin A, Desnick RJ. Genet Med. 2019 Nov 6. doi: 10.1038/s41436-019-0683-y. [Epub ahead of print]PMID:31690837
The fragmented red cell count can support the diagnosis of a microangiopathic neonatal condition. Bahr TM, Judkins AJ, Baer VL, Henry E, Grubb PH, Hulse W, Pysher TJ, Bennett ST, Christensen RD. J Perinatol. 2019 Nov 6. doi: 10.1038/s41372-019-0552-1. [Epub ahead of print] No abstract available. PMID:31695135
Response to interferon treatment in essential thrombocythemia with inv(3)(q21q26). Gangaraju R, Carroll AJ, Prchal JT. Ann Hematol. 2019 Nov 1. doi: 10.1007/s00277-019-03805-9. [Epub ahead of print] No abstract available. PMID:31676985
2019 Oct
Evaluating emergency-release blood transfusion of newborn infants at the Intermountain Healthcare hospitals. Bahr TM, DuPont TL, Christensen TR, Rees T, O’Brien EA, Ilstrup SJ, Christensen RD. Transfusion. 2019 Oct;59(10):3113-3119. doi: 10.1111/trf.15495. Epub 2019 Sep 3. PMID:31479169
First report of using low-titer cold-stored type O whole blood in massive postpartum hemorrhage. Bahr TM, DuPont TL, Morris DS, Pierson SE, Esplin MS, Brown SM, O’Brien EA, Ilstrup SJ, Christensen RD. Transfusion. 2019 Oct;59(10):3089-3092. doi: 10.1111/trf.15492. Epub 2019 Aug 30. PMID:31469450
Pegylated interferon alfa-2a for polycythemia vera or essential thrombocythemia resistant or intolerant to hydroxyurea. Yacoub A, Mascarenhas J, Kosiorek H, Prchal JT, Berenzon D, Baer MR, Ritchie E, Silver RT, Kessler C, Winton E, Finazzi MC, Rambaldi A, Vannucchi AM, Leibowitz D, Rondelli D, Arcasoy MO, Catchatourian R, Vadakara J, Rosti V, Hexner E, Kremyanskaya M, Sandy L, Tripodi J, Najfeld V, Farnoud N, Papaemmanuil E, Salama M, Singer-Weinberg R, Rampal R, Goldberg JD, Barbui T, Mesa R, Dueck AC, Hoffman R. Blood. 2019 Oct 31;134(18):1498-1509. doi: 10.1182/blood.2019000428. PMID:31515250
Experimental Modeling of Myeloproliferative Neoplasms. Lanikova L, Babosova O, Prchal JT. Genes (Basel). 2019 Oct 15;10(10). pii: E813. doi: 10.3390/genes10100813. Review. PMID:31618985
2019 Sep
Proteolytic Activation of Bmps: Analysis of Cleavage in Xenopus Oocytes and Embryos. Kim HS, McKnite A, Christian JL. Methods Mol Biol. 2019;1891:115-133. doi: 10.1007/978-1-4939-8904-1_9. PMID: 30414129
BMP7 functions predominantly as a heterodimer with BMP2 or BMP4 during mammalian embryogenesis. Kim HS, Neugebauer J, McKnite A, Tilak A, Christian JL. Elife. 2019 Sep 30;8:e48872. doi: 10.7554/eLife.48872. PMID: 31566563
A PCBP1-BolA2 chaperone complex delivers iron for cytosolic [2Fe-2S] cluster assembly. Patel SJ, Frey AG, Palenchar DJ, Achar S, Bullough KZ, Vashisht A, Wohlschlegel JA, Philpott CC. Nat Chem Biol. 2019 Sep;15(9):872-881. doi: 10.1038/s41589-019-0330-6. Epub 2019 Aug 12.PMID:31406370
NHR-14 loss of function couples intestinal iron uptake with innate immunity in C. elegans through PQM-1 signaling. Rajan M, Anderson CP, Rindler PM, Romney SJ, Ferreira Dos Santos MC, Gertz J, Leibold EA. Elife. 2019 Sep 18;8. pii: e44674. doi: 10.7554/eLife.44674. PMID:31532389
EXPLORE: A Prospective, Multinational, Natural History Study of Patients with Acute Hepatic Porphyria with Recurrent Attacks. Gouya L, Ventura P, Balwani M, Bissell DM, Rees DC, Stölzel U, Phillips JD, Kauppinen R, Langendonk JG, Desnick RJ, Deybach JC, Bonkovsky HL, Parker C, Naik H, Badminton M, Stein PE, Minder E, Windyga J, Bruha R, Cappellini MD, Sardh E, Harper P, Sandberg S, Aarsand AK, Andersen J, Alegre F, Ivanova A, Talbi N, Chan A, Querbes W, Ko J, Penz C, Liu S, Lin T, Simon A, Anderson KE. Hepatology. 2019 Sep 12. doi: 10.1002/hep.30936. [Epub ahead of print]PMID:31512765
Mitochondrial PE potentiates respiratory enzymes to amplify skeletal muscle aerobic capacity. Heden TD, Johnson JM, Ferrara PJ, Eshima H, Verkerke ARP, Wentzler EJ, Siripoksup P, Narowski TM, Coleman CB, Lin CT, Ryan TE, Reidy PT, de Castro Brás LE, Karner CM, Burant CF, Maschek JA, Cox JE, Mashek DG, Kardon G, Boudina S, Zeczycki TN, Rutter J, Shaikh SR, Vance JE, Drummond MJ, Neufer PD, Funai K. Sci Adv. 2019 Sep 11;5(9):eaax8352. doi: 10.1126/sciadv.aax8352. eCollection 2019 Sep. PMID:31535029
A Low Iron Diet Protects from Steatohepatitis in a Mouse Model. Salaye L, Bychkova I, Sink S, Kovalic AJ, Bharadwaj MS, Lorenzo F, Jain S, Harrison AV, Davis AT, Turnbull K, Meegalla NT, Lee SH, Cooksey R, Donati GL, Kavanagh K, Bonkovsky HL, McClain DA. Nutrients. 2019 Sep 10;11(9). pii: E2172. doi: 10.3390/nu11092172. PMID:31510077
2019 Aug
Transcription factor Oct1 protects against hematopoietic stress and promotes acute myeloid leukemia. Jafek JL, Shakya A, Tai PY, Ibarra A, Kim H, Maddox J, Chumley J, Spangrude GJ, Miles RR, Kelley TW, Tantin D. Exp Hematol. 2019 Aug;76:38-48.e2. doi: 10.1016/j.exphem.2019.07.002. Epub 2019 Jul 8. PMID:31295506
Knowing When to Let Go: Lysosomes Regulate Inter-Mitochondrial Tethering. English AM, Hughes AL. Dev Cell. 2019 Aug 5;50(3):259-260. doi: 10.1016/j.devcel.2019.07.019. PMID:31386859
Rsp5 and Mdm30 reshape the mitochondrial network in response to age-induced vacuole stress. Goodrum JM, Lever AR, Coody TK, Gottschling DE, Hughes AL. Mol Biol Cell. 2019 Aug 1;30(17):2141-2154. doi: 10.1091/mbc.E19-02-0094. Epub 2019 May 29. PMID:31141470
2019 July
Targeting a ceramide double bond improves insulin resistance and hepatic steatosis. Chaurasia B, Tippetts TS, Mayoral Monibas R, Liu J, Li Y, Wang L, Wilkerson JL, Sweeney CR, Pereira RF, Sumida DH, Maschek JA, Cox JE, Kaddai V, Lancaster GI, Siddique MM, Poss A, Pearson M, Satapati S, Zhou H, McLaren DG, Previs SF, Chen Y, Qian Y, Petrov A, Wu M, Shen X, Yao J, Nunes CN, Howard AD, Wang L, Erion MD, Rutter J, Holland WL, Kelley DE, Summers SA.Science. 2019 Jul 26;365(6451):386-392. doi: 10.1126/science.aav3722. Epub 2019 Jul 4.PMID:31273070
T cell-mediated regulation of the microbiota protects against obesity. Petersen C, Bell R, Klag KA, Lee SH, Soto R, Ghazaryan A, Buhrke K, Ekiz HA, Ost KS, Boudina S, O’Connell RM, Cox JE, Villanueva CJ, Stephens WZ, Round JL. Science. 2019 Jul 26;365(6451). pii: eaat9351. doi: 10.1126/science.aat9351. PMID:31346040
Impaired skeletal muscle mitochondrial pyruvate uptake rewires glucose metabolism to drive whole-body leanness. Sharma A, Oonthonpan L, Sheldon RD, Rauckhorst AJ, Zhu Z, Tompkins SC, Cho K, Grzesik WJ, Gray LR, Scerbo DA, Pewa AD, Cushing EM, Dyle MC, Cox JE, Adams C, Davies BS, Shields RK, Norris AW, Patti G, Zingman LV, Taylor EB. Elife. 2019 Jul 18;8. pii: e45873. doi: 10.7554/eLife.45873. PMID:31305240
Thrombotic risk in congenital erythrocytosis due to up-regulated hypoxia sensing is not associated with elevated hematocrit. Gordeuk VR, Miasnikova GY, Sergueeva AI, Lorenzo FR, Zhang X, Song J, Stockton DW, Prchal JT. Haematologica. 2019 Jul 9. pii: haematol.2019.216267. doi: 10.3324/haematol.2019.216267. [Epub ahead of print]PMID:31289208
2019 June
Redox Regulation of the Mitochondrial Quality Control Protease Oma1. Bohovych I, Dietz JV, Swenson S, Zahayko N, Khalimonchuk O. Antioxid Redox Signal. 2019 Jun 18. doi: 10.1089/ars.2018.7642. [Epub ahead of print] PMID:31011300
Growth Factor Independence 1B-Mediated Transcriptional Repression and Lineage Allocation Require Lysine-Specific Demethylase 1-Dependent Recruitment of the BHC Complex. McClellan D, Casey MJ, Bareyan D, Lucente H, Ours C, Velinder M, Singer J, Lone MD, Sun W, Coria Y, Mason CC, Engel ME. Mol Cell Biol. 2019 Jun 13;39(13). pii: e00020-19. doi: 10.1128/MCB.00020-19. Print 2019 Jul 1.PMID:30988160
2019 May
Ferritin in serum and urine: A pilot study. Bahr TM, Christensen RD, Ward DM, Meng F, Jackson LK, Doyle K, Christensen DR, Harvey AG, Yaish HM. Blood Cells Mol Dis. 2019 May;76:59-62. doi: 10.1016/j.bcmd.2019.02.001. Epub 2019 Feb 8. PMID;30777730
Phase evolution of conversion-type electrode for lithium ion batteries. Li J, Hwang S, Guo F, Li S, Chen Z, Kou R, Sun K, Sun CJ, Gan H, Yu A, Stach EA, Zhou H, Su D. Nat Commun. 2019 May 20;10(1):2224. doi: 10.1038/s41467-019-09931-2. PMID:31110173
Activation of PASK by mTORC1 is required for the onset of the terminal differentiation program. Kikani CK, Wu X, Fogarty S, Kang SAW, Dephoure N, Gygi SP, Sabatini DM, Rutter J. Proc Natl Acad Sci U S A. 2019 May 21;116(21):10382-10391. doi: 10.1073/pnas.1804013116. Epub 2019 May 9. PMID:31072927
Hypoxia-Associated Factor (HAF) Mediates Neurofibromin Ubiquitination and Degradation Leading to Ras-ERK Pathway Activation in Hypoxia. Green YS, Sargis T, Reichert EC, Rudasi E, Fuja D, Jonasch E, Koh MY. Mol Cancer Res. 2019 May;17(5):1220-1232. doi: 10.1158/1541-7786.MCR-18-1080. Epub 2019 Jan 31. PMID:30705246
International Porphyria Molecular Diagnostic Collaborative: an evidence-based database of verified pathogenic and benign variants for the porphyrias. Chen B, Whatley S, Badminton M, Aarsand AK, Anderson KE, Bissell DM, Bonkovsky HL, Cappellini MD, Floderus Y, Friesema ECH, Gouya L, Harper P, Kauppinen R, Loskove Y, Martásek P, Phillips JD, Puy H, Sandberg S, Schmitt C, To-Figueras J, Weiss Y, Yasuda M, Deybach JC, Desnick RJ. Genet Med. 2019 May 10. doi: 10.1038/s41436-019-0537-7. [Epub ahead of print] PMID:31073229
Glycogen Synthase Kinase-3α Promotes Fatty Acid Uptake and Lipotoxic Cardiomyopathy. Nakamura M, Liu T, Husain S, Zhai P, Warren JS, Hsu CP, Matsuda T, Phiel CJ, Cox JE, Tian B, Li H, Sadoshima J. Cell Metab. 2019 May 7;29(5):1119-1134.e12. doi: 10.1016/j.cmet.2019.01.005. Epub 2019 Feb 7. PMID:30745182
Oct1/Pou2f1 is selectively required for colon regeneration and regulates colon malignancy.Vazquez-Arreguin K, Bensard C, Schell JC, Swanson E, Chen X, Rutter J, Tantin D. (2019) PLoS Genetics. 2019 May 6;15(5):e1007687. PMID:31059499
2019 Apr
Re-evaluation of hematocrit as a determinant of thrombotic risk in erythrocytosis. Gordeuk VR, Key NS, Prchal JT. Haematologica. 2019 Apr;104(4):653-658. doi: 10.3324/haematol.2018.210732. Epub 2019 Mar 14. PMID:30872370
Proteolytic regulation of mitochondrial dynamics. Dietz JV, Bohovych I, Viana MP, Khalimonchuk O. Mitochondrion. 2019 Apr 25. pii: S1567-7249(19)30047-9. doi: 10.1016/j.mito.2019.04.008. [Epub ahead of print] Review. PMID:31029640
Development of a symptom assessment in patients with myelofibrosis: qualitative study findings. Mesa RA, Su Y, Woolfson A, Prchal JT, Turnbull K, Jabbour E, Scherber R, Shields AL, Krohe M, Ojo F, Pompilus F, Cappelleri JC, Harrison C. Health Qual Life Outcomes. 2019 Apr 11;17(1):61. doi: 10.1186/s12955-019-1121-1. PMID:30975150
Energetic Trade-Offs and Hypometabolic States Promote Disease Tolerance. Ganeshan K, Nikkanen J, Man K, Leong YA, Sogawa Y, Maschek JA, Van Ry T, Chagwedera DN, Cox JE, Chawla A. Cell. 2019 Apr 4;177(2):399-413.e12. doi: 10.1016/j.cell.2019.01.050. Epub 2019 Mar 7. PMID:30853215
2019 Feb
An extracellular histidine-containing motif in the zinc transporter ZIP4 plays a role in zinc sensing and zinc-induced endocytosis in mammalian cells. Chun H, Korolnek T, Lee CJ, Coyne HJ 3rd, Winge DR, Kim BE, Petris MJ. J Biol Chem. 2019 Feb 22;294(8):2815-2826. doi: 10.1074/jbc.RA118.005203. Epub 2018 Dec 28. PMID:30593504
Icariin Protects Bone Marrow Mesenchymal Stem Cells Against Iron Overload Induced Dysfunction Through Mitochondrial Fusion and Fission, PI3K/AKT/mTOR and MAPK Pathways.Yao X, Jing X, Guo J, Sun K, Deng Y, Zhang Y, Guo F, Ye Y. Front Pharmacol. 2019 Feb 28;10:163. doi: 10.3389/fphar.2019.00163. eCollection 2019. PMID:30873034
Harderoporphyria: Case of lifelong photosensitivity associated with compound heterozygous coproporphyrinogen oxidase (CPOX) mutations. Moghe A, Ramanujam VMS, Phillips JD, Desnick RJ, Anderson KE. Mol Genet Metab Rep. 2019 Feb 19;19:100457. doi: 10.1016/j.ymgmr.2019.100457. eCollection 2019 Jun. PMID:30828546
2019 Jan
Screening umbilical cord blood for congenital Iron deficiency. MacQueen, BC, Christensen, RD, Baer VL, Ward, DM, Snow GL. (2019). Blood Cells Mol Dis. 77:95-100 PMID: 31005753
Diagnosing or ruling out polycythemia vera in patients with erythrocytosis. Prchal JT. Clin Adv Hematol Oncol. 2019 Jan;17(1):24-27. PMID:30843892
Identification and characterization of 40 novel hydroxymethylbilane synthase mutations that cause acute intermittent porphyria. Chen B, Solis-Villa C, Erwin AL, Balwani M, Nazarenko I, Phillips JD, Desnick RJ, Yasuda M. J Inherit Metab Dis. 2019 Jan;42(1):186-194. doi: 10.1002/jimd.12040. PMID: 30740734
Pilot Study of Novel Intermittent Fasting Effects on Metabolomic and Trimethylamine N-oxide Changes During 24-hour Water-Only Fasting in the FEELGOOD Trial. Washburn RL, Cox JE, Muhlestein JB, May HT, Carlquist JF, Le VT, Anderson JL, Horne BD. Nutrients. 2019 Jan 23;11(2). pii: E246. doi: 10.3390/nu11020246. PMID:30678028
The mitochondrial heme metabolon: Insights into the complex(ity) of heme synthesis and distribution. Piel RB 3rd, Dailey HA Jr, Medlock AE. Mol Genet Metab. 2019 Jan 17. pii: S1096-7192(18)30634-6. doi: 10.1016/j.ymgme.2019.01.006. [Epub ahead of print] Review. PMID:30709775
Imbalanced sphingolipid signaling is maintained as a core proponent of a cancerous phenotype in spite of metabolic pressure and epigenetic drift. Speirs MMP, Swensen AC, Chan TY, Jones PM, Holman JC, Harris MB, Maschek JA, Cox JE, Carson RH, Hill JT, Andersen JL, Prince JT, Price JC. Oncotarget. 2019 Jan 11;10(4):449-479. doi: 10.18632/oncotarget.26533. eCollection 2019 Jan 11. PMID:30728898
Homozygous hydroxymethylbilane synthase knock-in mice provide pathogenic insights into the severe neurological impairments present in human homozygous dominant acute intermittent porphyria. Yasuda M, Gan L, Chen B, Yu C, Zhang J, Gama-Sosa MA, Pollak DD, Berger S, Phillips JD, Edelmann W, Desnick RJ. Hum Mol Genet. 2019 Jan 7. doi: 10.1093/hmg/ddz003. [Epub ahead of print] PMID:30615115
2018
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
The AAA ATPase Afg1 preserves mitochondrial fidelity and cellular health by maintaining mitochondrial matrix proteostasis. Germany EM, Zahayko N, Huebsch ML, Fox JL, Prahlad V, Khalimonchuk O. J Cell Sci. 2018 Nov 21;131(22). pii: jcs219956. doi: 10.1242/jcs.219956. PMID:30301782
Molecular Basis for the Evolution of Species-Specific Hemoglobin Capture by Staphylococcus aureus. Choby JE, Buechi HB, Farrand AJ, Skaar EP, Barber MF. MBio. 2018 Nov 20;9(6). pii: e01524-18. doi: 10.1128/mBio.01524-18. PMID:30459189
Glutamine via α-ketoglutarate dehydrogenase provides succinyl-CoA for heme synthesis during erythropoiesis. Burch JS, Marcero JR, Maschek JA, Cox JE, Jackson LK, Medlock AE, Phillips JD, Dailey HA Jr. Blood. 2018 Sep 6;132(10):987-998. doi: 10.1182/blood-2018-01-829036. Epub 2018 Jul 10. PMID:29991557
Cirrhosis in Hemochromatosis: Independent Risk Factors in 368 HFE p.C282Y Homozygotes. Barton JC, McLaren CE, Chen WP, Ramm GA, Anderson GJ, Powell LW, Subramaniam VN, Adams PC, Phatak PD, Gurrin LC, Phillips JD, Parker CJ, Emond MJ, McLaren GD. Ann Hepatol. 2018 Aug 24;17(5):871-879. doi: 10.5604/01.3001.0012.3169.PMID:30145563
ACP Acylation Is an Acetyl-CoA-Dependent Modification Required for Electron Transport Chain Assembly. Van Vranken JG, Nowinski SM, Clowers KJ, Jeong MY, Ouyang Y, Berg JA, Gygi JP, Gygi SP, Winge DR, Rutter J. Mol Cell. 2018 Aug 16;71(4):567-580.e4. doi: 10.1016/j.molcel.2018.06.039.PMID:30118679