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RICHARD SIFERS

TitleProfessor
InstitutionBaylor College of Medicine
DepartmentDepartment of Pathology & Immunology
DivisionPathology
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    Other Positions
    TitleProfessor
    InstitutionBaylor College of Medicine
    DepartmentDepartment of Molecular & Cellular Biology
    DivisionMolecular & Cellular Biology

    TitleProfessor
    InstitutionBaylor College of Medicine
    DepartmentDepartment of Molecular Physiology & Biophysics
    DivisionMolecular Physiology & Biophysics

    TitleProfessor
    InstitutionBaylor College of Medicine
    DepartmentHuffington Center on Aging
    DivisionHuffington Ctr on Aging


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    F32HL007343     (SIFERS, RICHARD NORMAN)Mar 15, 1986
    NIH/NHLBI
    ALPHA-1-ANTITRYPSIN DEFICIENCY
    Role: Principal Investigator

    R29DK042806     (SIFERS, RICHARD NORMAN)May 1, 1991 - Oct 31, 1996
    NIH/NIDDK
    PREGOLGI DEGRADATION OF MUTANT SECRETORY PROTEINS
    Role: Principal Investigator

    R01HL062553     (SIFERS, RICHARD NORMAN)Feb 1, 2000 - Jan 31, 2005
    NIH/NHLBI
    PATHOBIOGENESIS OF HERITABLE PULMONARY EMPHYSEMA
    Role: Principal Investigator

    K12GM084897     (SLAUGHTER, GAYLE)Aug 1, 2008 - Jul 31, 2017
    NIH/NIGMS
    Research Education and Career Horizons Program
    Role: Co-Principal Investigator

    R01DK064232     (SIFERS, RICHARD NORMAN)Jul 17, 2009 - Jun 30, 2011
    NIH/NIDDK
    Post-translational Surveillance of Genome Expression
    Role: Principal Investigator

    Collapse Bibliographic 
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    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Faculty can login to make corrections and additions.
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    1. Bajaj L, Sharma J, di Ronza A, Zhang P, Eblimit A, Pal R, Roman D, Collette JR, Booth C, Chang KT, Sifers RN, Jung SY, Weimer JM, Chen R, Schekman RW, Sardiello M. A CLN6-CLN8 complex recruits lysosomal enzymes at the ER for Golgi transfer. J Clin Invest. 2020 Aug 03; 130(8):4118-4132. PMID: 32597833.
      View in: PubMed
    2. Wang L, Adamski CJ, Bondar VV, Craigen E, Collette JR, Pang K, Han K, Jain A, Y Jung S, Liu Z, Sifers RN, Holder JL, Zoghbi HY. A kinome-wide RNAi screen identifies ERK2 as a druggable regulator of Shank3 stability. Mol Psychiatry. 2019 Jan 29. PMID: 30696942.
      View in: PubMed
    3. di Ronza A, Bajaj L, Sharma J, Sanagasetti D, Lotfi P, Adamski CJ, Collette J, Palmieri M, Amawi A, Popp L, Chang KT, Meschini MC, Leung HE, Segatori L, Simonati A, Sifers RN, Santorelli FM, Sardiello M. CLN8 is an endoplasmic reticulum cargo receptor that regulates lysosome biogenesis. Nat Cell Biol. 2018 12; 20(12):1370-1377. PMID: 30397314.
      View in: PubMed
    4. Mamrosh JL, Lee JM, Wagner M, Stambrook PJ, Whitby RJ, Sifers RN, Wu SP, Tsai MJ, DeMayo FJ, Moore DD. Correction: Nuclear receptor LRH-1/NR5A2 is required and targetable for liver endoplasmic reticulum stress resolution. Elife. 2015 Jul 24; 4:e10084. PMID: 26205891.
      View in: PubMed
    5. Mamrosh JL, Lee JM, Wagner M, Stambrook PJ, Whitby RJ, Sifers RN, Wu SP, Tsai MJ, Demayo FJ, Moore DD. Nuclear receptor LRH-1/NR5A2 is required and targetable for liver endoplasmic reticulum stress resolution. Elife. 2014 Apr 15; 3:e01694. PMID: 24737860.
      View in: PubMed
    6. Iannotti MJ, Figard L, Sokac AM, Sifers RN. A Golgi-localized mannosidase (MAN1B1) plays a non-enzymatic gatekeeper role in protein biosynthetic quality control. J Biol Chem. 2014 Apr 25; 289(17):11844-58. PMID: 24627495.
      View in: PubMed
    7. Pan S, Cheng X, Chen H, Castro PD, Ittmann MM, Hutson AW, Zapata SK, Sifers RN. ERManI is a target of miR-125b and promotes transformation phenotypes in hepatocellular carcinoma (HCC). PLoS One. 2013; 8(8):e72829. PMID: 23940818.
      View in: PubMed
    8. Sifers RN. Resurrecting the protein fold for disease intervention. Chem Biol. 2013 Mar 21; 20(3):298-300. PMID: 23521788.
      View in: PubMed
    9. Pan S, Cheng X, Sifers RN. Golgi-situated endoplasmic reticulum a-1, 2-mannosidase contributes to the retrieval of ERAD substrates through a direct interaction with ?-COP. Mol Biol Cell. 2013 Apr; 24(8):1111-21. PMID: 23427261.
      View in: PubMed
    10. Fong JJ, Nguyen BL, Bridger R, Medrano EE, Wells L, Pan S, Sifers RN. ß-N-Acetylglucosamine (O-GlcNAc) is a novel regulator of mitosis-specific phosphorylations on histone H3. J Biol Chem. 2012 Apr 06; 287(15):12195-203. PMID: 22371497.
      View in: PubMed
    11. Pan S, Wang S, Utama B, Huang L, Blok N, Estes MK, Moremen KW, Sifers RN. Golgi localization of ERManI defines spatial separation of the mammalian glycoprotein quality control system. Mol Biol Cell. 2011 Aug 15; 22(16):2810-22. PMID: 21697506.
      View in: PubMed
    12. Pan S, Iannotti MJ, Sifers RN. Analysis of serpin secretion, misfolding, and surveillance in the endoplasmic reticulum. Methods Enzymol. 2011; 499:1-16. PMID: 21683246.
      View in: PubMed
    13. Sifers RN. Intracellular processing of alpha1-antitrypsin. Proc Am Thorac Soc. 2010 Nov; 7(6):376-80. PMID: 21030516.
      View in: PubMed
    14. Sifers RN. Medicine. Clearing conformational disease. Science. 2010 Jul 09; 329(5988):154-5. PMID: 20616258.
      View in: PubMed
    15. Sifers RN. Manipulating proteostasis. Nat Chem Biol. 2010 Jun; 6(6):400-1. PMID: 20479748.
      View in: PubMed
    16. Sifers RN. Defining the ERAD connection: assembly required. Cell Cycle. 2009 Dec 15; 8(24):4026-7. PMID: 19959932.
      View in: PubMed
    17. Pan S, Huang L, McPherson J, Muzny D, Rouhani F, Brantly M, Gibbs R, Sifers RN. Single nucleotide polymorphism-mediated translational suppression of endoplasmic reticulum mannosidase I modifies the onset of end-stage liver disease in alpha1-antitrypsin deficiency. Hepatology. 2009 Jul; 50(1):275-81. PMID: 19444872.
      View in: PubMed
    18. Termine DJ, Moremen KW, Sifers RN. The mammalian UPR boosts glycoprotein ERAD by suppressing the proteolytic downregulation of ER mannosidase I. J Cell Sci. 2009 Apr 01; 122(Pt 7):976-84. PMID: 19258393.
      View in: PubMed
    19. Pan S, Wang R, Zhou X, Corvera J, Kloc M, Sifers R, Gallick GE, Lin SH, Kuang J. Extracellular Alix regulates integrin-mediated cell adhesions and extracellular matrix assembly. EMBO J. 2008 Aug 06; 27(15):2077-90. PMID: 18636094.
      View in: PubMed
    20. Mallya M, Phillips RL, Saldanha SA, Gooptu B, Brown SC, Termine DJ, Shirvani AM, Wu Y, Sifers RN, Abagyan R, Lomas DA. Small molecules block the polymerization of Z alpha1-antitrypsin and increase the clearance of intracellular aggregates. J Med Chem. 2007 Nov 01; 50(22):5357-63. PMID: 17918823.
      View in: PubMed
    21. Wu Y, Termine DJ, Swulius MT, Moremen KW, Sifers RN. Human endoplasmic reticulum mannosidase I is subject to regulated proteolysis. J Biol Chem. 2007 Feb 16; 282(7):4841-9. PMID: 17166854.
      View in: PubMed
    22. Termine D, Wu Y, Liu Y, Sifers RN. Alpha1-antitrypsin as model to assess glycan function in endoplasmic reticulum. Methods. 2005 Apr; 35(4):348-53. PMID: 15804606.
      View in: PubMed
    23. Mast SW, Diekman K, Karaveg K, Davis A, Sifers RN, Moremen KW. Human EDEM2, a novel homolog of family 47 glycosidases, is involved in ER-associated degradation of glycoproteins. Glycobiology. 2005 Apr; 15(4):421-36. PMID: 15537790.
      View in: PubMed
    24. Sifers RN. Insights into checkpoint capacity. Nat Struct Mol Biol. 2004 Feb; 11(2):108-9. PMID: 14749767.
      View in: PubMed
    25. Wu Y, Swulius MT, Moremen KW, Sifers RN. Elucidation of the molecular logic by which misfolded alpha 1-antitrypsin is preferentially selected for degradation. Proc Natl Acad Sci U S A. 2003 Jul 08; 100(14):8229-34. PMID: 12815101.
      View in: PubMed
    26. Sifers RN. Cell biology. Protein degradation unlocked. Science. 2003 Feb 28; 299(5611):1330-1. PMID: 12610289.
      View in: PubMed
    27. Han B, Luo G, Shi ZZ, Barrios R, Atwood D, Liu W, Habib GM, Sifers RN, Corry DB, Lieberman MW. Gamma-glutamyl leukotrienase, a novel endothelial membrane protein, is specifically responsible for leukotriene D(4) formation in vivo. Am J Pathol. 2002 Aug; 161(2):481-90. PMID: 12163373.
      View in: PubMed
    28. Cabral CM, Liu Y, Moremen KW, Sifers RN. Organizational diversity among distinct glycoprotein endoplasmic reticulum-associated degradation programs. Mol Biol Cell. 2002 Aug; 13(8):2639-50. PMID: 12181335.
      View in: PubMed
    29. Davis RL, Shrimpton AE, Carrell RW, Lomas DA, Gerhard L, Baumann B, Lawrence DA, Yepes M, Kim TS, Ghetti B, Piccardo P, Takao M, Lacbawan F, Muenke M, Sifers RN, Bradshaw CB, Kent PF, Collins GH, Larocca D, Holohan PD. Association between conformational mutations in neuroserpin and onset and severity of dementia. Lancet. 2002 Jun 29; 359(9325):2242-7. PMID: 12103288.
      View in: PubMed
    30. Cabral CM, Liu Y, Sifers RN. Dissecting glycoprotein quality control in the secretory pathway. Trends Biochem Sci. 2001 Oct; 26(10):619-24. PMID: 11590015.
      View in: PubMed
    31. Younes M, Pathak M, Finnie D, Sifers RN, Liu Y, Schwartz MR. Expression of the neutral amino acids transporter ASCT1 in esophageal carcinomas. Anticancer Res. 2000 Sep-Oct; 20(5C):3775-9. PMID: 11268453.
      View in: PubMed
    32. Ran Q, Wadhwa R, Kawai R, Kaul SC, Sifers RN, Bick RJ, Smith JR, Pereira-Smith OM. Extramitochondrial localization of mortalin/mthsp70/PBP74/GRP75. Biochem Biophys Res Commun. 2000 Aug 18; 275(1):174-9. PMID: 10944461.
      View in: PubMed
    33. Cabral CM, Choudhury P, Liu Y, Sifers RN. Processing by endoplasmic reticulum mannosidases partitions a secretion-impaired glycoprotein into distinct disposal pathways. J Biol Chem. 2000 Aug 11; 275(32):25015-22. PMID: 10827201.
      View in: PubMed
    34. Liu Y, Choudhury P, Cabral CM, Sifers RN. Oligosaccharide modification in the early secretory pathway directs the selection of a misfolded glycoprotein for degradation by the proteasome. J Biol Chem. 1999 Feb 26; 274(9):5861-7. PMID: 10026209.
      View in: PubMed
    35. Choudhury P, Liu Y, Bick RJ, Sifers RN. Intracellular association between UDP-glucose:glycoprotein glucosyltransferase and an incompletely folded variant of alpha1-antitrypsin. J Biol Chem. 1997 May 16; 272(20):13446-51. PMID: 9148970.
      View in: PubMed
    36. Liu Y, Choudhury P, Cabral CM, Sifers RN. Intracellular disposal of incompletely folded human alpha1-antitrypsin involves release from calnexin and post-translational trimming of asparagine-linked oligosaccharides. J Biol Chem. 1997 Mar 21; 272(12):7946-51. PMID: 9065464.
      View in: PubMed
    37. Sifers RN. Defective protein folding as a cause of disease. Nat Struct Biol. 1995 May; 2(5):355-7. PMID: 7664089.
      View in: PubMed
    38. Le A, Steiner JL, Ferrell GA, Shaker JC, Sifers RN. Association between calnexin and a secretion-incompetent variant of human alpha 1-antitrypsin. J Biol Chem. 1994 Mar 11; 269(10):7514-9. PMID: 8125971.
      View in: PubMed
    39. Sifers RN, Finegold MJ, Woo SL. Molecular biology and genetics of alpha 1-antitrypsin deficiency. Semin Liver Dis. 1992 Aug; 12(3):301-10. PMID: 1439881.
      View in: PubMed
    40. Sifers RN. Protein transport. Z and the insoluble answer. Nature. 1992 Jun 18; 357(6379):541-2. PMID: 1608463.
      View in: PubMed
    41. Le A, Ferrell GA, Dishon DS, Le QQ, Sifers RN. Soluble aggregates of the human PiZ alpha 1-antitrypsin variant are degraded within the endoplasmic reticulum by a mechanism sensitive to inhibitors of protein synthesis. J Biol Chem. 1992 Jan 15; 267(2):1072-80. PMID: 1530934.
      View in: PubMed
    42. Graham KS, Le A, Sifers RN. Accumulation of the insoluble PiZ variant of human alpha 1-antitrypsin within the hepatic endoplasmic reticulum does not elevate the steady-state level of grp78/BiP. J Biol Chem. 1990 Nov 25; 265(33):20463-8. PMID: 2122976.
      View in: PubMed
    43. Le A, Graham KS, Sifers RN. Intracellular degradation of the transport-impaired human PiZ alpha 1-antitrypsin variant. Biochemical mapping of the degradative event among compartments of the secretory pathway. J Biol Chem. 1990 Aug 15; 265(23):14001-7. PMID: 2380201.
      View in: PubMed
    44. Sifers RN, Ledley FD, Reed-Fourquet L, Ledbetter DH, Ledbetter SA, Woo SL. Complete cDNA sequence and chromosomal localization of mouse alpha 1-antitrypsin. Genomics. 1990 Jan; 6(1):100-4. PMID: 2303252.
      View in: PubMed
    45. Sifers RN, Finegold MJ, Woo SL. Alpha-1-antitrypsin deficiency: accumulation or degradation of mutant variants within the hepatic endoplasmic reticulum. Am J Respir Cell Mol Biol. 1989 Nov; 1(5):341-5. PMID: 2700304.
      View in: PubMed
    46. Sifers RN, Rogers BB, Hawkins HK, Finegold MJ, Woo SL. Elevated synthesis of human alpha 1-antitrypsin hinders the secretion of murine alpha 1-antitrypsin from hepatocytes of transgenic mice. J Biol Chem. 1989 Sep 15; 264(26):15696-700. PMID: 2788654.
      View in: PubMed
    47. Sifers RN, Shen RF, Woo SL. Genetic control of human alpha-1-antitrypsin. Mol Biol Med. 1989 Apr; 6(2):127-35. PMID: 2693888.
      View in: PubMed
    48. Carlson JA, Rogers BB, Sifers RN, Finegold MJ, Clift SM, DeMayo FJ, Bullock DW, Woo SL. Accumulation of PiZ alpha 1-antitrypsin causes liver damage in transgenic mice. J Clin Invest. 1989 Apr; 83(4):1183-90. PMID: 2784798.
      View in: PubMed
    49. Shen RF, Clift SM, DeMayo JL, Sifers RN, Finegold MJ, Woo SL. Tissue-specific regulation of human alpha 1-antitrypsin gene expression in transgenic mice. DNA. 1989 Mar; 8(2):101-8. PMID: 2784378.
      View in: PubMed
    50. Sifers RN, Hardick CP, Woo SL. Disruption of the 290-342 salt bridge is not responsible for the secretory defect of the PiZ alpha 1-antitrypsin variant. J Biol Chem. 1989 Feb 15; 264(5):2997-3001. PMID: 2563374.
      View in: PubMed
    51. Cohen AB, Woo S, Kelley R, Rosenbloom J, Crystal RG, Sifers R, Perlmutter D. Molecular genetics in pulmonary emphysema. Am Rev Respir Dis. 1988 Oct; 138(4):1041-3. PMID: 3264478.
      View in: PubMed
    52. Carlson JA, Rogers BB, Sifers RN, Hawkins HK, Finegold MJ, Woo SL. Multiple tissues express alpha 1-antitrypsin in transgenic mice and man. J Clin Invest. 1988 Jul; 82(1):26-36. PMID: 3260605.
      View in: PubMed
    53. Sifers RN, Brashears-Macatee S, Kidd VJ, Muensch H, Woo SL. A frameshift mutation results in a truncated alpha 1-antitrypsin that is retained within the rough endoplasmic reticulum. J Biol Chem. 1988 May 25; 263(15):7330-5. PMID: 3259232.
      View in: PubMed
    54. Bao JJ, Reed-Fourquet L, Sifers RN, Kidd VJ, Woo SL. Molecular structure and sequence homology of a gene related to alpha 1-antitrypsin in the human genome. Genomics. 1988 Feb; 2(2):165-73. PMID: 2842251.
      View in: PubMed
    55. Bao JJ, Sifers RN, Kidd VJ, Ledley FD, Woo SL. Molecular evolution of serpins: homologous structure of the human alpha 1-antichymotrypsin and alpha 1-antitrypsin genes. Biochemistry. 1987 Dec 01; 26(24):7755-9. PMID: 3501319.
      View in: PubMed
    56. Shen RF, Li Y, Sifers RN, Wang H, Hardick C, Tsai SY, Woo SL. Tissue-specific expression of the human alpha 1-antitrypsin gene is controlled by multiple cis-regulatory elements. Nucleic Acids Res. 1987 Oct 26; 15(20):8399-415. PMID: 2823229.
      View in: PubMed
    57. Sifers RN, Carlson JA, Clift SM, DeMayo FJ, Bullock DW, Woo SL. Tissue specific expression of the human alpha-1-antitrypsin gene in transgenic mice. Nucleic Acids Res. 1987 Feb 25; 15(4):1459-75. PMID: 3029716.
      View in: PubMed
    58. Hejtmancik JF, Sifers RN, Ward PA, Harris S, Mansfield T, Cox DW. Prenatal diagnosis of alpha 1-antitrypsin deficiency by restriction fragment length polymorphisms, and comparison with oligonucleotide probe analysis. Lancet. 1986 Oct 04; 2(8510):767-70. PMID: 2876232.
      View in: PubMed
    59. Sifers RN, Mayes JS, Nordquist RE. Loss of electron-dense lamellar material from Fabry's disease fibroblasts after enzyme replacement. Hum Genet. 1983; 65(1):85-7. PMID: 6315565.
      View in: PubMed
    60. Mayes JS, Cray EL, Dell VA, Scheerer JB, Sifers RN. Endocytosis of lysosomal alpha-galactosidase A by cultured fibroblasts from patients with Fabry disease. Am J Hum Genet. 1982 Jul; 34(4):602-10. PMID: 6285697.
      View in: PubMed
    61. Mayes JS, Scheerer JB, Sifers RN, Donaldson ML. Differential assay for lysosomal alpha-galactosidases in human tissues and its application to Fabry's disease. Clin Chim Acta. 1981 May 05; 112(2):247-51. PMID: 6263521.
      View in: PubMed
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