Hieronim Jakubowski


Affiliation: New Jersey
Country: USA


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    Jakubowski H. Protein homocysteinylation: possible mechanism underlying pathological consequences of elevated homocysteine levels. FASEB J. 1999;13:2277-83 pubmed
    ..Jakubowski, H. Protein homocysteinylation: possible mechanism underlying pathological consequences of elevated homocysteine levels. ..
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    Jakubowski H. Translational accuracy of aminoacyl-tRNA synthetases: implications for atherosclerosis. J Nutr. 2001;131:2983S-7S pubmed
  3. Jakubowski H. Quality control in tRNA charging. Wiley Interdiscip Rev RNA. 2012;3:295-310 pubmed publisher
    ..Elevated levels of mistranslation and the synthesis of statistical proteins can be beneficial for pathogens by increasing phenotypic variation essential for the evasion of host defenses. ..
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    Jakubowski H. Aminoacylation of coenzyme A and pantetheine by aminoacyl-tRNA synthetases: possible link between noncoded and coded peptide synthesis. Biochemistry. 1998;37:5147-53 pubmed
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    Jakubowski H, Guranowski A. Metabolism of homocysteine-thiolactone in plants. J Biol Chem. 2003;278:6765-70 pubmed
    ..Thus, Hcy-thiolactone and Hcy bound to protein by an amide (or peptide) linkage (Hcy-N-protein) are significant components of plant Hcy metabolism. ..
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    Jakubowski H. Quality control in tRNA charging -- editing of homocysteine. Acta Biochim Pol. 2011;58:149-63 pubmed
    ..Excessive homocysteine thiolactone synthesis in hyperhomocysteinemia caused by genetic or nutritional deficiencies is linked to human vascular and neurological diseases. ..
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    Jakubowski H. Misacylation of tRNALys with noncognate amino acids by lysyl-tRNA synthetase. Biochemistry. 1999;38:8088-93 pubmed
    ..A novel feature of this editing reaction is that ornithine lactam is formed by the facile cyclization of ornithyl adenylate. ..
  8. Jakubowski H, Perla Kajan J, Finnell R, Cabrera R, Wang H, Gupta S, et al. Genetic or nutritional disorders in homocysteine or folate metabolism increase protein N-homocysteinylation in mice. FASEB J. 2009;23:1721-7 pubmed publisher
    ..These findings provide evidence that N-Hcy-protein is an important metabolite associated with Hcy pathophysiology in the mouse. ..
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    Jakubowski H. Aminoacyl thioester chemistry of class II aminoacyl-tRNA synthetases. Biochemistry. 1997;36:11077-85 pubmed
    ..That the thiol-binding subsite exists also in AspRS and SerRS, which do not need editing function, suggests that these class II enzymes possess vestigial editing functions. ..

More Information


  1. Jakubowski H, Boers G, Strauss K. Mutations in cystathionine beta-synthase or methylenetetrahydrofolate reductase gene increase N-homocysteinylated protein levels in humans. FASEB J. 2008;22:4071-6 pubmed publisher
    ..We also show that CBS-deficient patients have significantly elevated plasma levels of prothrombotic N-Hcy-fibrinogen. These results provide a possible explanation for increased atherothrombosis observed in CBS-deficient patients. ..
  2. Jakubowski H. The role of paraoxonase 1 in the detoxification of homocysteine thiolactone. Adv Exp Med Biol. 2010;660:113-27 pubmed publisher
    ..This article outlines Hcy-thiolactone pathobiology and reviews evidence documenting the role of PON1 in minimizing Hcy-thiolactone and N-Hcy-protein accumulation. ..