© 2000 American Heart Association, Inc.
Special Article |
Homocysteine and Its Disulfide Derivatives
A Suggested Consensus Terminology
From the Laboratory of Molecular Biology (S.H.M.), National Institute of Mental Health, Bethesda, Md; the Veterans Administration Medical Center and George Washington University (J.D.F.), Washington, DC; the LOCUS for Homocysteine and Related Vitamins (H.R., P.M.U.), Armauer Hansens hus, University of Bergen, Bergen, Norway; the Oregon Regional Primate Research Center (M.R.M.), Beaverton, and Oregon Health Sciences University, Portland; the Department of Internal Medicine (S.R.L.), University of Iowa College of Medicine, Iowa City; the Department of Cell Biology (D.W.J.), Lerner Research Institute, Cleveland Clinic Foundation, Cleveland, Ohio; the Department of Medicine (L.B.), County Hospital, Kalmar, Sweden; the Department of Biochemical Genetics (B.W.), New Children’s Hospital, Sydney, Australia; the Department of Cardiovascular Medicine (D.E.L.W.), University of New South Wales, Prince of Wales Hospital, Sydney, Australia; the Department of Pediatrics (H.J.B.), University Hospital Nijmegen, Nijmegen, Netherlands; the Division of Hematology (S.P.S., R.H.A.), University of Colorado Health Sciences Center, Denver; and the Jean Mayer USDA Human Nutrition Research Center (J.S., I.H.R.), Tufts University, Boston, Mass.
Correspondence to S. Harvey Mudd, MD, NIMH/DIRP/LMB, Bldg 36, Room 1B-08, 36 Convent Dr MSC 4034, Bethesda, MD 20892-4034. E-mail shm@codon.nih.gov
Key Words: homocysteine • homocystine • disulfide • hyperhomocysteinemia • tHcy
In recent years, there has been an upsurge of interest in elevation of the plasma concentration of homocysteine and closely related metabolites as an independent risk factor for cardiovascular disease (reviewed, for example, in References 1 through 31 2 3 ). Homocysteine itself is a thiol- (sulfhydryl-) containing amino acid, but in normal human plasma and other tissues, a variety of related disulfide derivatives may be present. Different authors have written about these compounds and their effects by using differing terminologies. To promote clarity of meaning and to minimize uncertainty, perhaps even confusion, it is important that each article discussing these compounds either defines explicitly the terms and/or abbreviations used or cites a prior publication in which such definitions are provided. Optimally, a more uniform consensus terminology will be developed and adopted by the field. This article describes very briefly the structures of the relevant compounds and sets forth terms and abbreviations that, it is hoped, may provide a basis for such a consensus.
The word "homocysteine" was used first by Du Vigneaud and coworkers
>65 years ago when they discovered this compound4 and
provided definitive proof that it had the structure of a thiol (ie,
sulfhydryl) 4-carbon 
-amino acid:
HSCH2CH2CH(NH2)COOH.5
The symmetrical disulfide of homocysteine was termed "homocystine,"
both names being chosen to indicate that each carbon chain of these
compounds contained 1 -CH2- group more than those
of, respectively, cysteine and cystine (already well known by that
time). Since then, homocysteine and homocystine have been the
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