A Unique Neuronal Molecule in the Central Nervous System

  • John R. Moffett
  • Suzannah B. Tieman
  • Daniel R. Weinberger
  • Joseph T. Coyle
  • Aryan M. A. Namboodiri
Conference proceedings

Part of the Advances in Experimental Medicine and Biology book series (volume 576)

Table of contents

  1. Front Matter
    Pages i-xvii
  2. Kishore K. Bhakoo, Timothy Craig, Daniel Pearce
    Pages 27-47
  3. Chikkathur N. Madhavarao, Aryan M. A. Namboodiri
    Pages 49-66
  4. Reuben Matalon, Kimberlee Michals-Matalon, Sankar Surendran, Stephen K. Tyring
    Pages 77-93
  5. Morris H. Baslow, David N. Guilfoyle
    Pages 95-112
  6. Robert W. Ledeen, Jianfeng Wang, Gusheng Wu, Zi-Hua Lu, Goutam Chakraborty, Markus Meyenhofer et al.
    Pages 131-143
  7. Aryan M. A. Namboodiri, John R. Moffett, Peethambaran Arun, Raji Mathew, Sreela Namboodiri, Asha Potti et al.
    Pages 145-163
  8. Bai-Jin Zeng, Gregory M. Pastores, Paola Leone, Srinivasa Raghavan, Zhao-Hui Wang, Lucilene A. Ribeiro et al.
    Pages 165-173
  9. Shalini Kumar, Rasika Sowmyalakshmi, Sarah L. Daniels, Ruth Chang, Sankar Surendran, Reuben Matalon et al.
    Pages 175-182
  10. Peter B. Barker, David Bonekamp, Gerard Riedy, Mari Smith
    Pages 183-197
  11. Gerson A. Criste, Bruce D. Trapp
    Pages 199-214
  12. Ronald A. Yeo, William M. Brooks, Rex E. Jung
    Pages 215-226
  13. Stefano Marenco, Alessandro Bertolino, Daniel R. Weinberger
    Pages 227-240
  14. Norbert Schuff, Dieter J. Meyerhoff, Susanne Mueller, Linda Chao, Diana Truran Sacrey, Kenneth Laxer et al.
    Pages 241-262
  15. Kent Harris, Alexander Lin, Pratip Bhattacharya, Thao Tran, Willis Wong, Brian Ross
    Pages 263-273
  16. Alessandro P. Burlina, B. Schmitt, U. Engelke, Ron A. Wevers, Alberto B. Burlina, Eugen Boltshauser
    Pages 283-287
  17. Suzannah Bliss Tieman
    Pages 289-301
  18. Barbara Wroblewska
    Pages 317-325
  19. Ajit G. Thomas, Krystyna M. Wozniak, Takashi Tsukamoto, David Calvin, Ying Wu, Camilo Rojas et al.
    Pages 327-337
  20. James L. Meyerhoff, Debra L. Yourick, Barbara S. Slusher, Joseph B. Long
    Pages 339-351
  21. Alessandro P. Burlina, Vanni Ferrari, Alberto B. Burlina, Mario Ermani, Odile Boespflug-Tanguy, Enrico Bertini et al.
    Pages 353-359
  22. Pages 361-363
  23. Back Matter
    Pages 365-375

About these proceedings


N-acetylaspartate, or NAA, is the acetylated form of the amino acid aspartate, and it is present exclusively in the nervous system. Indeed, NAA is one of the most highly concentrated chemicals found in the brain of humans and animals, and yet the functions served by this brain-specific metabolite remain elusive, and controversial. Despite the uncertainties surrounding the functions of NAA in the development and operation of the nervous system, this molecule has attracted the attention of researchers and clinicians for two distinct reasons.

First, the acetyl proton on NAA gives off a very prominent signal in water-suppressed, proton magnetic resonance spectroscopy (MRS), which permits clinicians to monitor levels of NAA in the brains of patients in a non-invasive manner. Because NAA is found primarily in neurons, and because the levels in the brain have been found to change rapidly after injury, or slowly during neurodegenerative diseases, MRS has become a preferred method of analyzing nerve cell dysfunction and death without surgical intervention.

The second reason that NAA has attracted attention in recent years is that a congenital genetic disorder of NAA metabolism has been found to be the cause of the neurodegenerative disorder known as Canavan’s disease. Canavan’s disease is an inherited leukodystrophy that involves myelination pathologies of cortical white matter, leading to death within 10 years of birth. The genetic mutation results in a defective enzyme that de-acetylates NAA in the brain, resulting in a significant rise in NAA levels in the brain and urine. This enzyme, known as aspartoacylase (ASPA), appears to be involved in the process of myelination, such that a defective enzyme results in a disruption of the myelination of nerve fibers during development.

The purpose of this symposium is to bring together investigators from around the world who are interested in the study of NAA, and the roles it plays in neuronal development and functioning. It is hoped that bringing researchers and clinicians together in such a forum will facilitate rapid progress in this emerging field, and will help lead to discoveries that can alleviate the suffering caused by a deadly, inheritable infantile disease.


Alzheimer ELIZA Nervous System attention neurobiology neurons

Editors and affiliations

  • John R. Moffett
    • 1
  • Suzannah B. Tieman
    • 2
  • Daniel R. Weinberger
    • 3
  • Joseph T. Coyle
    • 4
  • Aryan M. A. Namboodiri
    • 1
  1. 1.Uniformed Services University of the Health SciencesBethesdaUSA
  2. 2.University of AlbanyAlbanyUSA
  3. 3.National Institute of Mental HealthBethesdaUSA
  4. 4.McLean HospitalBelmontUSA

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