N-Acetylaspartate

by Moffett, John R., Ph.D.; Tieman, Suzannah B., Ph.D.; Weinberger, Daniel R.; Coyle, Joseph T.

• ISBN13: 9780387301716
• ISBN10: 0387301712
• Format: Hardcover
• Copyright: 2006-04-12
• Publisher: Springer Nature

Summary

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.

Table of Contents

A Brief Overview of n-acetylaspartate and N-acetylaspartylglutamate	1	(6)
Joseph T. Coyle
Expression of N-acetylaspartate and N-acetylaspartylglutamate in the Nervous System	7	(20)
John R. Moffett
Aryan M. A. Namboodiri
N-acetylaspartate Metabolism in Neural Cells	27	(22)
Kishore K. Bhakoo
Timothy Craig
Daniel Pearce
Naa Synthesis and Functional Roles	49	(18)
Chikkathur N. Madhavarao
Aryan M. A. Namboodiri
Identity of The High-affinity Sodium/carbox Ylate Cotransporter NaC3 as The Naa Transporter	67	(10)
Vadivel Ganapathy
Takuya Fujita
Canvan Disease: Studies on The Knockout Mouse	77	(18)
Reuben Matalon
Kimberlee Michals-Matalon
Sankar Surendran
Stephen K. Tyring
Functions of N-acetylaspartate and N-acetylaspartylglutamate in Brain: Evidence of a Role in Maintenance of Higher Brain Integrative Activities of Information Processing and Cognition	95	(18)
Morris H. Baslow
David N. Guilfoyle
Control of Brain Volume During Hypoosmolality and Hyperosmolality	113	(18)
Joseph G. Verbalis
Physiological Role of N-acetylaspartate: Contribution to Myelinogenesis	131	(14)
Robert W. Ledeen
Jianfeng Wang
Gusheng Wu
Zi-Hua Lu
Goutam Chakraborty
Markus Meyenhofer
Stephen K. Tyring
Reuben Matalon
Defective Myelin Lipid Synthesis as a Pathogenic Mechanism of Canavan Disease	145	(20)
Aryan M. A. Namboodiri
John R. Moffett
Arun Peethambaran
Raji Mathew
Sreela Namboodiri
Asha Potti
Jeremy Hershfield
Batool Kirmani
David M. Jacobowitz
Chikkathur N. Madhavarao
Mutation Analysis of The Aspartoacylase Gene In Non-jewish Patients With Canavan Disease	165	(10)
Bai-Jin Zeng
Gregory M. Pastores
Paola Leone
Srinivasa Raghavan
Zhao-Hui Wang
Lucilene A. Ribeiro
Paola Torres
Elton Ong
Edwin H. Kolodny
Does Aspa Gene Mutation in Canavan Disease Alter Oligodendrocyte Development? a Tissue Culture Study of Aspa Ko Mice Brain	175	(8)
Shalini Kumar
Rasika Sowmyalakshmi
Sarah L. Daniels
Ruth Chang
Sankar Surendran
Reuben Matalon
Jean de Vellis
Quantitation of Naa in The Brain by Magnetic Resonance Spectroscopy	183	(16)
Peter B. Barker
David Bonekamp
Gerard Riedy
Mari Smith
N-acetyl-l-aspartate in Multiple Sclerosis	199	(16)
Gerson A. Criste
Bruce D. Trapp
Naa and Higher Cognitive Function in Humans	215	(12)
Ronald A. Yeo
William M. Brooks
Rex E. Jung
In Vivo Nmr Measures of Naa and The Neurobiology of Schizophrenia	227	(14)
Stefano Marenco
Alessandro Bertolino
Daniel R. Weinberger
N-acetylaspartate as a Marker of Neuronal Injury in Neurodegenerative Disease	241	(22)
Norbert Schuff
Dieter J. Meyerhoff
Susanne Mueller
Linda Chao
Diana Truran Sacrey
Kenneth Laxer
Michael W. Weiner
Regulation of Naa-synthesis in The Human Brain In Vivo: Canavan's Disease, Alzheimer's Disease and Schizophrenia	263	(12)
Kent Harris
Alexander Lin
Pratip Bhattacharya
Thao Tran
Willis Wong
Brian Ross
Magnetic Resonance Spectroscopy For Monitoring Neuronal Integrity in Amyotrophic Lateral Sclerosis	275	(8)
Sanjay Kalra
Douglas L. Arnold
Hypoacetylaspartia: Clinical and Biochemical Follow-up of a Patient	283	(6)
Alessandro P. Burlina
B. Schmitt
U. Engelke
Ron A. Wevers
Alberto B. Burlina
Eugen Boltshauser
Cellular Localization of Naag	289	(14)
Suzannah Bliss Tieman
Synthesis of N-acetylaspartylglutamate (naag) and N-acetylaspartate (naa) in Axons and Glia of The Crayfish Medial Giant Nerve Fiber	303	(14)
Edward M. Lieberman
Mohit Achreja
Albert K. Urazaev
Naag as a Neurotransmitter	317	(10)
Barbara Wroblewska
Glutamate Carboxypeptidase II (naaladase) Inhibition as a Novel Therapeutic Strategy	327	(12)
Ajit G. Thomas
Krystyna M. Wozniak
Takashi Tsukamoto
David Calvin
Ying Wu
Camilo Rojas
James Vornov
Barbara S. Slusher
N-acetylaspartylglutamate (naag) in Spinal Cord Injury and Disease	339	(14)
James L. Meyerhoff
Debra L. Yourick
Barbara S. Slusher
Joseph B. Long
N-acetylaspartylglutamate (naag) in Pelizaeus-merzbacher Disease	353	(8)
Alessandro P. Burlina
Vanni Ferrari
Alberto B. Burlina
Mario Ermani
Odile Boespflug-Tanguy
Enrico Bertini
Concluding Remarks	361	(4)
Index	365