N-acetylaspartylglutamate in Canavan disease: An adverse effector?

A. P. Burlina; V. Ferrari; P. Divry; W. Gradowska; C. Jakobs; M. J. Bennett; A. C. Sewell; C. Dionisi-Vici; A. B. Burlina

doi:10.1007/s004310051102

N-acetylaspartylglutamate in Canavan disease: An adverse effector?

A. P. Burlina, V. Ferrari, P. Divry, W. Gradowska, C. Jakobs, M. J. Bennett, A. C. Sewell, C. Dionisi-Vici, A. B. Burlina

Ospedale Pediatrico Bambino Gesu

Research output: Contribution to journal › Article › peer-review

Abstract

We measured N-acetylaspartate and its precursor/product N- acetylaspartylglutamate (NAAG) in urine of patients with Canavan disease using capillary zone electrophoresis. Abnormal levels of NAAG were found in 32 of 43 patients examined. Elevated NAAG was also present in the CSF of one patient. Given that NAAG may interfere with N-methyl-D-aspartate receptor function, the occurrence of high levels of NAAG in patients' urine conceivably represents a participating factor in the pathogenesis of Canavan disease. Conclusion The biochemical role of N-acetylaspartylglutamate and its relationship to glutamatergic function may be relevant to the pathogenesis of Canavan disease.

Original language	English
Pages (from-to)	406-409
Number of pages	4
Journal	European Journal of Pediatrics
Volume	158
Issue number	5
DOIs	https://doi.org/10.1007/s004310051102
Publication status	Published - 1999

Keywords

Canavan disease
Capillary zone electrophoresis
N-acetylaspartate
N-acetylaspartylglutamate
Urine

ASJC Scopus subject areas

Pediatrics, Perinatology, and Child Health

Access to Document

10.1007/s004310051102

Cite this

@article{d49c94db93144e98ba41e5c90c7059a6,

title = "N-acetylaspartylglutamate in Canavan disease: An adverse effector?",

abstract = "We measured N-acetylaspartate and its precursor/product N- acetylaspartylglutamate (NAAG) in urine of patients with Canavan disease using capillary zone electrophoresis. Abnormal levels of NAAG were found in 32 of 43 patients examined. Elevated NAAG was also present in the CSF of one patient. Given that NAAG may interfere with N-methyl-D-aspartate receptor function, the occurrence of high levels of NAAG in patients' urine conceivably represents a participating factor in the pathogenesis of Canavan disease. Conclusion The biochemical role of N-acetylaspartylglutamate and its relationship to glutamatergic function may be relevant to the pathogenesis of Canavan disease.",

keywords = "Canavan disease, Capillary zone electrophoresis, N-acetylaspartate, N-acetylaspartylglutamate, Urine",

author = "Burlina, {A. P.} and V. Ferrari and P. Divry and W. Gradowska and C. Jakobs and Bennett, {M. J.} and Sewell, {A. C.} and C. Dionisi-Vici and Burlina, {A. B.}",

year = "1999",

doi = "10.1007/s004310051102",

language = "English",

volume = "158",

pages = "406--409",

journal = "European Journal of Pediatrics",

issn = "0340-6199",

publisher = "Springer Berlin Heidelberg",

number = "5",

}

TY - JOUR

T1 - N-acetylaspartylglutamate in Canavan disease

T2 - An adverse effector?

AU - Burlina, A. P.

AU - Ferrari, V.

AU - Divry, P.

AU - Gradowska, W.

AU - Jakobs, C.

AU - Bennett, M. J.

AU - Sewell, A. C.

AU - Dionisi-Vici, C.

AU - Burlina, A. B.

PY - 1999

Y1 - 1999

N2 - We measured N-acetylaspartate and its precursor/product N- acetylaspartylglutamate (NAAG) in urine of patients with Canavan disease using capillary zone electrophoresis. Abnormal levels of NAAG were found in 32 of 43 patients examined. Elevated NAAG was also present in the CSF of one patient. Given that NAAG may interfere with N-methyl-D-aspartate receptor function, the occurrence of high levels of NAAG in patients' urine conceivably represents a participating factor in the pathogenesis of Canavan disease. Conclusion The biochemical role of N-acetylaspartylglutamate and its relationship to glutamatergic function may be relevant to the pathogenesis of Canavan disease.

AB - We measured N-acetylaspartate and its precursor/product N- acetylaspartylglutamate (NAAG) in urine of patients with Canavan disease using capillary zone electrophoresis. Abnormal levels of NAAG were found in 32 of 43 patients examined. Elevated NAAG was also present in the CSF of one patient. Given that NAAG may interfere with N-methyl-D-aspartate receptor function, the occurrence of high levels of NAAG in patients' urine conceivably represents a participating factor in the pathogenesis of Canavan disease. Conclusion The biochemical role of N-acetylaspartylglutamate and its relationship to glutamatergic function may be relevant to the pathogenesis of Canavan disease.

KW - Canavan disease

KW - Capillary zone electrophoresis

KW - N-acetylaspartate

KW - N-acetylaspartylglutamate

KW - Urine

UR - http://www.scopus.com/inward/record.url?scp=0013361563&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0013361563&partnerID=8YFLogxK

U2 - 10.1007/s004310051102

DO - 10.1007/s004310051102

M3 - Article

C2 - 10333125

AN - SCOPUS:0013361563

VL - 158

SP - 406

EP - 409

JO - European Journal of Pediatrics

JF - European Journal of Pediatrics

SN - 0340-6199

IS - 5

ER -

N-acetylaspartylglutamate in Canavan disease: An adverse effector?

Abstract

Keywords

ASJC Scopus subject areas

Access to Document

Other files and links

Fingerprint

Cite this