Defective N-acetylaspartate catabolism reduces brain acetate levels and myelin lipid synthesis in Canavan's disease

Proceedings of the National Academy of Sciences of the United States of America

Volumn 102, Issue 14, 2005, Pages 5221-5226

  Madhavarao, Chikkathur N ^a   Arun, Peethambaran ^a   Moffett, John R ^a   Szucs, Sylvia ^b   Surendran, Sankar ^b   Matalon, Reuben ^b   Garbern, James ^c   Hristova, Diana ^c   Johnson, Anne ^d   Jiang, Wei ^e   Namboodiri, M A Aryan ^a  

^a UNIFORMED SERVICES UNIVERSITY OF THE HEALTH SCIENCES   (United States)

^b University of Texas Medical Branch School of Medicine   (United States)

^c WAYNE STATE UNIVERSITY SCHOOL OF MEDICINE   (United States)

^d ALBERT EINSTEIN COLLEGE OF MEDICINE   (United States)

^e HENRY FORD HOSPITAL   (United States)

Author keywords

Acetyl CoA; Aspartoacylase; Leukodystrophy; Oligodendrocytes

Indexed keywords

ACETIC ACID; ASPARTIC ACID; CEREBROSIDE; MYELIN; N ACETYLASPARTIC ACID; SULFATIDE;

AGED; ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; ASPA GENE; CANAVAN DISEASE; CASE REPORT; CATABOLISM; CONTROLLED STUDY; FEMALE; GENE; GENE FUNCTION; GENE MUTATION; GENETIC CODE; HUMAN; HUMAN CELL; HUMAN TISSUE; HYDROLYSIS; LIPID COMPOSITION; LIPOGENESIS; MALE; MOUSE; MYELINATION; NERVOUS SYSTEM DEVELOPMENT; NONHUMAN; PATHOGENESIS; PRESCHOOL CHILD; PRIORITY JOURNAL; WHITE MATTER;

ACETIC ACID; AMIDOHYDROLASES; ANIMALS; ASPARTIC ACID; BASE SEQUENCE; BRAIN; CANAVAN DISEASE; DNA; HUMANS; LIPIDS; MALE; MICE; MICE, KNOCKOUT; MODELS, NEUROLOGICAL; MYELIN SHEATH; RATS;

EID: 20144389547     PISSN: 00278424     EISSN: None     Source Type: Journal    
DOI: 10.1073/pnas.0409184102     Document Type: Article

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