Skip Navigation

This Article
Right arrow Full Text (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Email this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My Personal Archive
Right arrow Download to citation manager
Right arrow Request Permissions
Google Scholar
Right arrow Articles by TSUJI, S.
Right arrow Articles by YAMAKAWA, T.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by TSUJI, S.
Right arrow Articles by YAMAKAWA, T.
Social Bookmarking
Add to CiteULike   Add to Connotea   Add to Del.icio.us  
What's this?

J. Biochem, 1984, Vol. 96, No. 4 1241-1247
© 1984 Japanese Biochemical Society

research-article

Fatty Acid Elongation Activity in Fibroblasts from Patients with Adrenoleukodystrophy (ALD)1

Shoji TSUJI*,2, Tsukasa OHNO*,3, Tadashi MIYATAKE**, Akemi SUZUKI*,4 and Tamio YAMAKAWA*

*Metabolism Section, The Tokyo Metropolitan Institute of Medical Science, Honkomagome 3-chome Bunkyo-ku, Tokyo 113
**Department of Neurology, Brain Research Institute, Niigata University Asahimachi-dori 1, Niigata, Niigata 951

4To whom all correspondence should be addressed.

The activities of microsomal fatty acidelongation and cytoplasmic de novo fatty acid synthesis were measured in human cultured skin fibroblasts. Both activities in fibroblasts from normal controls and patients with adrenoleukodystrophy (ALD) were compared and slight but a significant increase of elongation activities in ALD fibroblasts was demonstrated. On the other hand, there were no significant differences in the fatty acid synthetase activities. In this study, we measured microsomal fatty acid elongation activities in the presence of N-ethylmaleimide, which completely inhibited the activity of contaminating fatty acid synthetase and also the degradation of fatty acids, and made accurate determination of the elongation activities possible.

1This work was supported in part by Grants-in-Aid for Scientific Research from the Ministry of Education, Science and Culture of Japan.

2Clinical Investigation and Therapeutics Section, NIH, Bethesda, ML 20205, U.S.A.

3Department of Neurology, Brain Research Institute, Niigata University, Niigata


Add to CiteULike CiteULike   Add to Connotea Connotea   Add to Del.icio.us Del.icio.us    What's this?


This article has been cited by other articles:


Home page
 Arch NeurolHome page
R. Ferri and P. F. Chance
Lorenzo's Oil: Advances in the Treatment of Neurometabolic Disorders
Arch Neurol, July 1, 2005; 62(7): 1045 - 1046.
[Full Text] [PDF]

Home page
 Arch NeurolHome page
H. Takano, R. Koike, O. Onodera, R. Sasaki, and S. Tsuji
Mutational Analysis and Genotype-Phenotype Correlation of 29 Unrelated Japanese Patients With X-linked Adrenoleukodystrophy
Arch Neurol, March 1, 1999; 56(3): 295 - 300.
[Abstract] [Full Text] [PDF]

Home page
 J. Lipid Res.Home page
K. Pahan, M. Khan, and I. Singh
Therapy for X-adrenoleukodystrophy: normalization of very long chain fatty acids and inhibition of induction of cytokines by cAMP
J. Lipid Res., May 1, 1998; 39(5): 1091 - 1100.
[Abstract] [Full Text]


Disclaimer: Please note that abstracts for content published before 1996 were created through digital scanning and may therefore not exactly replicate the text of the original print issues. All efforts have been made to ensure accuracy, but the Publisher will not be held responsible for any remaining inaccuracies. If you require any further clarification, please contact our Customer Services Department.