| RRC ID |
38669
|
| 著者 |
Suzuki H, Yamada J, Watanabe T, Suga T.
|
| タイトル |
A specific method for determination of peroxisomal beta-oxidation activity in cultured human skin fibroblasts using a specific substrate, C9: a possible application for screening of peroxisomal disorders.
|
| ジャーナル |
Clin Chim Acta
|
| Abstract |
We developed a specific method for direct determination of peroxisomal beta-oxidation activity in cultured human skin fibroblasts. When control fibroblasts were incubated with N-(alpha-methylbenzyl)azelaamic acid (C9), a specific peroxisomal substrate, C5 and C7, the chain-shortened products, were detected with cell concentration and incubation time dependencies and no other products including C3 were detected. In glutaric aciduria type I and type II fibroblasts, the formation rates of C2 units liberated from C9 were almost similar to that in control cells. In contrast to these cell types, the fibroblasts from patient of Zellweger syndrome, in which peroxisomal beta-oxidation was impaired, showed no conversion of C9 to C5 and C7. The lack of the C2 units liberation in Zellweger fibroblasts was not due to an impairment of mitochondrial beta-oxidation and/or activation of C9 to C9-CoA derivative for subsequent beta-oxidation reaction, but rather, appeared to be due to the specific defect of peroxisomal beta-oxidation system. These results indicate that C9 is a useful substrate for the estimation of peroxisomal beta-oxidation activity in cultured human skin fibroblasts.
|
| 巻・号 |
207(1-2)
|
| ページ |
19-29
|
| 公開日 |
1992-4-30
|
| DOI |
10.1016/0009-8981(92)90147-i
|
| PII |
0009-8981(92)90147-I
|
| PMID |
1591864
|
| MeSH |
Cells, Cultured
Fatty Acids / metabolism*
Fibroblasts / metabolism*
Humans
Microbodies / enzymology
Microbodies / metabolism*
Zellweger Syndrome / metabolism
|
| IF |
2.615
|
| 引用数 |
0
|
|
WOS 分野
|
MEDICAL LABORATORY TECHNOLOGY
|
| リソース情報 |
| ヒト・動物細胞 |
human skin tibroblasts |
