A comparative study of straight chain and branched chain fatty acid oxidation in skin fibroblasts from patients with peroxisomal disorders.
The beta-oxidation of stearic acid and of alpha- and gamma-methyl isoprenoid-derived fatty acids (pristanic and tetramethylheptadecanoic acids, respectively) was investigated in normal skin fibroblasts and in fibroblasts from patients with inherited defects in peroxisomal biogenesis. Stearic acid be...
Main Authors: | , , , |
---|---|
Format: | Article |
Language: | English |
Published: |
Elsevier
1990-02-01
|
Series: | Journal of Lipid Research |
Online Access: | http://www.sciencedirect.com/science/article/pii/S0022227520432079 |
id |
doaj-1982c5c423e140f997a190601fdd0bc7 |
---|---|
record_format |
Article |
spelling |
doaj-1982c5c423e140f997a190601fdd0bc72021-04-25T04:23:06ZengElsevierJournal of Lipid Research0022-22751990-02-01312217225A comparative study of straight chain and branched chain fatty acid oxidation in skin fibroblasts from patients with peroxisomal disorders.H Singh0S Usher1D Johnson2A Poulos3Department of Chemical Pathology, Adelaide Children's Hospital, Australia.Department of Chemical Pathology, Adelaide Children's Hospital, Australia.Department of Chemical Pathology, Adelaide Children's Hospital, Australia.Department of Chemical Pathology, Adelaide Children's Hospital, Australia.The beta-oxidation of stearic acid and of alpha- and gamma-methyl isoprenoid-derived fatty acids (pristanic and tetramethylheptadecanoic acids, respectively) was investigated in normal skin fibroblasts and in fibroblasts from patients with inherited defects in peroxisomal biogenesis. Stearic acid beta-oxidation by normal fibroblast homogenates was several-fold greater compared to the oxidation of the two branched chain fatty acids. The effect of phosphatidylcholine, alpha-cyclodextrin, and bovine serum albumin on the three activities suggests that different enzymes are involved in the beta-oxidation of straight chain and branched chain fatty acids. Homogenates of fibroblasts from patients with a deficiency in peroxisomes (Zellweger syndrome and infantile Refsum's disease) showed a normal ability to beta-oxidize stearic acid, but the oxidation of pristanic and tetramethylheptadecanoic acid was decreased. Concomitantly, 14CO2 production from the branched chain fatty acids by Zellweger fibroblasts in culture (but not from stearic acid) was greatly diminished. The Zellweger fibroblasts also showed a marked reduction in the amount of water-soluble metabolites from the radiolabeled branched chain fatty acids that are released into the culture medium. The data presented indicate that the oxidation of alpha- and gamma-methyl isoprenoid-derived fatty acids takes place largely in peroxisomes in human skin fibroblasts.http://www.sciencedirect.com/science/article/pii/S0022227520432079 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
H Singh S Usher D Johnson A Poulos |
spellingShingle |
H Singh S Usher D Johnson A Poulos A comparative study of straight chain and branched chain fatty acid oxidation in skin fibroblasts from patients with peroxisomal disorders. Journal of Lipid Research |
author_facet |
H Singh S Usher D Johnson A Poulos |
author_sort |
H Singh |
title |
A comparative study of straight chain and branched chain fatty acid oxidation in skin fibroblasts from patients with peroxisomal disorders. |
title_short |
A comparative study of straight chain and branched chain fatty acid oxidation in skin fibroblasts from patients with peroxisomal disorders. |
title_full |
A comparative study of straight chain and branched chain fatty acid oxidation in skin fibroblasts from patients with peroxisomal disorders. |
title_fullStr |
A comparative study of straight chain and branched chain fatty acid oxidation in skin fibroblasts from patients with peroxisomal disorders. |
title_full_unstemmed |
A comparative study of straight chain and branched chain fatty acid oxidation in skin fibroblasts from patients with peroxisomal disorders. |
title_sort |
comparative study of straight chain and branched chain fatty acid oxidation in skin fibroblasts from patients with peroxisomal disorders. |
publisher |
Elsevier |
series |
Journal of Lipid Research |
issn |
0022-2275 |
publishDate |
1990-02-01 |
description |
The beta-oxidation of stearic acid and of alpha- and gamma-methyl isoprenoid-derived fatty acids (pristanic and tetramethylheptadecanoic acids, respectively) was investigated in normal skin fibroblasts and in fibroblasts from patients with inherited defects in peroxisomal biogenesis. Stearic acid beta-oxidation by normal fibroblast homogenates was several-fold greater compared to the oxidation of the two branched chain fatty acids. The effect of phosphatidylcholine, alpha-cyclodextrin, and bovine serum albumin on the three activities suggests that different enzymes are involved in the beta-oxidation of straight chain and branched chain fatty acids. Homogenates of fibroblasts from patients with a deficiency in peroxisomes (Zellweger syndrome and infantile Refsum's disease) showed a normal ability to beta-oxidize stearic acid, but the oxidation of pristanic and tetramethylheptadecanoic acid was decreased. Concomitantly, 14CO2 production from the branched chain fatty acids by Zellweger fibroblasts in culture (but not from stearic acid) was greatly diminished. The Zellweger fibroblasts also showed a marked reduction in the amount of water-soluble metabolites from the radiolabeled branched chain fatty acids that are released into the culture medium. The data presented indicate that the oxidation of alpha- and gamma-methyl isoprenoid-derived fatty acids takes place largely in peroxisomes in human skin fibroblasts. |
url |
http://www.sciencedirect.com/science/article/pii/S0022227520432079 |
work_keys_str_mv |
AT hsingh acomparativestudyofstraightchainandbranchedchainfattyacidoxidationinskinfibroblastsfrompatientswithperoxisomaldisorders AT susher acomparativestudyofstraightchainandbranchedchainfattyacidoxidationinskinfibroblastsfrompatientswithperoxisomaldisorders AT djohnson acomparativestudyofstraightchainandbranchedchainfattyacidoxidationinskinfibroblastsfrompatientswithperoxisomaldisorders AT apoulos acomparativestudyofstraightchainandbranchedchainfattyacidoxidationinskinfibroblastsfrompatientswithperoxisomaldisorders AT hsingh comparativestudyofstraightchainandbranchedchainfattyacidoxidationinskinfibroblastsfrompatientswithperoxisomaldisorders AT susher comparativestudyofstraightchainandbranchedchainfattyacidoxidationinskinfibroblastsfrompatientswithperoxisomaldisorders AT djohnson comparativestudyofstraightchainandbranchedchainfattyacidoxidationinskinfibroblastsfrompatientswithperoxisomaldisorders AT apoulos comparativestudyofstraightchainandbranchedchainfattyacidoxidationinskinfibroblastsfrompatientswithperoxisomaldisorders |
_version_ |
1721510216142422016 |