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Functional redundancy of mitochondrial enoyl-CoA isomerases in the oxidation of unsaturated fatty acids. / van Weeghel, Michel; te Brinke, Heleen; van Lenthe, Henk et al.

In: FASEB journal, Vol. 26, No. 10, 2012, p. 4316-4326.

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

Harvard

van Weeghel, M, te Brinke, H, van Lenthe, H, Kulik, W, Minkler, PE, Stoll, MSK, Sass, JO, Janssen, U, Stoffel, W, Schwab, KO, Wanders, RJA, Hoppel, CL & Houten, SM 2012, 'Functional redundancy of mitochondrial enoyl-CoA isomerases in the oxidation of unsaturated fatty acids', FASEB journal, vol. 26, no. 10, pp. 4316-4326. https://doi.org/10.1096/fj.12-206326

APA

van Weeghel, M., te Brinke, H., van Lenthe, H., Kulik, W., Minkler, P. E., Stoll, M. S. K., Sass, J. O., Janssen, U., Stoffel, W., Schwab, K. O., Wanders, R. J. A., Hoppel, C. L., & Houten, S. M. (2012). Functional redundancy of mitochondrial enoyl-CoA isomerases in the oxidation of unsaturated fatty acids. FASEB journal, 26(10), 4316-4326. https://doi.org/10.1096/fj.12-206326

Vancouver

van Weeghel M, te Brinke H, van Lenthe H, Kulik W, Minkler PE, Stoll MSK et al. Functional redundancy of mitochondrial enoyl-CoA isomerases in the oxidation of unsaturated fatty acids. FASEB journal. 2012;26(10):4316-4326. doi: 10.1096/fj.12-206326

Author

van Weeghel, Michel ; te Brinke, Heleen ; van Lenthe, Henk et al. / Functional redundancy of mitochondrial enoyl-CoA isomerases in the oxidation of unsaturated fatty acids. In: FASEB journal. 2012 ; Vol. 26, No. 10. pp. 4316-4326.

BibTeX

@article{52a7530463834a52b85f002eadf06aef,
title = "Functional redundancy of mitochondrial enoyl-CoA isomerases in the oxidation of unsaturated fatty acids",
abstract = "Mitochondrial enoyl-CoA isomerase (ECI1) is an auxiliary enzyme involved in unsaturated fatty acid oxidation. In contrast to most of the other enzymes involved in fatty acid oxidation, a deficiency of ECI1 has yet to be identified in humans. We used wild-type (WT) and Eci1-deficient knockout (KO) mice to explore a potential presentation of human ECI1 deficiency. Upon food withdrawal, Eci1-deficient mice displayed normal blood beta-hydroxybutyrate levels (WT 1.09 mM vs. KO 1.10 mM), a trend to lower blood glucose levels (WT 4.58 mM vs. KO 3.87 mM, P=0.09) and elevated blood levels of unsaturated acylcarnitines, in particular C12:1 acylcarnitine (WT 0.03 mu M vs. KO 0.09 mu M, P <0.01). Feeding an olive oil-rich diet induced an even greater increase in C12: 1 acylcarnitine levels (WT 0.01 mu M vs. KO 0.04 mu M, P <0.01). Overall, the phenotypic presentation of Eci1-deficient mice is mild, possibly caused by the presence of a second enoyl-CoA isomerase (Eci2) in mitochondria. Knockdown of Eci2 in Eci1-deficient fibroblasts caused a more pronounced accumulation of C12: 1 acylcarnitine on incubation with unsaturated fatty acids (12-fold, P <0.05). We conclude that Eci2 compensates for Eci1 deficiency explaining the mild phenotype of Eci1-deficient mice. Hypoglycemia and accumulation of C12: 1 acylcarnitine might be diagnostic markers to identify ECI1 deficiency in humans.-van Weeghel, M., te Brinke, H., van Lenthe, H., Kulik, W., Minkler, P. E., Stoll, M. S. K., Sass, J. O., Janssen, U., Stoffel, W., Schwab, O. K., Wanders, R. J. A., Hoppel, C. L., Houten, S. M. Functional redundancy of mitochondrial enoyl-CoA isomerases in the oxidation of unsaturated fatty acids. FASEB J. 26, 4316-4326 (2012). www.fasebj.org",
author = "{van Weeghel}, Michel and {te Brinke}, Heleen and {van Lenthe}, Henk and Wim Kulik and Minkler, {Paul E.} and Stoll, {Maria S. K.} and Sass, {J{\"o}rn Oliver} and Uwe Janssen and Wilhelm Stoffel and Schwab, {K. Otfried} and Wanders, {Ronald J. A.} and Hoppel, {Charles L.} and Houten, {Sander M.}",
year = "2012",
doi = "10.1096/fj.12-206326",
language = "English",
volume = "26",
pages = "4316--4326",
journal = "FASEB journal",
issn = "0892-6638",
publisher = "FASEB",
number = "10",

}

RIS

TY - JOUR

T1 - Functional redundancy of mitochondrial enoyl-CoA isomerases in the oxidation of unsaturated fatty acids

AU - van Weeghel, Michel

AU - te Brinke, Heleen

AU - van Lenthe, Henk

AU - Kulik, Wim

AU - Minkler, Paul E.

AU - Stoll, Maria S. K.

AU - Sass, Jörn Oliver

AU - Janssen, Uwe

AU - Stoffel, Wilhelm

AU - Schwab, K. Otfried

AU - Wanders, Ronald J. A.

AU - Hoppel, Charles L.

AU - Houten, Sander M.

PY - 2012

Y1 - 2012

N2 - Mitochondrial enoyl-CoA isomerase (ECI1) is an auxiliary enzyme involved in unsaturated fatty acid oxidation. In contrast to most of the other enzymes involved in fatty acid oxidation, a deficiency of ECI1 has yet to be identified in humans. We used wild-type (WT) and Eci1-deficient knockout (KO) mice to explore a potential presentation of human ECI1 deficiency. Upon food withdrawal, Eci1-deficient mice displayed normal blood beta-hydroxybutyrate levels (WT 1.09 mM vs. KO 1.10 mM), a trend to lower blood glucose levels (WT 4.58 mM vs. KO 3.87 mM, P=0.09) and elevated blood levels of unsaturated acylcarnitines, in particular C12:1 acylcarnitine (WT 0.03 mu M vs. KO 0.09 mu M, P <0.01). Feeding an olive oil-rich diet induced an even greater increase in C12: 1 acylcarnitine levels (WT 0.01 mu M vs. KO 0.04 mu M, P <0.01). Overall, the phenotypic presentation of Eci1-deficient mice is mild, possibly caused by the presence of a second enoyl-CoA isomerase (Eci2) in mitochondria. Knockdown of Eci2 in Eci1-deficient fibroblasts caused a more pronounced accumulation of C12: 1 acylcarnitine on incubation with unsaturated fatty acids (12-fold, P <0.05). We conclude that Eci2 compensates for Eci1 deficiency explaining the mild phenotype of Eci1-deficient mice. Hypoglycemia and accumulation of C12: 1 acylcarnitine might be diagnostic markers to identify ECI1 deficiency in humans.-van Weeghel, M., te Brinke, H., van Lenthe, H., Kulik, W., Minkler, P. E., Stoll, M. S. K., Sass, J. O., Janssen, U., Stoffel, W., Schwab, O. K., Wanders, R. J. A., Hoppel, C. L., Houten, S. M. Functional redundancy of mitochondrial enoyl-CoA isomerases in the oxidation of unsaturated fatty acids. FASEB J. 26, 4316-4326 (2012). www.fasebj.org

AB - Mitochondrial enoyl-CoA isomerase (ECI1) is an auxiliary enzyme involved in unsaturated fatty acid oxidation. In contrast to most of the other enzymes involved in fatty acid oxidation, a deficiency of ECI1 has yet to be identified in humans. We used wild-type (WT) and Eci1-deficient knockout (KO) mice to explore a potential presentation of human ECI1 deficiency. Upon food withdrawal, Eci1-deficient mice displayed normal blood beta-hydroxybutyrate levels (WT 1.09 mM vs. KO 1.10 mM), a trend to lower blood glucose levels (WT 4.58 mM vs. KO 3.87 mM, P=0.09) and elevated blood levels of unsaturated acylcarnitines, in particular C12:1 acylcarnitine (WT 0.03 mu M vs. KO 0.09 mu M, P <0.01). Feeding an olive oil-rich diet induced an even greater increase in C12: 1 acylcarnitine levels (WT 0.01 mu M vs. KO 0.04 mu M, P <0.01). Overall, the phenotypic presentation of Eci1-deficient mice is mild, possibly caused by the presence of a second enoyl-CoA isomerase (Eci2) in mitochondria. Knockdown of Eci2 in Eci1-deficient fibroblasts caused a more pronounced accumulation of C12: 1 acylcarnitine on incubation with unsaturated fatty acids (12-fold, P <0.05). We conclude that Eci2 compensates for Eci1 deficiency explaining the mild phenotype of Eci1-deficient mice. Hypoglycemia and accumulation of C12: 1 acylcarnitine might be diagnostic markers to identify ECI1 deficiency in humans.-van Weeghel, M., te Brinke, H., van Lenthe, H., Kulik, W., Minkler, P. E., Stoll, M. S. K., Sass, J. O., Janssen, U., Stoffel, W., Schwab, O. K., Wanders, R. J. A., Hoppel, C. L., Houten, S. M. Functional redundancy of mitochondrial enoyl-CoA isomerases in the oxidation of unsaturated fatty acids. FASEB J. 26, 4316-4326 (2012). www.fasebj.org

U2 - 10.1096/fj.12-206326

DO - 10.1096/fj.12-206326

M3 - Article

C2 - 22782973

VL - 26

SP - 4316

EP - 4326

JO - FASEB journal

JF - FASEB journal

SN - 0892-6638

IS - 10

ER -

ID: 1703312