Peroxisomal Heat Generation and Possible Relationship With Peroxisomal Disorders

Anna Petroni

doi:doi:10.11648/j.ab.20130101.12

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Peroxisomal Heat Generation and Possible Relationship With Peroxisomal Disorders

Anna Petroni

Published in Advances in Biochemistry (Volume 1, Issue 1)

Published: 2 April 2013

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Abstract

Peroxisomal disorders encompass a variety of pathologies with different clinical manifestations. X-linked adrenoleukodystrophy, an inherited neurodegenerative pathology, is characterized by mutation of a peroxisomal transport protein involved in the catabolism of very long-chain fatty acids (VLCFAs). These particular fatty acids abnormally accu-mulate in plasma and in all tissues due to the enhancement of fatty acid elongation and the impairment of fatty acid catabolism which takes place initially in the peroxisomes and afterward in the mitochondria. VLCFA accumulation cannot explain the molecular mechanisms underlying clinical manifestations in patients. Peroxisomal pathways include a product that is considered lost, but that could have a role in peroxisomal disorders: the heat produced during VLCFA catabolism. VLCFA accumulation is due to peroxisomal beta-oxidation impairment and to the enhancement of fatty acid elongation. The heat produced during peroxisomal metabolism could be a crucial factor related to the molecular mechanisms altered in X-linked adrenoleukodystrophy. VLCFA accumulation could be strongly related to the impairment of heat production, which is lost in X-linked adrenoleukodystrophy, a sort of energy necessary for the cell metabolism, which could contribute to the secondary clinical manifestations of peroxisomal disorders. Heat is not only produced during peroxisomal catabolism but also in countless metabolic pathways. It is a form of energy that could influence several parameters and could be involved in peroxisomal and metabolic disorders in general.

Published in	Advances in Biochemistry (Volume 1, Issue 1)
DOI	10.11648/j.ab.20130101.12
Page(s)	5-6
Creative Commons	This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.
Copyright	Copyright © The Author(s), 2013. Published by Science Publishing Group

Keywords

Keywords: Peroxisomal heat, VLCFA, Very Long-Chain Fatty Acid; X-ALD, X-linked Adrenoleukodystrophy

References

[1]	J. Mosser, A.M. Douar, C.O. Sarde, R. Feil, H. Moser, A.M. Poutska, J.L. Mandel, P. Aubourg P, "Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters," Nature 1993, 361:726-730.
[2]	H.W. Moser, J. Borel, "Dietary management of X-linked adrenoleukodystrophy," Annu Rev Nutr 1995, 15: 379–397.
[3]	H.W. Moser, "Adrenoleukodystrophy: phenotype, genetics, pathogenesis, and therapy," Brain 1997, 120: 1485–1508.
[4]	M. Khan, J. Singh, A.G. Gilg, T. Uto, I. Singh, "Very long-chain fatty acid accumulation causes lipotoxic response via 5-lipoxygenase in cerebral adrenoleukodystrophy," J Lipid Res 2010, 51: 1685–1695.
[5]	I. Singh, A. Pujol, "Pathomechanisms underlying X-adrenoleukodystrophy: a three-hit hypothesis," Brain Pathol 2010, 20:838-844.
[6]	I. Singh, A.B. Moser, S. Goldfischer, H.W. Moser, "Ligno-ceric acid is oxidized in the peroxisomes: implication for the Zelleweger cerebro-hepatorenal syndrome and adrenoleu-kodystrophy," PNAS 1984, 81: 4203-4207.
[7]	S. Tsuji, T. Ohno, T. Miyatake, A. Suzuki, T. Yamakawa, "Fatty acid elongation activity in fibroblasts from patients with a drenoleukodystrophy," J Biochem 1984, 96: 1241-1247.
[8]	H. Schulz, "Beta-oxidation in peroxisomes," In Biochemistry of Lipids, Lipoproteins and Membranes. Edited by Vance DE, Vance J. Amsterdam: Elsevier; 1996:91-93.
[9]	G. Ciasca, G. Campi, A. Battisti, G. Rea, M. Rodio, M. Papi, P. Pernot, A. Tenenbaum, A. Bianconi, "Continuous thermal collapse of the intrinsically disordered protein Tau is driven by its entropi flexible domain," Langmuir 2012, 28:13405-13410.
[10]	D.J. Hryb, "Peroxisomal respiration and energy conservation, possible relationship between heat production, ther-moosmosis and conformational changes," FEBS Lett 1981, 128:1-4.
[11]	H. Akanuma, Y. Kishimoto," Synthesis of ceramide and ce-rebroside containing both alpha-hydroxy and non hydroxy fatty acids from lignoceroyl-Coa by rat brain microsomes," J Biol Chem 1979, 254:1050-1056.
[12]	A. Hlousek-Radojcic, K.J. Evenson, J.G. Jaworski, D. Post-Beittenmiller, "Fatty acid elongation is indipendent of acyl-Coenzyme A synthetase activities in Leek and Brassica napus," Plant Phyisiol 1998, 116:251:258.
[13]	P.A. Watkins, J.M. Ellis, "Peroxisomal acyl-CoA synthetases," Biochim Biophys Acta 2012, 1822:1411-1420.
[14]	S. Kemp, R. Wanders, "Biochemical aspects of X-linked Adrenoleukodystrophy," Brain Pathol 2010, 20: 831-837.
[15]	X. Chen, D. Shen, B. Zhou, "Analysis of the temperature-sensitive mutation of Escherichia coli pantotenate kinase re-veals XbjN as a possible protein stabilizer," Biochem Bio-phys Res Commun 2006, 345: 834-842.

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APA Style

Anna Petroni. (2013). Peroxisomal Heat Generation and Possible Relationship With Peroxisomal Disorders. Advances in Biochemistry, 1(1), 5-6. https://doi.org/10.11648/j.ab.20130101.12

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ACS Style

Anna Petroni. Peroxisomal Heat Generation and Possible Relationship With Peroxisomal Disorders. Adv. Biochem. 2013, 1(1), 5-6. doi: 10.11648/j.ab.20130101.12

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AMA Style

Anna Petroni. Peroxisomal Heat Generation and Possible Relationship With Peroxisomal Disorders. Adv Biochem. 2013;1(1):5-6. doi: 10.11648/j.ab.20130101.12

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@article{10.11648/j.ab.20130101.12,
  author = {Anna Petroni},
  title = {Peroxisomal Heat Generation and Possible Relationship With Peroxisomal Disorders},
  journal = {Advances in Biochemistry},
  volume = {1},
  number = {1},
  pages = {5-6},
  doi = {10.11648/j.ab.20130101.12},
  url = {https://doi.org/10.11648/j.ab.20130101.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ab.20130101.12},
  abstract = {Peroxisomal disorders encompass a variety of pathologies with different clinical manifestations. X-linked adrenoleukodystrophy, an inherited neurodegenerative pathology, is characterized by mutation of a peroxisomal transport protein involved in the catabolism of very long-chain fatty acids (VLCFAs). These particular fatty acids abnormally accu-mulate in plasma and in all tissues due to the enhancement of fatty acid elongation and the impairment of fatty acid catabolism which takes place initially in the peroxisomes and afterward in the mitochondria. VLCFA accumulation cannot explain the molecular mechanisms underlying clinical manifestations in patients. Peroxisomal pathways include a product that is considered lost, but that could have a role in peroxisomal disorders: the heat produced during VLCFA catabolism. VLCFA accumulation is due to peroxisomal beta-oxidation impairment and to the enhancement of fatty acid elongation. The heat produced during peroxisomal metabolism could be a crucial factor related to the molecular mechanisms altered in X-linked adrenoleukodystrophy. VLCFA accumulation could be strongly related to the impairment of heat production, which is lost in X-linked adrenoleukodystrophy, a sort of energy necessary for the cell metabolism, which could contribute to the secondary clinical manifestations of peroxisomal disorders. Heat is not only produced during peroxisomal catabolism but also in countless metabolic pathways. It is a form of energy that could influence several parameters and could be involved in peroxisomal and metabolic disorders in general.},
 year = {2013}
}

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TY  - JOUR
T1  - Peroxisomal Heat Generation and Possible Relationship With Peroxisomal Disorders
AU  - Anna Petroni
Y1  - 2013/04/02
PY  - 2013
N1  - https://doi.org/10.11648/j.ab.20130101.12
DO  - 10.11648/j.ab.20130101.12
T2  - Advances in Biochemistry
JF  - Advances in Biochemistry
JO  - Advances in Biochemistry
SP  - 5
EP  - 6
PB  - Science Publishing Group
SN  - 2329-0862
UR  - https://doi.org/10.11648/j.ab.20130101.12
AB  - Peroxisomal disorders encompass a variety of pathologies with different clinical manifestations. X-linked adrenoleukodystrophy, an inherited neurodegenerative pathology, is characterized by mutation of a peroxisomal transport protein involved in the catabolism of very long-chain fatty acids (VLCFAs). These particular fatty acids abnormally accu-mulate in plasma and in all tissues due to the enhancement of fatty acid elongation and the impairment of fatty acid catabolism which takes place initially in the peroxisomes and afterward in the mitochondria. VLCFA accumulation cannot explain the molecular mechanisms underlying clinical manifestations in patients. Peroxisomal pathways include a product that is considered lost, but that could have a role in peroxisomal disorders: the heat produced during VLCFA catabolism. VLCFA accumulation is due to peroxisomal beta-oxidation impairment and to the enhancement of fatty acid elongation. The heat produced during peroxisomal metabolism could be a crucial factor related to the molecular mechanisms altered in X-linked adrenoleukodystrophy. VLCFA accumulation could be strongly related to the impairment of heat production, which is lost in X-linked adrenoleukodystrophy, a sort of energy necessary for the cell metabolism, which could contribute to the secondary clinical manifestations of peroxisomal disorders. Heat is not only produced during peroxisomal catabolism but also in countless metabolic pathways. It is a form of energy that could influence several parameters and could be involved in peroxisomal and metabolic disorders in general.
VL  - 1
IS  - 1
ER  -

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Author Information

Anna Petroni

Dept. of Pharmacological and Biomolecular Sciences, University of Milan, Milan, Italy

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Plain Text BibTeX RIS

APA Style

Anna Petroni. (2013). Peroxisomal Heat Generation and Possible Relationship With Peroxisomal Disorders. Advances in Biochemistry, 1(1), 5-6. https://doi.org/10.11648/j.ab.20130101.12

Copy | Download

ACS Style

Anna Petroni. Peroxisomal Heat Generation and Possible Relationship With Peroxisomal Disorders. Adv. Biochem. 2013, 1(1), 5-6. doi: 10.11648/j.ab.20130101.12

Copy | Download

AMA Style

Anna Petroni. Peroxisomal Heat Generation and Possible Relationship With Peroxisomal Disorders. Adv Biochem. 2013;1(1):5-6. doi: 10.11648/j.ab.20130101.12

Copy | Download

@article{10.11648/j.ab.20130101.12,
  author = {Anna Petroni},
  title = {Peroxisomal Heat Generation and Possible Relationship With Peroxisomal Disorders},
  journal = {Advances in Biochemistry},
  volume = {1},
  number = {1},
  pages = {5-6},
  doi = {10.11648/j.ab.20130101.12},
  url = {https://doi.org/10.11648/j.ab.20130101.12},
  eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ab.20130101.12},
  abstract = {Peroxisomal disorders encompass a variety of pathologies with different clinical manifestations. X-linked adrenoleukodystrophy, an inherited neurodegenerative pathology, is characterized by mutation of a peroxisomal transport protein involved in the catabolism of very long-chain fatty acids (VLCFAs). These particular fatty acids abnormally accu-mulate in plasma and in all tissues due to the enhancement of fatty acid elongation and the impairment of fatty acid catabolism which takes place initially in the peroxisomes and afterward in the mitochondria. VLCFA accumulation cannot explain the molecular mechanisms underlying clinical manifestations in patients. Peroxisomal pathways include a product that is considered lost, but that could have a role in peroxisomal disorders: the heat produced during VLCFA catabolism. VLCFA accumulation is due to peroxisomal beta-oxidation impairment and to the enhancement of fatty acid elongation. The heat produced during peroxisomal metabolism could be a crucial factor related to the molecular mechanisms altered in X-linked adrenoleukodystrophy. VLCFA accumulation could be strongly related to the impairment of heat production, which is lost in X-linked adrenoleukodystrophy, a sort of energy necessary for the cell metabolism, which could contribute to the secondary clinical manifestations of peroxisomal disorders. Heat is not only produced during peroxisomal catabolism but also in countless metabolic pathways. It is a form of energy that could influence several parameters and could be involved in peroxisomal and metabolic disorders in general.},
 year = {2013}
}

Copy | Download

TY  - JOUR
T1  - Peroxisomal Heat Generation and Possible Relationship With Peroxisomal Disorders
AU  - Anna Petroni
Y1  - 2013/04/02
PY  - 2013
N1  - https://doi.org/10.11648/j.ab.20130101.12
DO  - 10.11648/j.ab.20130101.12
T2  - Advances in Biochemistry
JF  - Advances in Biochemistry
JO  - Advances in Biochemistry
SP  - 5
EP  - 6
PB  - Science Publishing Group
SN  - 2329-0862
UR  - https://doi.org/10.11648/j.ab.20130101.12
AB  - Peroxisomal disorders encompass a variety of pathologies with different clinical manifestations. X-linked adrenoleukodystrophy, an inherited neurodegenerative pathology, is characterized by mutation of a peroxisomal transport protein involved in the catabolism of very long-chain fatty acids (VLCFAs). These particular fatty acids abnormally accu-mulate in plasma and in all tissues due to the enhancement of fatty acid elongation and the impairment of fatty acid catabolism which takes place initially in the peroxisomes and afterward in the mitochondria. VLCFA accumulation cannot explain the molecular mechanisms underlying clinical manifestations in patients. Peroxisomal pathways include a product that is considered lost, but that could have a role in peroxisomal disorders: the heat produced during VLCFA catabolism. VLCFA accumulation is due to peroxisomal beta-oxidation impairment and to the enhancement of fatty acid elongation. The heat produced during peroxisomal metabolism could be a crucial factor related to the molecular mechanisms altered in X-linked adrenoleukodystrophy. VLCFA accumulation could be strongly related to the impairment of heat production, which is lost in X-linked adrenoleukodystrophy, a sort of energy necessary for the cell metabolism, which could contribute to the secondary clinical manifestations of peroxisomal disorders. Heat is not only produced during peroxisomal catabolism but also in countless metabolic pathways. It is a form of energy that could influence several parameters and could be involved in peroxisomal and metabolic disorders in general.
VL  - 1
IS  - 1
ER  -

Copy | Download