Impaired mitochondrial oxidative phosphorylation in the peroxisomal disease X-linked adrenoleukodystrophy. (Q51055442)

27 March 2018

title

Impaired mitochondrial oxidative phosphorylation in the peroxisomal disease X-linked adrenoleukodystrophy. (English)

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27 March 2018

ATP-binding cassette, sub-family D (ALD), member 1

main subject

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GOA release 2020-03-11

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3

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Isabel Fabregat

5

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Jordi Boada

7

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Reinald Pamplona

10

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Francesc Villarroya

11

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Aurora Pujol

14

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Stéphane Fourcade

object named as

S Fourcade

13

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Manuel Portero-Otin

12

object named as

M Portero-Otín

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author name string

J López-Erauskin

1

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J Galino

2

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J M Cuezva

4

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D Cacabelos

6

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J Martínez

8

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I Ferrer

9

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publication date

20 April 2013

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published in

Human Molecular Genetics

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volume

22

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issue

16

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27 March 2018

page(s)

3296-3305

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General aspects and neuropathology of X-linked adrenoleukodystrophy.

cites work

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Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters

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The human peroxisomal ABC half transporter ALDP functions as a homodimer and accepts acyl-CoA esters

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A key role for the peroxisomal ABCD2 transporter in fatty acid homeostasis.

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Functional overlap between ABCD1 (ALD) and ABCD2 (ALDR) transporters: a therapeutic target for X-adrenoleukodystrophy

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Late onset neurological phenotype of the X-ALD gene inactivation in mice: a mouse model for adrenomyeloneuropathy.

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Valproic acid induces antioxidant effects in X-linked adrenoleukodystrophy

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Adreno-leukodystrophy: oxidative stress of mice and men.

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Early oxidative damage underlying neurodegeneration in X-adrenoleukodystrophy

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Oxidative stress modulates mitochondrial failure and cyclophilin D function in X-linked adrenoleukodystrophy

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Pathomechanisms underlying X-adrenoleukodystrophy: a three-hit hypothesis.

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Oxidative stress underlying axonal degeneration in adrenoleukodystrophy: a paradigm for multifactorial neurodegenerative diseases?

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Oxidative damage compromises energy metabolism in the axonal degeneration mouse model of X-adrenoleukodystrophy

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Antioxidants halt axonal degeneration in a mouse model of X-adrenoleukodystrophy.

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Inactivation of the peroxisomal ABCD2 transporter in the mouse leads to late-onset ataxia involving mitochondria, Golgi and endoplasmic reticulum damage.

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Role of ALDP (ABCD1) and mitochondria in X-linked adrenoleukodystrophy

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Peroxisomal and mitochondrial defects in the cerebro-hepato-renal syndrome.

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Mitochondrial alterations caused by defective peroxisomal biogenesis in a mouse model for Zellweger syndrome (PEX5 knockout mouse)

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Functional genomic analysis unravels a metabolic-inflammatory interplay in adrenoleukodystrophy

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Time course of post-traumatic mitochondrial oxidative damage and dysfunction in a mouse model of focal traumatic brain injury: implications for neuroprotective therapy

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Accumulation of very long-chain fatty acids does not affect mitochondrial function in adrenoleukodystrophy protein deficiency

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Mitochondrial bioenergetics and dynamics interplay in complex I-deficient fibroblasts.

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Multiparameter metabolic analysis reveals a close link between attenuated mitochondrial bioenergetic function and enhanced glycolysis dependency in human tumor cells

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Respiration and growth defects in transmitochondrial cell lines carrying the 11778 mutation associated with Leber's hereditary optic neuropathy

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Nonviability of cells with oxidative defects in galactose medium: a screening test for affected patient fibroblasts

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Oxidative stress and altered mitochondrial function in neurodegenerative diseases: lessons from mouse models

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Simple quantitative detection of mitochondrial superoxide production in live cells

21 January 2018

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Evidence of oxidative stress in very long chain fatty acid--treated oligodendrocytes and potentialization of ROS production using RNA interference-directed knockdown of ABCD1 and ACOX1 peroxisomal proteins.

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Highly resistant macromolecular components and low rate of generation of endogenous damage: two key traits of longevity.

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Analysis of differential DNA damage in the mitochondrial genome employing a semi-long run real-time PCR approach

21 January 2018

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Mitochondria: are they the seat of senescence?

21 January 2018

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Proteins in human brain cortex are modified by oxidation, glycoxidation, and lipoxidation. Effects of Alzheimer disease and identification of lipoxidation targets.

21 January 2018

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Mitochondrial matters of the brain: the role in Huntington's disease

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The mitochondrial impairment, oxidative stress and neurodegeneration connection: reality or just an attractive hypothesis?

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Mitochondrial functional alterations in relation to pathophysiology of Huntington's disease

21 January 2018

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Increased oxidative damage in nuclear and mitochondrial DNA in Alzheimer's disease.

21 January 2018

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Oxidative damage to mitochondrial DNA in Huntington's disease parietal cortex

21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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21 January 2018

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Phospholipids in X-linked adrenoleukodystrophy white matter: fatty acid abnormalities before the onset of demyelination

21 January 2018

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A mouse model for X-linked adrenoleukodystrophy

21 January 2018

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21 January 2018

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21 January 2018

Identifiers

DOI

10.1093/HMG/DDT186

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27 March 2018

PubMed ID

23604518

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27 March 2018