A novel bile acid biosynthesis defect due to a deficiency of peroxisomal ABCD3

S Ferdinandusse, G Jimenez-Sanchez… - Human molecular …, 2015 - academic.oup.com
ABCD3 is one of three ATP-binding cassette (ABC) transporters present in the peroxisomal
membrane catalyzing ATP-dependent transport of substrates for metabolic pathways …

The peroxisomal transporter ABCD3 plays a major role in hepatic dicarboxylic fatty acid metabolism and lipid homeostasis

P Ranea‐Robles, H Chen, B Stauffer… - Journal of inherited …, 2021 - Wiley Online Library
Peroxisomes metabolize a specific subset of fatty acids, which include dicarboxylic fatty
acids (DCAs) generated by ω‐oxidation. Data obtained in vitro suggest that the peroxisomal …

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

I Ferrer, JP Kapfhammer, C Hindelang… - Human molecular …, 2005 - academic.oup.com
ATP-binding cassette (ABC) transporters facilitate unidirectional translocation of chemically
diverse substances, ranging from peptides to lipids, across cell or organelle membranes. In …

[HTML][HTML] Peroxisomal ABC transporters: an update

A Tawbeh, C Gondcaille, D Trompier… - International Journal of …, 2021 - mdpi.com
ATP-binding cassette (ABC) transporters constitute one of the largest superfamilies of
conserved proteins from bacteria to mammals. In humans, three members of this family are …

A role for the human peroxisomal half-transporter ABCD3 in the oxidation of dicarboxylic acids

CWT van Roermund, L IJlst, T Wagemans… - … et Biophysica Acta (BBA …, 2014 - Elsevier
Peroxisomes play a major role in human cellular lipid metabolism, including fatty acid β-
oxidation. Free fatty acids (FFAs) can enter peroxisomes through passive diffusion or by …

Mammalian peroxisomal ABC transporters: from endogenous substrates to pathology and clinical significance

S Kemp, FL Theodoulou… - British journal of …, 2011 - Wiley Online Library
Peroxisomes are indispensable organelles in higher eukaryotes. They are essential for a
number of important metabolic pathways, including fatty acid α‐and β‐oxidation, and …

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

S Fourcade, M Ruiz, C Camps… - American Journal …, 2009 - journals.physiology.org
Peroxisomes are essential organelles exerting key functions in fatty acid metabolism such
as the degradation of very long-chain fatty acids (VLCFAs). VLCFAs accumulate in X …

Murine deficiency of peroxisomal l-bifunctional protein (EHHADH) causes medium-chain 3-hydroxydicarboxylic aciduria and perturbs hepatic cholesterol …

P Ranea-Robles, S Violante, C Argmann… - Cellular and Molecular …, 2021 - Springer
Peroxisomes play an essential role in the β-oxidation of dicarboxylic acids (DCAs), which
are metabolites formed upon ω-oxidation of fatty acids. Genetic evidence linking transporters …

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

CWT van Roermund, WF Visser, L IJlst… - The FASEB …, 2008 - Wiley Online Library
Peroxisomes play a major role in human cellular lipid metabolism, including the β‐oxidation
of fatty acids. The most frequent peroxisomal disorder is X‐linked adrenoleukodystrophy (X …

Differential substrate specificities of human ABCD1 and ABCD2 in peroxisomal fatty acid β-oxidation

CWT van Roermund, WF Visser, L IJlst… - … et Biophysica Acta (BBA …, 2011 - Elsevier
The gene mutated in X-linked adrenoleukodystrophy (X-ALD) codes for the HsABCD1
protein, also named ALDP, which is a member of the superfamily of ATP-binding cassette …