Dendrimer–N‐acetyl‐L‐cysteine modulates monophagocytic response in adrenoleukodystrophy

BR Turk, CL Nemeth, JS Marx, C Tiffany… - Annals of …, 2018 - Wiley Online Library
Objective X‐linked adrenoleukodystrophy (ALD) is a neurodegenerative disorder due to
mutations in the peroxisomal very long‐chain fatty acyl‐CoA transporter, ABCD1, with …

Protective effect of oleic acid against very long-chain fatty acid-induced apoptosis in peroxisome-deficient CHO cells

H Ali, M Yamanishi, K Sunagawa, M Kumon… - … et Biophysica Acta (BBA …, 2024 - Elsevier
Very long-chain fatty acids (VLCFAs) are degraded exclusively in peroxisomes, as
evidenced by the accumulation of VLCFAs in patients with certain peroxisomal disorders …

[HTML][HTML] Targeted Brain Delivery of Dendrimer-4-Phenylbutyrate Ameliorates Neurological Deficits in a Long-Term ABCD1-Deficient Mouse Model of X-Linked …

CL Nemeth, Ö Gӧk, SN Tomlinson, A Sharma… - …, 2023 - Springer
X-linked adrenoleukodystrophy (ALD) is a genetic disorder that presents neurologically as
either a rapid and fatal cerebral demyelinating disease in childhood (childhood cerebral …

Activating cannabinoid receptor 2 preserves axonal health through GSK-3β/NRF2 axis in adrenoleukodystrophy

J Parameswaran, L Goicoechea, L Planas-Serra… - Acta …, 2022 - Springer
Aberrant endocannabinoid signaling accompanies several neurodegenerative disorders,
including multiple sclerosis. Here, we report altered endocannabinoid signaling in X-linked …

[HTML][HTML] Antioxidant response in human X-Linked adrenoleukodystrophy fibroblasts

S Petrillo, J D'Amico, F Nicita, C Torda, G Vasco… - Antioxidants, 2022 - mdpi.com
Redox imbalance, mitochondrial dysfunction, and inflammation play a major role in the
pathophysiology of X-linked adrenoleukodystrophy (X-ALD), an inherited …

Can endoplasmic reticulum stress observed in the PTZ-kindling model seizures be prevented with TUDCA and 4-PBA?

Z Doğanyiğit, A Okan, E Akyüz, S Yılmaz, Ş Ateş… - European Journal of …, 2023 - Elsevier
Epilepsy is a chronic neurological disease with recurrent seizures. Increasing evidence
suggests that endoplasmic reticulum (ER) stress may play a role in the pathogenesis of …

[HTML][HTML] Management of adrenoleukodystrophy: From pre-clinical studies to the development of new therapies

CY Ma, C Li, X Zhou, Z Zhang, H Jiang, H Liu… - Biomedicine & …, 2021 - Elsevier
Abstract X-linked adrenoleukodystrophy (X-ALD) is an inherited neurodegenerative disorder
associated with mutations of the ABCD1 gene that encodes a peroxisomal transmembrane …

[HTML][HTML] TDP-43 proteinopathy in frontotemporal lobar degeneration and amyotrophic lateral sclerosis: From pathomechanisms to therapeutic strategies

PC Ho, TC Hsieh, KJ Tsai - Ageing Research Reviews, 2024 - Elsevier
Proteostasis failure is a common pathological characteristic in neurodegenerative diseases.
Revitalizing clearance systems could effectively mitigate these diseases. The transactivation …

Imbalanced mitochondrial dynamics contributes to the pathogenesis of X-linked adrenoleukodystrophy

N Launay, J Lopez-Erauskin, P Bianchi, S Guha… - Brain, 2024 - academic.oup.com
The peroxisomal disease adrenoleukodystrophy (X-ALD) is caused by loss of the transporter
of very-long-chain fatty acids (VLCFAs), ABCD1. An excess of VLCFAs disrupts essential …

Modulation of mitochondrial and inflammatory homeostasis through RIP140 is neuroprotective in an adrenoleukodystrophy mouse model

P Ranea‐Robles, J Galino, L Espinosa… - Neuropathology and …, 2022 - Wiley Online Library
Aims Mitochondrial dysfunction and inflammation are at the core of axonal degeneration in
several multifactorial neurodegenerative diseases, including multiple sclerosis, Alzheimer's …