Dendrimer–N‐acetyl‐L‐cysteine modulates monophagocytic response in adrenoleukodystrophy
Objective X‐linked adrenoleukodystrophy (ALD) is a neurodegenerative disorder due to
mutations in the peroxisomal very long‐chain fatty acyl‐CoA transporter, ABCD1, with …
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 …
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 …
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
Aberrant endocannabinoid signaling accompanies several neurodegenerative disorders,
including multiple sclerosis. Here, we report altered endocannabinoid signaling in X-linked …
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 …
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?
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 …
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 …
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 …
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 …
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
Aims Mitochondrial dysfunction and inflammation are at the core of axonal degeneration in
several multifactorial neurodegenerative diseases, including multiple sclerosis, Alzheimer's …
several multifactorial neurodegenerative diseases, including multiple sclerosis, Alzheimer's …