[HTML][HTML] Peroxisomes in brain development and function

J Berger, F Dorninger, S Forss-Petter… - Biochimica Et Biophysica …, 2016 - Elsevier
Peroxisomes contain numerous enzymatic activities that are important for mammalian
physiology. Patients lacking either all peroxisomal functions or a single enzyme or …

[HTML][HTML] Brain lipotoxicity of phytanic acid and very long-chain fatty acids. Harmful cellular/mitochondrial activities in Refsum disease and X-linked …

P Schönfeld, G Reiser - Aging and disease, 2016 - ncbi.nlm.nih.gov
It is increasingly understood that in the aging brain, especially in the case of patients
suffering from neurodegenerative diseases, some fatty acids at pathologically high …

Pioglitazone ameliorates the phenotype of a novel Parkinson's disease mouse model by reducing neuroinflammation

M Pinto, N Nissanka, S Peralta, R Brambilla… - Molecular …, 2016 - Springer
Background Parkinson's disease (PD) is a progressive neurodegenerative disorder
characterized by motor and non-motor symptoms. The cause of the motor symptoms is the …

In vivo evidence of mitochondrial dysfunction and altered redox homeostasis in a genetic mouse model of propionic acidemia: implications for the pathophysiology of …

L Gallego-Villar, A Rivera-Barahona… - Free Radical Biology …, 2016 - Elsevier
Accumulation of toxic metabolites has been described to inhibit mitochondrial enzymes,
thereby inducing oxidative stress in propionic acidemia (PA), an autosomal recessive …

Enhanced mitochondrial biogenesis ameliorates disease phenotype in a full-length mouse model of Huntington's disease

A Chandra, A Sharma, NY Calingasan… - Human molecular …, 2016 - academic.oup.com
Huntington's disease (HD) is a devastating illness and at present there is no disease
modifying therapy or cure for it; and management of the disease is limited to a few treatment …

Metformin‐induced mitochondrial function and ABCD 2 up‐regulation in X‐linked adrenoleukodystrophy involves AMP‐activated protein kinase

J Singh, B Olle, H Suhail, MM Felicella… - Journal of …, 2016 - Wiley Online Library
Abstract X‐linked adrenoleukodystrophy (X‐ALD) is a progressive neurometabolic disease
caused by mutations/deletions in the Abcd1 gene. Similar mutations/deletions in the Abcd1 …

Brain lipotoxicity of phytanic acid and very long-chain fatty acids. harmful cellular/mitochondrial activities in Refsum disease and X-linked adrenoleukodystrophy

P Schonfeld, G Reiser - Aging and disease, 2016 - go.gale.com
It is increasingly understood that in the aging brain, especially in the case of patients
suffering from neurodegenerative diseases, some fatty acids at pathologically high …

Novel therapeutic targets and drug candidates for modifying disease progression in adrenoleukodystrophy

A Pujol - Advanced Therapies in Pediatric Endocrinology and …, 2016 - karger.com
X-linked adrenoleukodystrophy (X-ALD) is the most frequent inherited monogenic
demyelinating disease. It is often lethal and currently lacks a satisfactory therapy. The …

X-linked adrenoleukodystrophy in Norway Clinical and epidemiological aspects

MA Horn - 2016 - duo.uio.no
In this thesis for the degree of PhD, cand. med. Morten Andreas Horn and his coworkers
have surveyed the Norwegian population of patients with X-linked adrenoleukodystrophy …

[PDF][PDF] X 연관부신백질이영양증의분류, 진단및치료의최신지견

정을식, 고아라, 강훈철 - 대한소아신경학회지, 2016 - annchildneurol.org
X-linked adrenoleukodystrophy (X-ALD) is caused by mutations in the ATP binding cassette
subfamily D member 1 (ABCD1), a gene that encodes peroxisomal membrane located on …