4.5 Article

Therapeutic approach with commercial supplements for pantothenate kinase-associated neurodegeneration with residual PANK2 expression levels

相关参考文献

注意:仅列出部分参考文献,下载原文获取全部文献信息。
Article Neurosciences

Vitamin E prevents lipid peroxidation and iron accumulation in PLA2G6-Associated Neurodegeneration

Irene Villalon-Garcia et al.

Summary: In this study, the presence of lipid peroxidation, iron accumulation and mitochondrial dysfunction in two cellular models of PLA2G6-Associated Neurodegeneration (PLAN) was examined. Vitamin E was found to reverse the pathological features in both models, suggesting its potential as a therapeutic target for the disease.

NEUROBIOLOGY OF DISEASE (2022)

Article Neurosciences

Treatment for mitochondrial diseases

Tongling Liufu et al.

Summary: Mitochondrial diseases are primarily caused by mutations in mitochondrial or nuclear DNA, and current treatments are mainly supportive. While some interventions have shown improvement in outcome measurements, further evaluation and clinical trials are still needed.

REVIEWS IN THE NEUROSCIENCES (2021)

Review Clinical Neurology

Rational Design of Novel Therapies for Pantothenate Kinase-Associated Neurodegeneration

Nivedita Thakur et al.

Summary: PKAN is a rare neurogenetic disorder caused by genetic variants in the PANK2 gene, leading to dysfunction in pantothenate kinase activity. Recent scientific advances have enabled the design of novel therapeutic approaches for PKAN.

MOVEMENT DISORDERS (2021)

Article Genetics & Heredity

Down regulation of the expression of mitochondrial phosphopantetheinyl-proteins in pantothenate kinase-associated neurodegeneration: pathophysiological consequences and therapeutic perspectives

Monica Alvarez-Cordoba et al.

Summary: Mitochondrial phosphopantetheinyl-proteins expression levels are severely reduced in PKAN cells, and treatment with pantothenate can correct the expression levels of these proteins and restore the affected pathways.

ORPHANET JOURNAL OF RARE DISEASES (2021)

Review Geriatrics & Gerontology

Iron Accumulation and Lipid Peroxidation in the Aging Retina: Implication of Ferroptosis in Age-Related Macular Degeneration

Tantai Zhao et al.

Summary: Iron is crucial in various biological processes in the human body, playing a critical role in the visual phototransduction cascade in the retina. Excess iron can be toxic and contribute to oxidative stress-induced cell death in the aging retina, predisposing it to conditions like AMD characterized by iron accumulation and lipid peroxidation. Understanding iron accumulation mechanisms and lipid peroxidation, particularly through ferroptosis, is essential for delving into the pathogenesis of cell death and AMD.

AGING AND DISEASE (2021)

Review Biochemistry & Molecular Biology

Oxidative Stress, a Crossroad between Rare Diseases and Neurodegeneration

Carmen Espinos et al.

ANTIOXIDANTS (2020)

Review Biotechnology & Applied Microbiology

Multitarget Therapeutic Strategies for Alzheimer's Disease: Review on Emerging Target Combinations

Samuele Maramai et al.

BIOMED RESEARCH INTERNATIONAL (2020)

Review Endocrinology & Metabolism

Inborn errors of coenzyme A metabolism and neurodegeneration

Ivano Di Meo et al.

JOURNAL OF INHERITED METABOLIC DISEASE (2019)

Review Biochemistry & Molecular Biology

Omega-3 Fatty Acids and Neurodegenerative Diseases: New Evidence in Clinical Trials

Rossella Avallone et al.

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES (2019)

Article Medicine, Research & Experimental

CoA-dependent activation of mitochondrial acyl carrier protein links four neurodegenerative diseases

Roald A. Lambrechts et al.

EMBO MOLECULAR MEDICINE (2019)

Review Cell Biology

Multitarget therapeutic strategies for Alzheimer's disease

Mostafa M. Ibrahim et al.

NEURAL REGENERATION RESEARCH (2019)

Review Biochemistry & Molecular Biology

Protein lipoylation: an evolutionarily conserved metabolic regulator of health and disease

Elizabeth A. Rowland et al.

CURRENT OPINION IN CHEMICAL BIOLOGY (2018)

Review Chemistry, Medicinal

Links Between Iron and Lipids: Implications in Some Major Human Diseases

Stephanie Rockfield et al.

PHARMACEUTICALS (2018)

Article Medicine, Research & Experimental

Coenzyme A corrects pathological defects in human neurons of PANK2-associated neurodegeneration

Daniel I. Orellana et al.

EMBO MOLECULAR MEDICINE (2016)

Article Biochemistry & Molecular Biology

Allosteric Regulation of Mammalian Pantothenate Kinase

Chitra Subramanian et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2016)

Review Nutrition & Dietetics

Dietary DHA and health: cognitive function ageing

Carlos Cardoso et al.

NUTRITION RESEARCH REVIEWS (2016)

Article Biochemistry & Molecular Biology

Metformin and caloric restriction induce an AMPK-dependent restoration of mitochondrial dysfunction in fibroblasts from Fibromyalgia patients

Elisabet Alcocer-Gomez et al.

BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE (2015)

Article Endocrinology & Metabolism

Correction of a genetic deficiency in pantothenate kinase 1 using phosphopantothenate replacement therapy

Stephen P. Zano et al.

MOLECULAR GENETICS AND METABOLISM (2015)

Review Neurosciences

Vitamin E and neurodegeneration

Lynn M. Ulatowski et al.

NEUROBIOLOGY OF DISEASE (2015)

Review Biochemistry & Molecular Biology

The phosphopantetheinyl transferases: catalysis of a post-translational modification crucial for life

Joris Beld et al.

NATURAL PRODUCT REPORTS (2014)

Review Cell Biology

Lipid Peroxidation: Production, Metabolism, and Signaling Mechanisms of Malondialdehyde and 4-Hydroxy-2-Nonenal

Antonio Ayala et al.

OXIDATIVE MEDICINE AND CELLULAR LONGEVITY (2014)

Review Pharmacology & Pharmacy

Neurodegeneration with brain iron accumulation: update on pathogenic mechanisms

Sonia Levi et al.

FRONTIERS IN PHARMACOLOGY (2014)

Article Biochemistry & Molecular Biology

Omega-3 fatty acids in neurodegenerative diseases: Focus on mitochondria

Gunter P. Eckert et al.

PROSTAGLANDINS LEUKOTRIENES AND ESSENTIAL FATTY ACIDS (2013)

Review Biochemistry & Molecular Biology

Lipofuscin: formation, effects and role of macroautophagy

Annika Hoehn et al.

REDOX BIOLOGY (2013)

Article Radiology, Nuclear Medicine & Medical Imaging

Missense PANK2 mutation without Eye of the tiger sign: MR findings in a large group of patients with pantothenate kinase-associated neurodegeneration (PKAN)

Rafael Fermin Delgado et al.

JOURNAL OF MAGNETIC RESONANCE IMAGING (2012)

Review Chemistry, Multidisciplinary

Free Radical Lipid Peroxidation: Mechanisms and Analysis

Huiyong Yin et al.

CHEMICAL REVIEWS (2011)

Article Genetics & Heredity

Characterization of the human PANK2 promoter

Brenda J. Polster et al.

Article Multidisciplinary Sciences

Pantethine rescues a Drosophila model for pantothenate kinase-associated neurodegeneration

Anil Rana et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2010)

Review Biochemistry & Molecular Biology

Mitochondrial Fatty Acid Synthesis Type II: More than Just Fatty Acids

J. Kalervo Hiltunen et al.

JOURNAL OF BIOLOGICAL CHEMISTRY (2009)

Review Genetics & Heredity

Clinical and genetic delineation of neurodegeneration with brain iron accumulation

A. Gregory et al.

JOURNAL OF MEDICAL GENETICS (2009)

Article Biochemistry & Molecular Biology

Neuroprotective action of omega-3 polyunsaturated fatty acids against neurodegenerative diseases: Evidence from animal studies

Frederic Calon et al.

PROSTAGLANDINS LEUKOTRIENES AND ESSENTIAL FATTY ACIDS (2007)

Review Biochemistry & Molecular Biology

Vitamin E and neurodegenerative diseases

Roberta Ricciarelli et al.

MOLECULAR ASPECTS OF MEDICINE (2007)

Review Pharmacology & Pharmacy

Multi-target therapeutics: when the whole is greater than the sum of the parts

Grant R. Zimmermann et al.

DRUG DISCOVERY TODAY (2007)

Review Multidisciplinary Sciences

Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases

Michael T. Lin et al.

NATURE (2006)

Review Integrative & Complementary Medicine

Review of the biochemistry, metabolism and clinical benefits of thiamin(e) and its derivatives

D Lonsdale

EVIDENCE-BASED COMPLEMENTARY AND ALTERNATIVE MEDICINE (2006)

Article Biotechnology & Applied Microbiology

Can cell systems biology rescue drug discovery?

EC Butcher

NATURE REVIEWS DRUG DISCOVERY (2005)

Review Biochemistry & Molecular Biology

Coenzyme A: Back in action

R Leonardi et al.

PROGRESS IN LIPID RESEARCH (2005)

Article Biotechnology & Applied Microbiology

Multicomponent therapeutics for networked systems

CT Keith et al.

NATURE REVIEWS DRUG DISCOVERY (2005)

Article Biochemistry & Molecular Biology

Mitochondrial transcription factor A regulates mtDNA copy number in mammals

MI Ekstrand et al.

HUMAN MOLECULAR GENETICS (2004)

Article Biochemical Research Methods

Colorimetric ferrozine-based assay for the quantitation of iron in cultured cells

J Riemer et al.

ANALYTICAL BIOCHEMISTRY (2004)

Article Multidisciplinary Sciences

Systematic discovery of multicomponent therapeutics

AA Borisy et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2003)

Article Medicine, General & Internal

Genetic, clinical, and radiographic delineation of Hallervorden-Spatz syndrome.

SJ Hayflick et al.

NEW ENGLAND JOURNAL OF MEDICINE (2003)

Article Multidisciplinary Sciences

The remarkable structural and functional organization of the eukaryotic pyruvate dehydrogenase complexes

ZH Zhou et al.

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2001)