Page last updated: 2024-08-17

coenzyme a and Pantothenate Kinase-Associated Neurodegeneration

coenzyme a has been researched along with Pantothenate Kinase-Associated Neurodegeneration in 25 studies

Research

Studies (25)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's4 (16.00)29.6817
2010's12 (48.00)24.3611
2020's9 (36.00)2.80

Authors

AuthorsStudies
Ben Mamoun, C; Munshi, MI; Yao, SJ1
Cavestro, C; Cozzi, A; Di Meo, I; Levi, S; Raimondi, M; Ripamonti, M; Rubio, A; Santambrogio, P; Taverna, S; Tiranti, V1
Hayflick, SJ; Jeong, SY; Sibon, OCM1
De Palma, G; Facchinello, N; Finazzi, D; Khatri, D; Mignani, L; Schiavone, M; Zizioli, D1
Cavestro, C; Di Meo, I; Diodato, D; Tiranti, V1
Cobb, J; Duffy, M; Fox, R; Freed, A; Gray, N; Gregory, AM; Hamada, J; Hayflick, SJ; Hogarth, P; Jeong, SY; Jin, H; Lambrechts, R; Nilsen, A; Pham, T; Placzek, A; Rai, P; Ralle, M; Schwanemann, L; Sibon, OC; van der Zwaag, M; Wakeman, K; Woltjer, RL; Zhen, D1
Frank, MW; Jackowski, S; Rock, CO; Subramanian, C; Yao, J1
Finazzi, D; Khatri, D; Mignani, L; Monti, E; Ritelli, M; Zizioli, D1
Auciello, G; Beconi, M; Bracacel, E; Ciammaichella, A; Cicero, D; Di Marco, A; Elbaum, D; Fezzardi, P; Gonzalez Paz, O; Harper, S; Malancona, S; Monteagudo, E; Rossetti, I; Vecchi, A1
Ayciriex, S; Cavestro, C; Di Meo, I; Fu, T; Lavermicocca, L; Levi, S; Ligorio, S; Manocchio, A; Mitro, N; Pedretti, S; Ripamonti, M; Santambrogio, P; Tiranti, V1
Abramov, AY; Angelova, PR; Arber, C; Bhatia, KP; Duce, JA; Gout, I; Hardy, J; Houlden, H; Mazzacuva, F; Mills, K; Preza, E; Tsuchiya, Y; Wiethoff, S; Wray, S1
Beconi, MG; Di Marco, A; Elbaum, D; Harper, S; Lyons, KA; Monteagudo, E; Quinton, MS; Vaino, A1
Álvarez-Córdoba, M; Armengol, JA; Cotán, D; de la Mata, M; Díaz-Quintana, A; Fernández Khoury, A; Gómez-Navarro, C; Pérez-Pulido, AJ; Pérez-Villegas, EM; Povea-Cabello, S; Salas, JJ; Sánchez-Alcázar, JA; Suárez-Rivero, JM; Talaverón-Rey, M; Villalón-García, I; Villanueva-Paz, M1
Frank, MW; Jackowski, S; Lee, RE; Rock, CO; Sharma, LK; Subramanian, C; White, SW; Yun, MK1
Hayflick, SJ1
Alfonso-Pecchio, A; Fagone, P; Gray, JT; Jackowski, S; Leonardi, R; Rehg, JE; Shumar, SA1
Frank, M; Jackowski, S; Pate, C; Rock, CO; Zano, SP1
Broccoli, V; Cancellieri, C; Cozzi, A; Dusi, S; Ferrari, M; Garavaglia, B; Giannelli, SG; Levi, S; Mazzara, PG; Orellana, DI; Rubio, A; Santambrogio, P; Taverna, S; Tiranti, V; Venco, P; Yekhlef, L1
Hayflick, S; Kayser, O; Muntendam, R; Rana, A; Reijngoud, DJ; Seinen, E; Sibon, OC; Siudeja, K; Srinivasan, B; van der Want, JJ1
de Jong, J; Hayflick, S; Jackowski, S; Nollen, EA; Rana, A; Sanford, L; Sibon, OC; Siudeja, K; Srinivasan, B; Xu, L1
Bertini, E; Caccia, C; Ciusani, E; Clish, CB; Deik, A; Dusi, S; Garavaglia, B; Leoni, V; Mootha, VK; Nardocci, N; Rimoldi, M; Souza, AL; Strittmatter, L; Tiranti, V; Venco, P; Zibordi, F; Zorzi, G1
Botz, E; Garavaglia, B; Hörtnagel, K; Klopstock, T; Meitinger, T; Nardocci, N; Zorzi, G1
Kotzbauer, PT; Lee, VM; Trojanowski, JQ; Truax, AC1
Albanese, A; Barone, P; Bentivoglio, AR; Bonuccelli, U; Cif, L; Coubes, P; D'Amico, A; Dallapiccola, B; Di Giorgio, A; Marelli, C; Pellecchia, MT; Salvi, S; Scarano, V; Valente, EM1
Gregory, A; Hayflick, SJ1

Reviews

5 review(s) available for coenzyme a and Pantothenate Kinase-Associated Neurodegeneration

ArticleYear
Redesigning therapies for pantothenate kinase-associated neurodegeneration.
    The Journal of biological chemistry, 2022, Volume: 298, Issue:3

    Topics: Animals; Coenzyme A; Humans; Mutation; Neurodegenerative Diseases; Pantothenate Kinase-Associated Neurodegeneration; Phosphotransferases (Alcohol Group Acceptor)

2022
PKAN pathogenesis and treatment.
    Molecular genetics and metabolism, 2022, Volume: 137, Issue:3

    Topics: Coenzyme A; Humans; Pantothenate Kinase-Associated Neurodegeneration; Phosphotransferases (Alcohol Group Acceptor)

2022
Inherited Disorders of Coenzyme A Biosynthesis: Models, Mechanisms, and Treatments.
    International journal of molecular sciences, 2023, Mar-21, Volume: 24, Issue:6

    Topics: Biosynthetic Pathways; Cardiomyopathy, Dilated; Coenzyme A; Humans; Iron; Pantothenate Kinase-Associated Neurodegeneration; Phosphotransferases (Alcohol Group Acceptor)

2023
Defective pantothenate metabolism and neurodegeneration.
    Biochemical Society transactions, 2014, Volume: 42, Issue:4

    Topics: Animals; Coenzyme A; Humans; Neurodegenerative Diseases; Pantothenate Kinase-Associated Neurodegeneration; Pantothenic Acid; Phosphotransferases (Alcohol Group Acceptor)

2014
Neurodegeneration with brain iron accumulation.
    Folia neuropathologica, 2005, Volume: 43, Issue:4

    Topics: Animals; Brain; Coenzyme A; Disease Models, Animal; Drosophila; Humans; Iron; Mice; Mutation; Nerve Degeneration; Pantothenate Kinase-Associated Neurodegeneration; Phosphotransferases (Alcohol Group Acceptor)

2005

Other Studies

20 other study(ies) available for coenzyme a and Pantothenate Kinase-Associated Neurodegeneration

ArticleYear
Massive iron accumulation in PKAN-derived neurons and astrocytes: light on the human pathological phenotype.
    Cell death & disease, 2022, 02-25, Volume: 13, Issue:2

    Topics: Astrocytes; Coenzyme A; Humans; Iron; Neurons; Pantothenate Kinase-Associated Neurodegeneration; Phenotype; Phosphotransferases (Alcohol Group Acceptor)

2022
Bi-Allelic Mutations in Zebrafish
    International journal of molecular sciences, 2022, Oct-26, Volume: 23, Issue:21

    Topics: Animals; Atrophy; Coenzyme A; Mutation; Pantothenate Kinase-Associated Neurodegeneration; Phosphotransferases (Alcohol Group Acceptor); Zebrafish

2022
4'-Phosphopantetheine corrects CoA, iron, and dopamine metabolic defects in mammalian models of PKAN.
    EMBO molecular medicine, 2019, Volume: 11, Issue:12

    Topics: Animals; Biomarkers; Coenzyme A; Dopamine; Genotype; Iron; Mice; Pantetheine; Pantothenate Kinase-Associated Neurodegeneration; Phosphotransferases (Alcohol Group Acceptor)

2019
A pantothenate kinase-deficient mouse model reveals a gene expression program associated with brain coenzyme a reduction.
    Biochimica et biophysica acta. Molecular basis of disease, 2020, 05-01, Volume: 1866, Issue:5

    Topics: Animals; Apoptosis Regulatory Proteins; Brain; Brain Chemistry; Coenzyme A; Disease Models, Animal; Female; Gene Expression Profiling; Gene Expression Regulation; Heme; Hemoglobins; Humans; Male; Mice; Mice, Knockout; Neurons; Oxidation-Reduction; Pantothenate Kinase-Associated Neurodegeneration; Phosphotransferases (Alcohol Group Acceptor); Repressor Proteins

2020
Abnormal Vasculature Development in Zebrafish Embryos with Reduced Expression of Pantothenate Kinase 2 Gene.
    Bulletin of experimental biology and medicine, 2020, Volume: 170, Issue:1

    Topics: Animals; Blood Vessels; Coenzyme A; Disease Models, Animal; Embryo, Nonmammalian; Gene Expression Regulation, Developmental; Globus Pallidus; Head; Humans; Morpholinos; Pantothenate Kinase-Associated Neurodegeneration; Phosphotransferases (Alcohol Group Acceptor); Tail; Torso; Tumor Suppressor Protein p53; Zebrafish

2020
Cyclic Phosphopantothenic Acid Prodrugs for Treatment of Pantothenate Kinase-Associated Neurodegeneration.
    Journal of medicinal chemistry, 2020, 12-24, Volume: 63, Issue:24

    Topics: Animals; Blood-Brain Barrier; Brain; Coenzyme A; Cyclization; Disease Models, Animal; Half-Life; Hepatocytes; Humans; Lipid Droplets; Mice; Mice, Inbred C57BL; Mice, Knockout; Pantothenate Kinase-Associated Neurodegeneration; Pantothenic Acid; Prodrugs; Structure-Activity Relationship

2020
Neuronal Ablation of CoA Synthase Causes Motor Deficits, Iron Dyshomeostasis, and Mitochondrial Dysfunctions in a CoPAN Mouse Model.
    International journal of molecular sciences, 2020, Dec-19, Volume: 21, Issue:24

    Topics: Animals; Coenzyme A; Coenzyme A Ligases; Female; Hemochromatosis; Homeostasis; Iron; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Mitochondria; Mitochondrial Diseases; Motor Disorders; Pantothenate Kinase-Associated Neurodegeneration; Synapsins

2020
iPSC-derived neuronal models of PANK2-associated neurodegeneration reveal mitochondrial dysfunction contributing to early disease.
    PloS one, 2017, Volume: 12, Issue:9

    Topics: Acetyl Coenzyme A; Adolescent; Biopsy; Brain; Cell Differentiation; Child; Coenzyme A; Female; Fibroblasts; Humans; Induced Pluripotent Stem Cells; Iron; Karyotyping; Lipid Peroxidation; Male; Membrane Potential, Mitochondrial; Mitochondria; Mitochondrial Diseases; Mutation; NAD; Neurons; Pantothenate Kinase-Associated Neurodegeneration; Pantothenic Acid; Phenotype; Phosphotransferases (Alcohol Group Acceptor); Plasmids; Reactive Oxygen Species

2017
Fosmetpantotenate (RE-024), a phosphopantothenate replacement therapy for pantothenate kinase-associated neurodegeneration: Mechanism of action and efficacy in nonclinical models.
    PloS one, 2018, Volume: 13, Issue:3

    Topics: Animals; Blood-Brain Barrier; Cell Line, Tumor; Coenzyme A; Disease Models, Animal; Humans; Macaca fascicularis; Male; Mice, Inbred C57BL; Neurons; Pantothenate Kinase-Associated Neurodegeneration; Pantothenic Acid; Phosphotransferases (Alcohol Group Acceptor); Prodrugs; Rats, Sprague-Dawley; RNA Interference; Species Specificity

2018
Pantothenate Rescues Iron Accumulation in Pantothenate Kinase-Associated Neurodegeneration Depending on the Type of Mutation.
    Molecular neurobiology, 2019, Volume: 56, Issue:5

    Topics: Cell Death; Cell Shape; Coenzyme A; Energy Metabolism; Fibroblasts; Gene Expression Regulation, Enzymologic; Humans; Iron; Lipid Peroxidation; Lipofuscin; Mitochondria; Mutation; Neurons; Neuroprotective Agents; Oxidative Stress; Pantothenate Kinase-Associated Neurodegeneration; Pantothenic Acid; Phosphotransferases (Alcohol Group Acceptor); Protein Carbonylation

2019
A therapeutic approach to pantothenate kinase associated neurodegeneration.
    Nature communications, 2018, 10-23, Volume: 9, Issue:1

    Topics: Adenosine Triphosphate; Allosteric Regulation; Animals; Cells, Cultured; Coenzyme A; Disease Models, Animal; Enzyme Stability; Female; Ligands; Magnesium; Male; Mice, Knockout; Neurons; Organ Specificity; Pantothenate Kinase-Associated Neurodegeneration; Phosphotransferases (Alcohol Group Acceptor); Protein Conformation; Protein Multimerization

2018
Induction of Neuron-Specific Degradation of Coenzyme A Models Pantothenate Kinase-Associated Neurodegeneration by Reducing Motor Coordination in Mice.
    PloS one, 2015, Volume: 10, Issue:6

    Topics: Animals; Coenzyme A; Mice; Mice, Inbred C57BL; Motor Activity; Neurons; Nudix Hydrolases; Pantothenate Kinase-Associated Neurodegeneration; Pyrophosphatases

2015
Correction of a genetic deficiency in pantothenate kinase 1 using phosphopantothenate replacement therapy.
    Molecular genetics and metabolism, 2015, Volume: 116, Issue:4

    Topics: Administration, Oral; Amides; Animals; Coenzyme A; Disease Models, Animal; Embryo, Mammalian; Female; Fibroblasts; Gene Expression; Humans; Liver; Male; Mice; Mice, Knockout; Pantothenate Kinase-Associated Neurodegeneration; Pantothenic Acid; Phosphoric Acids; Phosphotransferases (Alcohol Group Acceptor); Primary Cell Culture

2015
Coenzyme A corrects pathological defects in human neurons of PANK2-associated neurodegeneration.
    EMBO molecular medicine, 2016, Volume: 8, Issue:10

    Topics: Cell Death; Cells, Cultured; Coenzyme A; Humans; Mitochondria; Neurons; Pantothenate Kinase-Associated Neurodegeneration; Phosphotransferases (Alcohol Group Acceptor); Pluripotent Stem Cells; Reactive Oxygen Species

2016
Pantethine rescues a Drosophila model for pantothenate kinase-associated neurodegeneration.
    Proceedings of the National Academy of Sciences of the United States of America, 2010, Apr-13, Volume: 107, Issue:15

    Topics: Animals; Brain; Coenzyme A; Drosophila; Gene Expression Regulation; Humans; Mitochondria; Models, Biological; Mutation; Oxidative Stress; Oxygen; Pantetheine; Pantothenate Kinase-Associated Neurodegeneration; Phenotype; Phosphotransferases (Alcohol Group Acceptor)

2010
Impaired Coenzyme A metabolism affects histone and tubulin acetylation in Drosophila and human cell models of pantothenate kinase associated neurodegeneration.
    EMBO molecular medicine, 2011, Volume: 3, Issue:12

    Topics: Acetylation; Animals; Coenzyme A; Disease Models, Animal; Drosophila; Histones; Humans; Pantetheine; Pantothenate Kinase-Associated Neurodegeneration; Protein Processing, Post-Translational; Tubulin

2011
Metabolic consequences of mitochondrial coenzyme A deficiency in patients with PANK2 mutations.
    Molecular genetics and metabolism, 2012, Volume: 105, Issue:3

    Topics: Adolescent; Adult; Bile Acids and Salts; Child; Child, Preschool; Codon, Nonsense; Coenzyme A; Cohort Studies; Female; Humans; Iron Metabolism Disorders; Lactic Acid; Lipid Metabolism; Lipid Metabolism Disorders; Male; Metabolome; Mitochondria; Neuroaxonal Dystrophies; Pantothenate Kinase-Associated Neurodegeneration; Pantothenic Acid; Phosphotransferases (Alcohol Group Acceptor); Sphingomyelins; Young Adult

2012
Infantile neuroaxonal dystrophy and pantothenate-kinase-associated neurodegeneration: locus heterogeneity.
    Neurology, 2004, Sep-14, Volume: 63, Issue:5

    Topics: Alleles; Axons; Coenzyme A; Female; Genetic Heterogeneity; Genetic Markers; Genotype; Haplotypes; Humans; Male; Microsatellite Repeats; Neuroaxonal Dystrophies; Pantothenate Kinase-Associated Neurodegeneration; Pedigree; Phosphotransferases (Alcohol Group Acceptor)

2004
Altered neuronal mitochondrial coenzyme A synthesis in neurodegeneration with brain iron accumulation caused by abnormal processing, stability, and catalytic activity of mutant pantothenate kinase 2.
    The Journal of neuroscience : the official journal of the Society for Neuroscience, 2005, Jan-19, Volume: 25, Issue:3

    Topics: Amino Acid Sequence; Catalysis; Cell Line; Coenzyme A; Enzyme Stability; Half-Life; Humans; Immunohistochemistry; Mitochondria; Molecular Sequence Data; Neurons; Pantothenate Kinase-Associated Neurodegeneration; Phosphotransferases (Alcohol Group Acceptor); Point Mutation; Protein Isoforms; Protein Processing, Post-Translational

2005
The diverse phenotype and genotype of pantothenate kinase-associated neurodegeneration.
    Neurology, 2005, May-24, Volume: 64, Issue:10

    Topics: Adolescent; Adult; Brain Diseases, Metabolic, Inborn; Child; Coenzyme A; DNA Mutational Analysis; Female; Genetic Testing; Globus Pallidus; Heredodegenerative Disorders, Nervous System; Humans; Iron; Iron Metabolism Disorders; Male; Mental Disorders; Middle Aged; Mutation; Obsessive-Compulsive Disorder; Pantothenate Kinase-Associated Neurodegeneration; Phenotype; Phosphotransferases (Alcohol Group Acceptor); Pyramidal Tracts; Tics

2005