pantothenic acid and Hallervorden-Spatz Disease

pantothenic acid has been researched along with Hallervorden-Spatz Disease in 12 studies

Research

Studies (12)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's1 (8.33)29.6817
2010's8 (66.67)24.3611
2020's3 (25.00)2.80

Authors

AuthorsStudies
Ben Mamoun, C; Ceccatelli Berti, C; Choi, JY; Figuccia, S; Gihaz, S; Goffrini, P; Pal, AC1
Bischoff, AT; Bonnet, C; Burns, C; Cif, L; Comella, C; Correa-Vela, M; Escolar, ML; Fraser, JL; Gonzalez, V; Greblikas, F; Hermanowicz, N; Jech, R; Jinnah, HA; Klopstock, T; Kmiec, T; Lang, A; Martí, MJ; Mercimek-Andrews, S; Monduy, M; Nimmo, GAM; Perez-Dueñas, B; Pfeiffer, HCV; Planellas, L; Roze, E; Thakur, N; Tochen, L; Vanegas-Arroyave, N; Videnovic, A; Zorzi, G1
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
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
Escolar, ML; Greblikas, F; Klopstock, T; Marshall, RD; Perez-Dueñas, B; Tuller, S; Videnovic, A1
Hayflick, SJ1
Frank, M; Jackowski, S; Pate, C; Rock, CO; Zano, SP1
Csányi, B; Gregory, A; Hayflick, SJ; Hogarth, P; Klopstock, T; Klucken, A; Kmiec, T; Kurian, MA; Nardocci, N; Scalise, N; Sofia, F; Wood, P; Zagustin, T; Zorzi, G1
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
Trimble, M1

Reviews

1 review(s) available for pantothenic acid and Hallervorden-Spatz Disease

ArticleYear
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

Trials

2 trial(s) available for pantothenic acid and Hallervorden-Spatz Disease

ArticleYear
Fosmetpantotenate Randomized Controlled Trial in Pantothenate Kinase-Associated Neurodegeneration.
    Movement disorders : official journal of the Movement Disorder Society, 2021, Volume: 36, Issue:6

    Topics: Activities of Daily Living; Double-Blind Method; Humans; Pantothenate Kinase-Associated Neurodegeneration; Pantothenic Acid

2021
The FOsmetpantotenate Replacement Therapy (FORT) randomized, double-blind, Placebo-controlled pivotal trial: Study design and development methodology of a novel primary efficacy outcome in patients with pantothenate kinase-associated neurodegeneration.
    Clinical trials (London, England), 2019, Volume: 16, Issue:4

    Topics: Activities of Daily Living; Adolescent; Adult; Aged; Child; Clinical Trials, Phase III as Topic; Double-Blind Method; Humans; Middle Aged; Multicenter Studies as Topic; Pantothenate Kinase-Associated Neurodegeneration; Pantothenic Acid; Patient Reported Outcome Measures; Randomized Controlled Trials as Topic; Research Design; Treatment Outcome; Vitamin B Complex; Young Adult

2019

Other Studies

9 other study(ies) available for pantothenic acid and Hallervorden-Spatz Disease

ArticleYear
Evidence for a Conserved Function of Eukaryotic Pantothenate Kinases in the Regulation of Mitochondrial Homeostasis and Oxidative Stress.
    International journal of molecular sciences, 2022, Dec-27, Volume: 24, Issue:1

    Topics: Homeostasis; Humans; Iron; Mitochondria; Oxidative Stress; Pantothenate Kinase-Associated Neurodegeneration; Pantothenic Acid; Phosphotransferases (Alcohol Group Acceptor); Saccharomyces cerevisiae

2022
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
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
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
Consensus clinical management guideline for pantothenate kinase-associated neurodegeneration (PKAN).
    Molecular genetics and metabolism, 2017, Volume: 120, Issue:3

    Topics: Adolescent; Adult; Child; Humans; Iron Chelating Agents; Pantothenate Kinase-Associated Neurodegeneration; Pantothenic Acid; Phosphotransferases (Alcohol Group Acceptor); Practice Guidelines as Topic

2017
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
Magnetic resonance imaging and Hallervorden-Spatz syndrome.
    CNS spectrums, 2003, Volume: 8, Issue:6

    Topics: Brain; Humans; Iron; Magnetic Resonance Imaging; Pantothenate Kinase-Associated Neurodegeneration; Pantothenic Acid; Phosphotransferases (Alcohol Group Acceptor); Point Mutation

2003