4′‐Phosphopantetheine corrects CoA, iron, and dopamine metabolic defects in mammalian models of PKAN

Pantothenate kinase‐associated neurodegeneration (PKAN) is an inborn error of CoA metabolism causing dystonia, parkinsonism, and brain iron accumulation. Lack of a good mammalian model has impeded studies of pathogenesis and development of rational therapeutics. We took a new approach to investigati...

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Published inEMBO molecular medicine Vol. 11; no. 12; pp. e10489 - n/a
Main Authors Jeong, Suh Young, Hogarth, Penelope, Placzek, Andrew, Gregory, Allison M, Fox, Rachel, Zhen, Dolly, Hamada, Jeffrey, van der Zwaag, Marianne, Lambrechts, Roald, Jin, Haihong, Nilsen, Aaron, Cobb, Jared, Pham, Thao, Gray, Nora, Ralle, Martina, Duffy, Megan, Schwanemann, Leila, Rai, Puneet, Freed, Alison, Wakeman, Katrina, Woltjer, Randall L, Sibon, Ody CM, Hayflick, Susan J
Format Journal Article
LanguageEnglish
Published London Nature Publishing Group UK 01.12.2019
EMBO Press
John Wiley and Sons Inc
Springer Nature
Subjects
4′‐phosphopantetheine
Acyl carrier protein
Animals
Basal ganglia
Biomarkers
Biomarkers - metabolism
Brain research
Central nervous system diseases
coenzyme A
Coenzyme A - metabolism
Defects
Dopamine
Dopamine - metabolism
Drug development
Dystonia
EMBO08
EMBO27
Gene expression
Genotype
Homeostasis
Iron
Iron - metabolism
Kinases
Mammals
Metabolism
Mice
Mitochondria
Movement disorders
Mutation
NBIA
Neurodegeneration
Oxidative phosphorylation
PANK2
Pantetheine - analogs & derivatives
Pantetheine - pharmacology
Pantetheine - therapeutic use
Pantothenate kinase
Pantothenate Kinase-Associated Neurodegeneration - drug therapy
Pantothenate Kinase-Associated Neurodegeneration - metabolism
Pathogenesis
Pharmacodynamics
Phosphorylation
Phosphotransferases (Alcohol Group Acceptor) - metabolism
PKAN
Pyruvic acid
Sulfur
NBIA
PANK2
4′‐phosphopantetheine
PKAN
coenzyme A
4′-phosphopantetheine
Online AccessGet full text
ISSN1757-4676
1757-4684
1757-4684
DOI10.15252/emmm.201910489

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Abstract Pantothenate kinase‐associated neurodegeneration (PKAN) is an inborn error of CoA metabolism causing dystonia, parkinsonism, and brain iron accumulation. Lack of a good mammalian model has impeded studies of pathogenesis and development of rational therapeutics. We took a new approach to investigating an existing mouse mutant of Pank2 and found that isolating the disease‐vulnerable brain revealed regional perturbations in CoA metabolism, iron homeostasis, and dopamine metabolism and functional defects in complex I and pyruvate dehydrogenase. Feeding mice a CoA pathway intermediate, 4′‐phosphopantetheine, normalized levels of the CoA‐, iron‐, and dopamine‐related biomarkers as well as activities of mitochondrial enzymes. Human cell changes also were recovered by 4′‐phosphopantetheine. We can mechanistically link a defect in CoA metabolism to these secondary effects via the activation of mitochondrial acyl carrier protein, which is essential to oxidative phosphorylation, iron–sulfur cluster biogenesis, and mitochondrial fatty acid synthesis. We demonstrate the fidelity of our model in recapitulating features of the human disease. Moreover, we identify pharmacodynamic biomarkers, provide insights into disease pathogenesis, and offer evidence for 4′‐phosphopantetheine as a candidate therapeutic for PKAN. Synopsis Mutations in PANK2 cause pantothenate kinase‐associated neurodegeneration (PKAN), a neurodegeneration with brain iron accumulation (NBIA) disorder. This study presents a mouse model that recapitulates key features of the human disease and shows rescue by a coenzyme A pathway intermediate. Germline deletion of Pank2 , encoding pantothenate kinase 2, causes defects in CoA, iron, and dopamine metabolism and diminished activities of mitochondrial aconitase, complex I, and pyruvate dehydrogenase (PDH) in globus pallidus. Regional biomarker abnormalities, which are revealed by isolating disease‐vulnerable brain regions, are specifically attributable to a defect in Pank2 alone, without the need to superimpose further genetic or metabolic defects. Correction of the CoA metabolic defect by oral administration of 4′‐phosphopantetheine recovers iron and dopamine homeostasis in brain and normalizes mitochondrial complex I and PDH activities. Graphical Abstract Mutations in PANK2 cause pantothenate kinase‐associated neurodegeneration (PKAN), a neurodegeneration with brain iron accumulation (NBIA) disorder. This study presents a mouse model that recapitulates key features of the human disease and shows rescue by a coenzyme A pathway intermediate.
AbstractList Pantothenate kinase‐associated neurodegeneration ( PKAN ) is an inborn error of CoA metabolism causing dystonia, parkinsonism, and brain iron accumulation. Lack of a good mammalian model has impeded studies of pathogenesis and development of rational therapeutics. We took a new approach to investigating an existing mouse mutant of Pank2 and found that isolating the disease‐vulnerable brain revealed regional perturbations in CoA metabolism, iron homeostasis, and dopamine metabolism and functional defects in complex I and pyruvate dehydrogenase. Feeding mice a CoA pathway intermediate, 4′‐phosphopantetheine, normalized levels of the CoA‐, iron‐, and dopamine‐related biomarkers as well as activities of mitochondrial enzymes. Human cell changes also were recovered by 4′‐phosphopantetheine. We can mechanistically link a defect in CoA metabolism to these secondary effects via the activation of mitochondrial acyl carrier protein, which is essential to oxidative phosphorylation, iron–sulfur cluster biogenesis, and mitochondrial fatty acid synthesis. We demonstrate the fidelity of our model in recapitulating features of the human disease. Moreover, we identify pharmacodynamic biomarkers, provide insights into disease pathogenesis, and offer evidence for 4′‐phosphopantetheine as a candidate therapeutic for PKAN . Mutations in PANK 2 cause pantothenate kinase‐associated neurodegeneration ( PKAN ), a neurodegeneration with brain iron accumulation ( NBIA ) disorder. This study presents a mouse model that recapitulates key features of the human disease and shows rescue by a coenzyme A pathway intermediate.
Abstract Pantothenate kinase‐associated neurodegeneration (PKAN) is an inborn error of CoA metabolism causing dystonia, parkinsonism, and brain iron accumulation. Lack of a good mammalian model has impeded studies of pathogenesis and development of rational therapeutics. We took a new approach to investigating an existing mouse mutant of Pank2 and found that isolating the disease‐vulnerable brain revealed regional perturbations in CoA metabolism, iron homeostasis, and dopamine metabolism and functional defects in complex I and pyruvate dehydrogenase. Feeding mice a CoA pathway intermediate, 4′‐phosphopantetheine, normalized levels of the CoA‐, iron‐, and dopamine‐related biomarkers as well as activities of mitochondrial enzymes. Human cell changes also were recovered by 4′‐phosphopantetheine. We can mechanistically link a defect in CoA metabolism to these secondary effects via the activation of mitochondrial acyl carrier protein, which is essential to oxidative phosphorylation, iron–sulfur cluster biogenesis, and mitochondrial fatty acid synthesis. We demonstrate the fidelity of our model in recapitulating features of the human disease. Moreover, we identify pharmacodynamic biomarkers, provide insights into disease pathogenesis, and offer evidence for 4′‐phosphopantetheine as a candidate therapeutic for PKAN.
Pantothenate kinase‐associated neurodegeneration (PKAN) is an inborn error of CoA metabolism causing dystonia, parkinsonism, and brain iron accumulation. Lack of a good mammalian model has impeded studies of pathogenesis and development of rational therapeutics. We took a new approach to investigating an existing mouse mutant of Pank2 and found that isolating the disease‐vulnerable brain revealed regional perturbations in CoA metabolism, iron homeostasis, and dopamine metabolism and functional defects in complex I and pyruvate dehydrogenase. Feeding mice a CoA pathway intermediate, 4′‐phosphopantetheine, normalized levels of the CoA‐, iron‐, and dopamine‐related biomarkers as well as activities of mitochondrial enzymes. Human cell changes also were recovered by 4′‐phosphopantetheine. We can mechanistically link a defect in CoA metabolism to these secondary effects via the activation of mitochondrial acyl carrier protein, which is essential to oxidative phosphorylation, iron–sulfur cluster biogenesis, and mitochondrial fatty acid synthesis. We demonstrate the fidelity of our model in recapitulating features of the human disease. Moreover, we identify pharmacodynamic biomarkers, provide insights into disease pathogenesis, and offer evidence for 4′‐phosphopantetheine as a candidate therapeutic for PKAN.
Pantothenate kinase-associated neurodegeneration (PKAN) is an inborn error of CoA metabolism causing dystonia, parkinsonism, and brain iron accumulation. Lack of a good mammalian model has impeded studies of pathogenesis and development of rational therapeutics. We took a new approach to investigating an existing mouse mutant of Pank2 and found that isolating the disease-vulnerable brain revealed regional perturbations in CoA metabolism, iron homeostasis, and dopamine metabolism and functional defects in complex I and pyruvate dehydrogenase. Feeding mice a CoA pathway intermediate, 4'-phosphopantetheine, normalized levels of the CoA-, iron-, and dopamine-related biomarkers as well as activities of mitochondrial enzymes. Human cell changes also were recovered by 4'-phosphopantetheine. We can mechanistically link a defect in CoA metabolism to these secondary effects via the activation of mitochondrial acyl carrier protein, which is essential to oxidative phosphorylation, iron-sulfur cluster biogenesis, and mitochondrial fatty acid synthesis. We demonstrate the fidelity of our model in recapitulating features of the human disease. Moreover, we identify pharmacodynamic biomarkers, provide insights into disease pathogenesis, and offer evidence for 4'-phosphopantetheine as a candidate therapeutic for PKAN.Pantothenate kinase-associated neurodegeneration (PKAN) is an inborn error of CoA metabolism causing dystonia, parkinsonism, and brain iron accumulation. Lack of a good mammalian model has impeded studies of pathogenesis and development of rational therapeutics. We took a new approach to investigating an existing mouse mutant of Pank2 and found that isolating the disease-vulnerable brain revealed regional perturbations in CoA metabolism, iron homeostasis, and dopamine metabolism and functional defects in complex I and pyruvate dehydrogenase. Feeding mice a CoA pathway intermediate, 4'-phosphopantetheine, normalized levels of the CoA-, iron-, and dopamine-related biomarkers as well as activities of mitochondrial enzymes. Human cell changes also were recovered by 4'-phosphopantetheine. We can mechanistically link a defect in CoA metabolism to these secondary effects via the activation of mitochondrial acyl carrier protein, which is essential to oxidative phosphorylation, iron-sulfur cluster biogenesis, and mitochondrial fatty acid synthesis. We demonstrate the fidelity of our model in recapitulating features of the human disease. Moreover, we identify pharmacodynamic biomarkers, provide insights into disease pathogenesis, and offer evidence for 4'-phosphopantetheine as a candidate therapeutic for PKAN.
Pantothenate kinase‐associated neurodegeneration (PKAN) is an inborn error of CoA metabolism causing dystonia, parkinsonism, and brain iron accumulation. Lack of a good mammalian model has impeded studies of pathogenesis and development of rational therapeutics. We took a new approach to investigating an existing mouse mutant of Pank2 and found that isolating the disease‐vulnerable brain revealed regional perturbations in CoA metabolism, iron homeostasis, and dopamine metabolism and functional defects in complex I and pyruvate dehydrogenase. Feeding mice a CoA pathway intermediate, 4′‐phosphopantetheine, normalized levels of the CoA‐, iron‐, and dopamine‐related biomarkers as well as activities of mitochondrial enzymes. Human cell changes also were recovered by 4′‐phosphopantetheine. We can mechanistically link a defect in CoA metabolism to these secondary effects via the activation of mitochondrial acyl carrier protein, which is essential to oxidative phosphorylation, iron–sulfur cluster biogenesis, and mitochondrial fatty acid synthesis. We demonstrate the fidelity of our model in recapitulating features of the human disease. Moreover, we identify pharmacodynamic biomarkers, provide insights into disease pathogenesis, and offer evidence for 4′‐phosphopantetheine as a candidate therapeutic for PKAN. Synopsis Mutations in PANK2 cause pantothenate kinase‐associated neurodegeneration (PKAN), a neurodegeneration with brain iron accumulation (NBIA) disorder. This study presents a mouse model that recapitulates key features of the human disease and shows rescue by a coenzyme A pathway intermediate. Germline deletion of Pank2 , encoding pantothenate kinase 2, causes defects in CoA, iron, and dopamine metabolism and diminished activities of mitochondrial aconitase, complex I, and pyruvate dehydrogenase (PDH) in globus pallidus. Regional biomarker abnormalities, which are revealed by isolating disease‐vulnerable brain regions, are specifically attributable to a defect in Pank2 alone, without the need to superimpose further genetic or metabolic defects. Correction of the CoA metabolic defect by oral administration of 4′‐phosphopantetheine recovers iron and dopamine homeostasis in brain and normalizes mitochondrial complex I and PDH activities. Graphical Abstract Mutations in PANK2 cause pantothenate kinase‐associated neurodegeneration (PKAN), a neurodegeneration with brain iron accumulation (NBIA) disorder. This study presents a mouse model that recapitulates key features of the human disease and shows rescue by a coenzyme A pathway intermediate.
Pantothenate kinase‐associated neurodegeneration (PKAN) is an inborn error of CoA metabolism causing dystonia, parkinsonism, and brain iron accumulation. Lack of a good mammalian model has impeded studies of pathogenesis and development of rational therapeutics. We took a new approach to investigating an existing mouse mutant of Pank2 and found that isolating the disease‐vulnerable brain revealed regional perturbations in CoA metabolism, iron homeostasis, and dopamine metabolism and functional defects in complex I and pyruvate dehydrogenase. Feeding mice a CoA pathway intermediate, 4′‐phosphopantetheine, normalized levels of the CoA‐, iron‐, and dopamine‐related biomarkers as well as activities of mitochondrial enzymes. Human cell changes also were recovered by 4′‐phosphopantetheine. We can mechanistically link a defect in CoA metabolism to these secondary effects via the activation of mitochondrial acyl carrier protein, which is essential to oxidative phosphorylation, iron–sulfur cluster biogenesis, and mitochondrial fatty acid synthesis. We demonstrate the fidelity of our model in recapitulating features of the human disease. Moreover, we identify pharmacodynamic biomarkers, provide insights into disease pathogenesis, and offer evidence for 4′‐phosphopantetheine as a candidate therapeutic for PKAN. Synopsis Mutations in PANK2 cause pantothenate kinase‐associated neurodegeneration (PKAN), a neurodegeneration with brain iron accumulation (NBIA) disorder. This study presents a mouse model that recapitulates key features of the human disease and shows rescue by a coenzyme A pathway intermediate. Germline deletion of Pank2, encoding pantothenate kinase 2, causes defects in CoA, iron, and dopamine metabolism and diminished activities of mitochondrial aconitase, complex I, and pyruvate dehydrogenase (PDH) in globus pallidus. Regional biomarker abnormalities, which are revealed by isolating disease‐vulnerable brain regions, are specifically attributable to a defect in Pank2 alone, without the need to superimpose further genetic or metabolic defects. Correction of the CoA metabolic defect by oral administration of 4′‐phosphopantetheine recovers iron and dopamine homeostasis in brain and normalizes mitochondrial complex I and PDH activities. Mutations in PANK2 cause pantothenate kinase‐associated neurodegeneration (PKAN), a neurodegeneration with brain iron accumulation (NBIA) disorder. This study presents a mouse model that recapitulates key features of the human disease and shows rescue by a coenzyme A pathway intermediate.
Author Nilsen, Aaron
Gregory, Allison M
Jeong, Suh Young
Pham, Thao
Sibon, Ody CM
Duffy, Megan
Freed, Alison
Placzek, Andrew
Lambrechts, Roald
Jin, Haihong
Rai, Puneet
Wakeman, Katrina
van der Zwaag, Marianne
Hogarth, Penelope
Hamada, Jeffrey
Woltjer, Randall L
Hayflick, Susan J
Schwanemann, Leila
Fox, Rachel
Cobb, Jared
Zhen, Dolly
Gray, Nora
Ralle, Martina
AuthorAffiliation 4 Department of Cell Biology University Medical Center Groningen Groningen the Netherlands
3 Medicinal Chemistry Core Oregon Health & Science University Portland OR USA
1 Department of Molecular & Medical Genetics Oregon Health & Science University Portland OR USA
5 Department of Pathology Oregon Health & Science University Portland OR USA
6 Department of Pediatrics Oregon Health & Science University Portland OR USA
2 Department of Neurology Oregon Health & Science University Portland OR USA
AuthorAffiliation_xml – name: 1 Department of Molecular & Medical Genetics Oregon Health & Science University Portland OR USA
– name: 4 Department of Cell Biology University Medical Center Groningen Groningen the Netherlands
– name: 3 Medicinal Chemistry Core Oregon Health & Science University Portland OR USA
– name: 6 Department of Pediatrics Oregon Health & Science University Portland OR USA
– name: 2 Department of Neurology Oregon Health & Science University Portland OR USA
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BackLink https://www.ncbi.nlm.nih.gov/pubmed/31660701$$D View this record in MEDLINE/PubMed
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ContentType Journal Article
Copyright The Author(s) 2019
2019 The Authors. Published under the terms of the CC BY 4.0 license
2019 The Authors. Published under the terms of the CC BY 4.0 license.
2019. This work is published under http://creativecommons.org/licenses/by/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.
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Issue 12
Keywords NBIA
PANK2
4′‐phosphopantetheine
PKAN
coenzyme A
4′-phosphopantetheine
Language English
License Attribution
2019 The Authors. Published under the terms of the CC BY 4.0 license.
This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
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See also: https://doi.org/10.15252/emmm.201910488 (December 2019)
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Snippet Pantothenate kinase‐associated neurodegeneration (PKAN) is an inborn error of CoA metabolism causing dystonia, parkinsonism, and brain iron accumulation. Lack...
Pantothenate kinase-associated neurodegeneration (PKAN) is an inborn error of CoA metabolism causing dystonia, parkinsonism, and brain iron accumulation. Lack...
Pantothenate kinase‐associated neurodegeneration ( PKAN ) is an inborn error of CoA metabolism causing dystonia, parkinsonism, and brain iron accumulation....
Abstract Pantothenate kinase‐associated neurodegeneration (PKAN) is an inborn error of CoA metabolism causing dystonia, parkinsonism, and brain iron...
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SubjectTerms 4′‐phosphopantetheine
Acyl carrier protein
Animals
Basal ganglia
Biomarkers
Biomarkers - metabolism
Brain research
Central nervous system diseases
coenzyme A
Coenzyme A - metabolism
Defects
Dopamine
Dopamine - metabolism
Drug development
Dystonia
EMBO08
EMBO27
Gene expression
Genotype
Homeostasis
Iron
Iron - metabolism
Kinases
Mammals
Metabolism
Mice
Mitochondria
Movement disorders
Mutation
NBIA
Neurodegeneration
Oxidative phosphorylation
PANK2
Pantetheine - analogs & derivatives
Pantetheine - pharmacology
Pantetheine - therapeutic use
Pantothenate kinase
Pantothenate Kinase-Associated Neurodegeneration - drug therapy
Pantothenate Kinase-Associated Neurodegeneration - metabolism
Pathogenesis
Pharmacodynamics
Phosphorylation
Phosphotransferases (Alcohol Group Acceptor) - metabolism
PKAN
Pyruvic acid
Sulfur
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Title 4′‐Phosphopantetheine corrects CoA, iron, and dopamine metabolic defects in mammalian models of PKAN
URI https://link.springer.com/article/10.15252/emmm.201910489
https://onlinelibrary.wiley.com/doi/abs/10.15252%2Femmm.201910489
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Volume 11
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