Pantethine treatment is effective in recovering the disease phenotype induced by ketogenic diet in a pantothenate kinase-associated neurodegeneration mouse model

Brain

Volumn 137, Issue 1, 2014, Pages 57-68

  Brunetti, Dario ^a   Dusi, Sabrina ^a   Giordano, Carla ^b   Lamperti, Costanza ^a   Morbin, Michela ^a   Fugnanesi, Valeria ^a   Marchet, Silvia ^a   Fagiolari, Gigliola ^c   Sibon, Ody ^d   Moggio, Maurizio ^c   D'Amati, Giulia ^b   Tiranti, Valeria ^a  

^a DEPARTMENT OF NEUROSURGERY   (Italy)

^b SAPIENZA UNIVERSITY OF ROME   (Italy)

^c FONDAZIONE IRCCS CA GRANDA OSPEDALE MAGGIORE POLICLINICO   (Italy)

^d UNIVERSITY MEDICAL CENTER GRONINGEN   (Netherlands)

Author keywords

ketogenic diet; mitochondria; pantethine; pantothenate kinase associated neurodegeneration (PKAN)

Indexed keywords

COENZYME A; GLUCOSE; LIPID; PANTETHINE; PANTOTHENATE KINASE 2;

ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BODY WEIGHT; CENTRAL NERVOUS SYSTEM; CONTROLLED STUDY; CONVALESCENCE; FATTY ACID OXIDATION; FEMALE; HUMAN; HUMAN TISSUE; IMMUNOHISTOCHEMISTRY; KETOGENIC DIET; LIPID DIET; LIPID METABOLISM; MALE; MITOCHONDRIAL MEMBRANE POTENTIAL; MITOCHONDRIAL RESPIRATION; MITOCHONDRION; MOTOR ACTIVITY; MOTOR DYSFUNCTION; MOTOR PERFORMANCE; MOUSE; MUSCLE TISSUE; NERVE DEGENERATION; NEURODEGENERATION WITH BRAIN IRON ACCUMULATION; NONHUMAN; PERIPHERAL NERVOUS SYSTEM; PHENOTYPE; PRIORITY JOURNAL; SCIATIC NERVE;

KETOGENIC DIET; MITOCHONDRIA; PANTETHINE; PANTOTHENATE KINASE-ASSOCIATED NEURODEGENERATION (PKAN);

ANIMALS; BEHAVIOR, ANIMAL; BRAIN; CHOLESTEROL; ENERGY METABOLISM; FEMALE; HEREDODEGENERATIVE DISORDERS, NERVOUS SYSTEM; IMMUNOHISTOCHEMISTRY; KETOGENIC DIET; MALE; MEMBRANE POTENTIAL, MITOCHONDRIAL; MICE; MICE, KNOCKOUT; MICROSCOPY, ELECTRON; MITOCHONDRIA; MOTOR SKILLS; NEURONS; PANTETHEINE; PERIPHERAL NERVOUS SYSTEM; PHENOTYPE; PHOSPHOTRANSFERASES (ALCOHOL GROUP ACCEPTOR); SCIATIC NERVE; TRIGLYCERIDES;

EID: 84892734388     PISSN: 00068950     EISSN: 14602156     Source Type: Journal    
DOI: 10.1093/brain/awt325     Document Type: Article

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