Peroxisomal multifunctional protein-2 deficiency causes neuroinflammation and degeneration of Purkinje cells independent of very long chain fatty acid accumulation

Neurobiology of Disease

Volumn 58, Issue , 2013, Pages 258-269

  Verheijden, Simon ^a   Bottelbergs, Astrid ^a   Krysko, Olga ^b   Krysko, Dmitri V ^c,f   Beckers, Lien ^a   De Munter, Stephanie ^a   Van Veldhoven, Paul P ^d   Wyns, Sabine ^a,e   Kulik, Wim ^c,g   Nave, Klaus Armin ^a   Ramer, Matt S ^a,e   Carmeliet, Peter ^a,e   Kassmann, Celia M ^a   Baes, Myriam ^a  

^a UNIVERSITY OF LEUVEN   (Belgium)

^b GHENT UNIVERSITY HOSPITAL   (Belgium)

^c DEPARTMENT OF PLANT SYSTEMS BIOLOGY   (Belgium)

^d GHENT UNIVERSITY   (Belgium)

^e UNIVERSITY OF AMSTERDAM   (Netherlands)

^f MAX PLANCK INSTITUTE OF EXPERIMENTAL MEDICINE   (Germany)

^g UNIVERSITY OF BRITISH COLUMBIA   (Canada)

Author keywords

Cerebellum; D bifunctional protein deficiency; HSD17B4; Microglia; Mouse model; Multifunctional protein 2; Neuroinflammation; Oligodendrocyte; Peroxisomal oxidation; Purkinje cell; X linked adrenoleukodystrophy

Indexed keywords

NESTIN; PEROXISOMAL MULTIFUNCTIONAL PROTEIN 2; PROTEIN; UNCLASSIFIED DRUG; VERY LONG CHAIN FATTY ACID;

ANIMAL EXPERIMENT; ARTICLE; ATAXIA; CEREBELLUM; CEREBELLUM DEGENERATION; CONTROLLED STUDY; DEGENERATIVE DISEASE; DISORDERS OF PEROXISOMAL FUNCTIONS; FATTY ACID OXIDATION; INFLAMMATION; MALE; MOTOR DYSFUNCTION; MOTOR PERFORMANCE; MOUSE; NERVOUS SYSTEM INFLAMMATION; NONHUMAN; OLIGODENDROGLIA; PEROXISOMAL MULTIFUNCTIONAL PROTEIN 2 DEFICIENCY; PRIORITY JOURNAL; PROTEIN DEFICIENCY; PURKINJE CELL;

17Β-HYDROXYSTEROID DEHYDROGENASE TYPE 4 (ALSO CALLED DBP OR MFP2); ADRENOMYELONEUROPATHY; AMN; CALD; CENTRAL NERVOUS SYSTEM; CEREBELLUM; CEREBRAL ADRENOLEUKODYSTROPHY; CNS; D-BIFUNCTIONAL PROTEIN (ALSO CALLED MFP2 AND HSD17B4); D-BIFUNCTIONAL PROTEIN DEFICIENCY; DBP; H&E; HEMATOXYLIN & EOSIN; HSD17B4; MFP2; MICROGLIA; MOUSE MODEL; MULTIFUNCTIONAL PROTEIN-2; NEUROINFLAMMATION; OLIGODENDROCYTE; PBD; PEROXISOMAL Β-OXIDATION; PEROXISOME BIOGENESIS DISORDERS; PURKINJE CELL; VERY LONG CHAIN FATTY ACIDS; VLCFA; X-ALD; X-LINKED ADRENOLEUKODYSTROPHY;

2',3'-CYCLIC NUCLEOTIDE 3'-PHOSPHODIESTERASE; AGE FACTORS; ANIMALS; ANTIGENS, DIFFERENTIATION; BRAIN; CALCIUM-BINDING PROTEINS; CYTOKINES; DEFICIENCY DISEASES; ENCEPHALITIS; FATTY ACIDS; GAS CHROMATOGRAPHY-MASS SPECTROMETRY; GENE EXPRESSION REGULATION; LOCOMOTION; MICE; MICE, TRANSGENIC; MICROFILAMENT PROTEINS; MYELIN BASIC PROTEIN; NERVE DEGENERATION; NESTIN; PEROXISOMAL MULTIFUNCTIONAL PROTEIN-2; PURKINJE CELLS;

EID: 84880422343     PISSN: 09699961     EISSN: 1095953X     Source Type: Journal    
DOI: 10.1016/j.nbd.2013.06.006     Document Type: Article

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