Glial fibrillary acidic protein is elevated in the lysosomal storage disease classical late-infantile neuronal ceroid lipofuscinosis, but is not a component of the storage material

Biochemical Journal

Volumn 428, Issue 3, 2010, Pages 355-362

  Xu, Su ^a   Sleat, David E ^a   Jadot, Michel ^b   Lobel, Peter ^a  

^a RUTGERS UNIVERSITY   (United States)

^b UNIVERSITY OF NAMUR   (Belgium)

Author keywords

Classical late infantile neuronal ceroid lipofuscinosis (LINCL); Glial fibrillary acidic protein (GFAP); Lysosome; Mass spectrometry; Storage material

Indexed keywords

ATP SYNTHASE; CEROID LIPOFUSCINOSIS; GLIAL FIBRILLARY ACIDIC PROTEIN (GFAP); GLIAL FIBRILLARY ACIDIC PROTEINS; INTRACELLULAR DISTRIBUTION; MOUSE MODELS; NATURAL SUBSTRATES; NEURODEGENERATIVE DISEASE; NORMAL CONTROLS; PATHOPHYSIOLOGY; PROTEIN COMPONENTS; PROTEOMIC ANALYSIS; SUBCELLULAR FRACTIONATION; SUBCELLULAR FRACTIONS;

GENE ENCODING; MASS SPECTROMETRY; PEDIATRICS; PROTEINS; SUBSTRATES;

MATERIALS;

GLIAL FIBRILLARY ACIDIC PROTEIN; AMINOPEPTIDASE; DIPEPTIDYL PEPTIDASE; SERINE PROTEINASE; TRIPEPTIDYL PEPTIDASE I; TRIPEPTIDYL-PEPTIDASE 1;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; CELL FRACTIONATION; CELLULAR DISTRIBUTION; CONTROLLED STUDY; LYSOSOME STORAGE DISEASE; MORPHOLOGY; MOUSE; NEURONAL CEROID LIPOFUSCINOSIS; NONHUMAN; PRIORITY JOURNAL; PROTEOMICS; ANIMAL; CONFOCAL MICROSCOPY; GENETICS; LYSOSOME; METABOLISM;

MUS;

AMINOPEPTIDASES; ANIMALS; DIPEPTIDYL-PEPTIDASES AND TRIPEPTIDYL-PEPTIDASES; GLIAL FIBRILLARY ACIDIC PROTEIN; LYSOSOMAL STORAGE DISEASES; LYSOSOMES; MICE; MICROSCOPY, CONFOCAL; MODELS, ANIMAL; NEURONAL CEROID-LIPOFUSCINOSES; SERINE PROTEASES;

EID: 77954943244     PISSN: 02646021     EISSN: 14708728     Source Type: Journal    
DOI: 10.1042/BJ20100128     Document Type: Article

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