Distinct early molecular responses to mutations causing vLINCL and JNCL presage ATP synthase subunit c accumulation in cerebellar cells

PLoS ONE

Volumn 6, Issue 2, 2011, Pages

  Cao, Yi ^a   Staropoli, John F ^a   Biswas, Sunita ^a   Espinola, Janice A ^a   MacDonald, Marcy E ^a   Lee, Jong Min ^a   Cotman, Susan L ^a  

^a MASSACHUSETTS GENERAL HOSPITAL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CELL PROTEIN; PROTEIN CLN3; PROTEIN CLN6; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATE SYNTHASE; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATE SYNTHASE C; UNCLASSIFIED DRUG; CHAPERONE; CLN3 PROTEIN, HUMAN; CLN6 PROTEIN, HUMAN; MEMBRANE PROTEIN; PROTEIN SUBUNIT;

ANIMAL CELL; ARTICLE; CELL AGING; CELL LABELING; CELL SURVIVAL; CELL VACUOLE; CELLULAR DISTRIBUTION; CONTROLLED STUDY; DISEASE CLASSIFICATION; ENDOCYTOSIS; ENDOPLASMIC RETICULUM; GENE EXPRESSION PROFILING; GENE MUTATION; GENETIC VARIABILITY; HOMOZYGOSITY; HUMAN; HUMAN CELL; IMMUNOHISTOCHEMISTRY; LYSOSOME; MALE; MOUSE; NEURONAL CEROID LIPOFUSCINOSIS; NONHUMAN; PHENOTYPIC VARIATION; PROTEIN FUNCTION; PROTEIN METABOLISM; STRUCTURAL HOMOLOGY; WILD TYPE; ANIMAL; C57BL MOUSE; CELL CULTURE; CEREBELLUM; CLUSTER ANALYSIS; GENETICS; METABOLISM; MICROARRAY ANALYSIS; MUTATION; NERVE CELL; PATHOLOGY; PHYSIOLOGY; SIGNAL TRANSDUCTION; TIME; TRANSGENIC MOUSE;

MURINAE;

ANIMALS; CELL SURVIVAL; CELLS, CULTURED; CEREBELLUM; CLUSTER ANALYSIS; GENE EXPRESSION PROFILING; HUMANS; MEMBRANE GLYCOPROTEINS; MEMBRANE PROTEINS; MICE; MICE, INBRED C57BL; MICE, TRANSGENIC; MICROARRAY ANALYSIS; MITOCHONDRIAL PROTON-TRANSLOCATING ATPASES; MOLECULAR CHAPERONES; MUTATION; NEURONAL CEROID-LIPOFUSCINOSES; NEURONS; PROTEIN SUBUNITS; SIGNAL TRANSDUCTION; TIME FACTORS;

EID: 79951993462     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0017118     Document Type: Article

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