Alterations in ROS Activity and Lysosomal pH Account for Distinct Patterns of Macroautophagy in LINCL and JNCL Fibroblasts

PLoS ONE

Volumn 8, Issue 2, 2013, Pages

  Vidal Donet, José Manuel ^a   Cárcel Trullols, Jaime ^a   Casanova, Bonaventura ^c   Aguado, Carmen ^a,b   Knecht, Erwin ^a,b  

^a CENTRO DE INVESTIGACIÓN PRÍNCIPE FELIPE   (Spain)

^b CENTRO DE INVESTIGACIÓN BIOMÉDICA EN RED DE ENFERMEDADES RARAS CIBERER   (Spain)

^c HOSPITAL UNIVERSITARIO LA FE   (Spain)

Author keywords

[No Author keywords available]

Indexed keywords

CATALASE; DIPEPTIDYL PEPTIDASE I; LIPOFUSCIN; MAMMALIAN TARGET OF RAPAMYCIN; MEMBRANE PROTEIN; MITOGEN ACTIVATED PROTEIN KINASE 1; MITOGEN ACTIVATED PROTEIN KINASE 14; PROTEIN CLN3P; PROTEIN KINASE B; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATASE; REACTIVE OXYGEN METABOLITE; UNCLASSIFIED DRUG;

ADOLESCENT; ARTICLE; AUTOPHAGOSOME; BIOACCUMULATION; CASE REPORT; CELL DAMAGE; CELL MATURATION; CELL PH; CHILD; CLN3 GENE; CONTROLLED STUDY; DISEASE SEVERITY; ENDOCYTOSIS; ENZYME ACTIVITY; ENZYME DEFECT; FEMALE; FIBROBLAST; GENE; GENE MUTATION; HUMAN; HUMAN CELL; INTRACELLULAR SIGNALING; LIGHT CHAIN; MACROAUTOPHAGY; MALE; NEURONAL CEROID LIPOFUSCINOSIS; PRESCHOOL CHILD; PROTEIN CLEAVAGE; PROTEIN DEGRADATION; SCHOOL CHILD; TPP1 GENE;

AUTOPHAGY; CATALASE; CELLS, CULTURED; FIBROBLASTS; HUMANS; HYDROGEN-ION CONCENTRATION; LYSOSOMES; NEURONAL CEROID-LIPOFUSCINOSES; OXIDATIVE STRESS; REACTIVE OXYGEN SPECIES;

EID: 84873578783     PISSN: None     EISSN: 19326203     Source Type: Journal    
DOI: 10.1371/journal.pone.0055526     Document Type: Article

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