Lysosomal dysfunction and impaired autophagy in a novel mouse model deficient for the lysosomal membrane protein Cln7

Human Molecular Genetics

Volumn 25, Issue 4, 2016, Pages 777-791

  Brandenstein, Laura ^a   Schweizer, Michaela ^a   Sedlacik, Jan ^a   Fiehler, Jens ^a   Storch, Stephan ^a  

^a UNIVERSITY MEDICAL CENTER HAMBURG EPPENDORF   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

CATHEPSIN B; CATHEPSIN D; CATHEPSIN X; CLN7 PROTEIN; MEMBRANE PROTEIN; PROTEIN AGGREGATE; SEQUESTOSOME 1; UNCLASSIFIED DRUG; CARRIER PROTEIN; LYSOSOME ASSOCIATED MEMBRANE PROTEIN; MFSD8 PROTEIN, HUMAN;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; ASTROCYTOSIS; AUTOPHAGY; BRAIN CORTEX; CELL INCLUSION; CEREBELLUM; CONTROLLED STUDY; EMBRYO; EXON; GENE DELETION; GENE DISRUPTION; GLIOSIS; IMMUNOHISTOCHEMISTRY; LYSOSOME MEMBRANE; LYSOSOME STORAGE DISEASE; MICROGLIA; MOUSE; MYOCLONUS; NERVE DEGENERATION; NEUROPATHOLOGY; NONHUMAN; OLFACTORY BULB; PRIORITY JOURNAL; PROTEIN EXPRESSION; PURKINJE CELL; ULTRASTRUCTURE; ANIMAL; BRAIN; C57BL MOUSE; DEFICIENCY; DISEASE MODEL; GENETICS; HUMAN; INTRACELLULAR MEMBRANE; KNOCKOUT MOUSE; LYSOSOME; METABOLISM; NERVE CELL; NEURONAL CEROID LIPOFUSCINOSIS; PATHOLOGY; PHYSIOLOGY;

ANIMALS; AUTOPHAGY; BRAIN; DISEASE MODELS, ANIMAL; HUMANS; INTRACELLULAR MEMBRANES; LYSOSOME-ASSOCIATED MEMBRANE GLYCOPROTEINS; LYSOSOMES; MEMBRANE PROTEINS; MEMBRANE TRANSPORT PROTEINS; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT; MICROGLIA; NEURONAL CEROID-LIPOFUSCINOSES; NEURONS; PURKINJE CELLS;

EID: 85017083937     PISSN: 09646906     EISSN: 14602083     Source Type: Journal    
DOI: 10.1093/hmg/ddv615     Document Type: Article

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