Behavioral deficits, early gliosis, dysmyelination and synaptic dysfunction in a mouse model of mucolipidosis IV

Acta Neuropathologica Communications

Volumn 2, Issue 1, 2014, Pages

  Grishchuk, Yulia ^a   Sri, Sarmi ^b   Rudinskiy, Nikita ^a   Ma, Weiyuan ^a   Stember, Katherine G ^a   Cottle, Matthew W ^a   Sapp, Ellen ^a   Difiglia, Marian ^a   Muzikansky, Alona ^c   Betensky, Rebecca A ^c   Wong, Andrew M S ^b   Bacskai, Brian J ^a   Hyman, Bradley T ^a   Kelleher, Raymond J ^a   Cooper, Jonathan D ^b   Slaugenhaupt, Susan A ^a  

^a MASSACHUSETTS GENERAL HOSPITAL   (United States)

^b KING'S COLLEGE LONDON   (United Kingdom)

^c HARVARD SCHOOL OF PUBLIC HEALTH   (United States)

Author keywords

Glia; In vivo Ca2+ imaging; Lysosomal storage disease; Mucolipidosis IV; Neuropathology

Indexed keywords

MCOLN1 PROTEIN, MOUSE; TRANSIENT RECEPTOR POTENTIAL CHANNEL;

ANIMAL; ASTROCYTE; BRAIN; DISEASE MODEL; EXPLORATORY BEHAVIOR; GENETICS; GLIOSIS; KNOCKOUT MOUSE; MALE; MICROGLIA; MOTOR ACTIVITY; MOUSE; MUCOLIPIDOSIS; MYELIN SHEATH; NERVE CELL; NERVE CELL PLASTICITY; PATHOLOGY; PATHOPHYSIOLOGY; PHYSIOLOGY;

ANIMALS; ASTROCYTES; BRAIN; DISEASE MODELS, ANIMAL; EXPLORATORY BEHAVIOR; GLIOSIS; MALE; MICE; MICE, KNOCKOUT; MICROGLIA; MOTOR ACTIVITY; MUCOLIPIDOSES; MYELIN SHEATH; NEURONAL PLASTICITY; NEURONS; TRANSIENT RECEPTOR POTENTIAL CHANNELS;

EID: 84965087966     PISSN: None     EISSN: 20515960     Source Type: Journal    
DOI: 10.1186/s40478-014-0133-7     Document Type: Review

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