Progressive neurologic and somatic disease in a novel mouse model of human mucopolysaccharidosis type IIIC

DMM Disease Models and Mechanisms

Volumn 9, Issue 9, 2016, Pages 999-1013

  Marcò, Sara ^a   Pujol, Anna ^a,b   Roca, Carles ^a,b   Motas, Sandra ^a   Ribera, Albert ^a,b   Garcia, Miguel ^a,b   Molas, Maria ^a,b   Villacampa, Pilar ^a,b   Melia, Cristian S ^a   Sánchez, Víctor ^a   Sanchez, Xavier ^a   Bertolin, Joan ^a   Ruberte, Jesús ^a,b   Haurigot, Virginia ^a,b   Bosch, Fatima ^a,b  

^a UNIVERSITAT AUTÒNOMA DE BARCELONA   (Spain)

^b INSTITUTO DE SALUD CARLOS III   (Spain)

Author keywords

Animal model; HGSNAT; Lysosomal storage disease; MPSIIIC; Neurodegeneration

Indexed keywords

ACYLTRANSFERASE; ALPHA N ACETYLGLUCOSAMINIDASE; BETA GALACTOSIDASE; BETA GLUCURONIDASE; BETA N ACETYLHEXOSAMINIDASE; GLYCOSAMINOGLYCAN; HEPARAN ALPHA GLUCOSAMINIDE N ACETYLTRANSFERASE; IDURONATE 2 SULFATASE; LYSOSOME ENZYME; N ACETYLGALACTOSAMINE 6 SULFATASE; N SULFOGLUCOSAMINE SULFOHYDROLASE; UNCLASSIFIED DRUG;

AGE; ANIMAL BEHAVIOR; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; BEHAVIOR CHANGE; BEHAVIOR DISORDER; CELL ULTRASTRUCTURE; CENTRAL NERVOUS SYSTEM; CONTROLLED STUDY; DISEASE COURSE; DISEASE SEVERITY; EXPLORATORY BEHAVIOR; FEMALE; GENE DISRUPTION; GENE EXPRESSION; GENE TARGETING; LIFESPAN; LIVER; LOCOMOTION; LYSOSOME; MALE; MOUSE; MUCOPOLYSACCHARIDOSIS TYPE IIIC; NERVOUS SYSTEM INFLAMMATION; NEUROLOGIC DISEASE; NEUROPATHOLOGY; NONHUMAN; PHYSICAL DISEASE BY ANATOMICAL STRUCTURE; PRIORITY JOURNAL; PROMOTER REGION; SANFILIPPO SYNDROME; SOMATIC DISEASE; ANIMAL; ANTIBODY SPECIFICITY; BRAIN; C57BL MOUSE; DEFICIENCY; DISEASE EXACERBATION; DISEASE MODEL; ENZYMOLOGY; HOMEOSTASIS; HUMAN; INFLAMMATION; KNOCKOUT MOUSE; LONGEVITY; METABOLISM; MICROGLIA; PATHOLOGY; SURVIVAL ANALYSIS; ULTRASTRUCTURE;

ACETYLTRANSFERASES; ANIMALS; BEHAVIOR, ANIMAL; BRAIN; DISEASE MODELS, ANIMAL; DISEASE PROGRESSION; GLYCOSAMINOGLYCANS; HOMEOSTASIS; HUMANS; INFLAMMATION; LONGEVITY; LYSOSOMES; MALE; MICE; MICE, INBRED C57BL; MICE, KNOCKOUT; MICROGLIA; MUCOPOLYSACCHARIDOSIS III; ORGAN SPECIFICITY; SURVIVAL ANALYSIS;

EID: 84987624553     PISSN: 17548403     EISSN: 17548411     Source Type: Journal    
DOI: 10.1242/dmm.025171     Document Type: Article

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