A zebrafish model of CLN2 disease is deficient in tripeptidyl peptidase 1 and displays progressive neurodegeneration accompanied by a reduction in proliferation

Brain

Volumn 136, Issue 5, 2013, Pages 1488-1507

  Mahmood, Fahad ^a   Fu, Sonia ^b   Cooke, Jennifer ^a   Wilson, Stephen W ^b   Cooper, Jonathan D ^c   Russell, Claire ^a  

^a ROYAL VETERINARY COLLEGE   (United Kingdom)

^b UNIVERSITY COLLEGE LONDON   (United Kingdom)

^c KING'S COLLEGE LONDON   (United Kingdom)

Author keywords

CLN2 disease; late infantile neuronal ceroid lipofuscinosis; lysosomal storage disorder; model; TPP1; tripeptidyl peptidase 1; zebrafish

Indexed keywords

COMPLEMENTARY DNA; PEPTIDASE; PROTON TRANSPORTING ADENOSINE TRIPHOSPHATE SYNTHASE; TRIPEPTIDYL PEPTIDASE I; UNCLASSIFIED DRUG;

ALLELE; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANTERIOR COMMISSURE; APOPTOSIS; ARTICLE; AUTOSOMAL RECESSIVE INHERITANCE; BASAL GANGLION; BRAIN COMMISSURE; CELL PROLIFERATION; CEREBELLUM; EXON; FOREBRAIN; GENOTYPE; GENOTYPE PHENOTYPE CORRELATION; HIGH THROUGHPUT SCREENING; LYSOSOME; MOTOR DYSFUNCTION; NERVE CELL; NERVE DEGENERATION; NERVE FIBER; NERVOUS SYSTEM DEVELOPMENT; NEURONAL CEROID LIPOFUSCINOSIS; NONHUMAN; OPTIC NERVE; PHENOTYPE; PRIORITY JOURNAL; RETINA; SEIZURE; SPINAL CORD; STOP CODON; SUPERIOR COLLICULUS; THALAMUS; TROCHLEAR NERVE; WESTERN BLOTTING; WHITE MATTER; ZEBRA FISH;

EID: 84877285307     PISSN: 00068950     EISSN: 14602156     Source Type: Journal    
DOI: 10.1093/brain/awt043     Document Type: Article

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