Tissue-specific variation in nonsense mutant transcript level and drug-induced read-through efficiency in the Cln1R151X mouse model of INCL

Journal of Cellular and Molecular Medicine

Volumn 20, Issue 2, 2016, Pages 381-385

  Thada, Vaughn ^a,b   Miller, Jake N ^b,c   Kovács, Attila D ^b   Pearce, David A ^b,c  

^a AUGUSTANA COLLEGE   (United States)

^b SANFORD RESEARCH   (United States)

^c UNIVERSITY OF SOUTH DAKOTA   (United States)

Author keywords

Cln1R151X; Ataluren; Infantile neuronal ceroid lipofuscinosis; Nonsense mutation; Read through drug

Indexed keywords

ATALUREN; MESSENGER RNA; PALMITOYL PROTEIN THIOESTERASE; PALMITOYL PROTEIN THIOESTERASE 1; UNCLASSIFIED DRUG; PALMITOYL-PROTEIN THIOESTERASE; STOP CODON; THIOL ESTER HYDROLASE;

ANALYTICAL PARAMETERS; ANIMAL EXPERIMENT; ANIMAL TISSUE; ARTICLE; BRAIN CORTEX; CONTROLLED STUDY; DNA TRANSCRIPTION; DRUG ACTIVITY; ENZYME ACTIVITY; MALE; MOUSE; NEURONAL CEROID LIPOFUSCINOSIS; NONHUMAN; NONSENSE MUTATION; PRIORITY JOURNAL; PROTEIN EXPRESSION; PROTEIN ISOLATION; READ THROUGH EFFICIENCY; REAL TIME POLYMERASE CHAIN REACTION; REVERSE TRANSCRIPTION POLYMERASE CHAIN REACTION; RNA EXTRACTION; TESTIS; TISSUE SPECIFIC VARIATION; ANIMAL; C57BL MOUSE; DISEASE MODEL; GENETICS; STOP CODON;

ANIMALS; CODON, NONSENSE; DISEASE MODELS, ANIMAL; MALE; MICE; MICE, INBRED C57BL; NEURONAL CEROID-LIPOFUSCINOSES; RNA, MESSENGER; THIOLESTER HYDROLASES;

EID: 84955676977     PISSN: 15821838     EISSN: None     Source Type: Journal    
DOI: 10.1111/jcmm.12744     Document Type: Article

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