Granulin knock out zebrafish lack frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis pathology.
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ABSTRACT:
SUBMITTER: Solchenberger B
PROVIDER: S-EPMC4365039 | biostudies-literature | 2015
REPOSITORIES: biostudies-literature
Publications
Granulin knock out zebrafish lack frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis pathology.
Solchenberger Barbara B Russell Claire C Kremmer Elisabeth E Haass Christian C Schmid Bettina B
PloS one 20150318 3
Loss of function mutations in granulin (GRN) are linked to two distinct neurological disorders, frontotemporal lobar degeneration (FTLD) and neuronal ceroid lipofuscinosis (NCL). It is so far unknown how a complete loss of GRN in NCL and partial loss of GRN in FTLD can result in such distinct diseases. In zebrafish, there are two GRN homologues, Granulin A (Grna) and Granulin B (Grnb). We have generated stable Grna and Grnb loss of function zebrafish mutants by zinc finger nuclease mediated geno ...[more]
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