Articles with "lipofuscinosis" as a keyword



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Conditional loss of progranulin in neurons is not sufficient to cause neuronal ceroid lipofuscinosis-like neuropathology in mice

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Published in 2017 at "Neurobiology of Disease"

DOI: 10.1016/j.nbd.2017.06.012

Abstract: Progranulin deficiency due to heterozygous null mutations in the GRN gene is a common cause of familial frontotemporal lobar degeneration (FTLD), while homozygous loss-of-function GRN mutations cause neuronal ceroid lipofuscinosis (NCL). Aged progranulin-knockout mice display… read more here.

Keywords: cause; progranulin; lipofuscinosis; nes cko ... See more keywords
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Selective depletion of microglial progranulin in mice is not sufficient to cause neuronal ceroid lipofuscinosis or neuroinflammation

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Published in 2017 at "Journal of Neuroinflammation"

DOI: 10.1186/s12974-017-1000-9

Abstract: BackgroundProgranulin deficiency due to heterozygous null mutations in the GRN gene are a common cause of familial frontotemporal lobar degeneration (FTLD), while homozygous loss-of-function GRN mutations are thought to be a rare cause of neuronal… read more here.

Keywords: cause; progranulin; lipofuscinosis; expression ... See more keywords