http://www.cnr.it/ontology/cnr/individuo/prodotto/ID12582
Different early ER-stress responses in the CLN8mnd mouse model of neuronal ceroid lipofuscinosis. (Articolo in rivista)
- Type
- Label
- Different early ER-stress responses in the CLN8mnd mouse model of neuronal ceroid lipofuscinosis. (Articolo in rivista) (literal)
- Anno
- 2011-01-01T00:00:00+01:00 (literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#doi
- 10.1016/j.neulet.2010.11.041 (literal)
- Alternative label
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
- Galizzi Giacoma; Russo Domenica; Deidda Irene; Cascio Caterina; Passantino Rosa; Guarneri Rosetta; Bigini Paolo; Mennini Tiziana; Drago Gaspare; Guarneri Patrizia. (literal)
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- Rivista
- Note
- ISI Web of Science (WOS) (literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
- CNR-Istituto di biomedicina e di immunologia molecolare \"Alberto Monroy\"; Mario Negri Institute for Pharmacological Research. (literal)
- Titolo
- Different early ER-stress responses in the CLN8mnd mouse model of neuronal ceroid lipofuscinosis. (literal)
- Abstract
- Neuronal ceroid lipofuscinoses (NCLs) are a group of inherited neurodegenerative disorders characterized
by epilepsy, progressive motor and cognitive decline, blindness, and by the accumulation of autofluorescent
lipopigment. Late-infantile onset forms (LINCL) include those linked to mutations in CLN8 gene,
encoding a transmembrane protein at the endoplasmic reticulum (ER). In the motor neuron degeneration
(mnd) mouse model of the CLN8-LINCL (CLN8mnd), we carried out an analysis of ER stress-related
molecules in CNS structures that exhibit a variable rate of disease progression (early retinal degeneration
and delayed brain and motoneuron dysfunction). At the presymptomatic state of 1-month-old CLN8mnd
mice, we found an upregulation of GRP78 and activation of the transcription factor-6 (ATF6) in all structures
examined, an activation of a CHOP-dependent pathway in the cerebellum, hippocampus and retina,
a caspase-12-dependent pathway in the retina and no activation of these two pathways in the cerebral
cortex and spinal cord. An increased CHOP expression was detected in the cortex and spinal cord at the
early symptomatic state (4 months). Caspase-3 cleavage occurred presymptomatically in the cerebellum,
hippocampus and retina, and symptomatically in the cerebral cortex and spinal cord. We also monitored
activation of NF-?B, which is engaged in the alarming phase of ER stress, together with increased levels
of TRAF2, TNF-? and TNFR1, and no activation of ASK-1/JNK signalling pathway, all over mnd structures.
The results suggest that early ER-stress responses distinctly combined and ER-stress pathways integrated
with inflammatory responses may contribute to the progression of the CLN8mnd disease in CNS structures. (literal)
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