http://www.cnr.it/ontology/cnr/individuo/prodotto/ID183146
Retinal oxidation and apoptosis in the motor neuron degeneration mouse, a model of neuronal ceroid lipofuscinosis (Contributo in atti di convegno)
- Type
- Label
- Retinal oxidation and apoptosis in the motor neuron degeneration mouse, a model of neuronal ceroid lipofuscinosis (Contributo in atti di convegno) (literal)
- Anno
- 2004-01-01T00:00:00+01:00 (literal)
- Alternative label
Guarneri P. ; D'Agostino S.; Russo D. ; Cascio C. ; Bigini P. ; Mennini T. ; Guarneri R. (2004)
Retinal oxidation and apoptosis in the motor neuron degeneration mouse, a model of neuronal ceroid lipofuscinosis
in 4th FENS (Forum of European Neuroscience), Lisbona/Portogallo, 10-14 Luglio, 2004
(literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori
- Guarneri P. ; D'Agostino S.; Russo D. ; Cascio C. ; Bigini P. ; Mennini T. ; Guarneri R. (literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#altreInformazioni
- prodotto digitalizzato (literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#titoloVolume
- 4th Forum of European Neuroscience (literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni
- Istituto di Biomedicina e Immunologia Molecolare \"Alberto Monroy\". C N R - Palermo, Italy;
Lab. Rec. Pharmacol. - Mario Negri, Milano, Italy (literal)
- Titolo
- Retinal oxidation and apoptosis in the motor neuron degeneration mouse, a model of neuronal ceroid lipofuscinosis (literal)
- Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#isbn
- Abstract
- The neuronal ceroid lipofuscinoses (NCLs) are a group of autosomal
recessively inherited diseases resulting in severe and progressive
neurological disorders. Mutations in at least eight genes which encode
proteins with known or unknown functions have been identified, however the
neuropathogenic mechanisms remain still unclear. Retinal degeneration is a
clue in many forms of NCLs, including a murine NCL model, the motor
neuron degeneration (mnd) mouse bearing a mutation of CLN8 gene and
representing the counterpart of CLN8 form in humans, the Northern
Epilepsy. To gain insights into the mechanisms contributing to retinal
degeneration in NCL, we here investigated the oxidative state and apoptotic
cell death in retinas of mnd mouse and with regard to gender- and ageassociated
differences. A progressive increase in oxyradicals and lipid
peroxides as monitored by superoxide dismutase activity and TBARS levels
was reported in retinas of female and male mnd mice. Female mnd retinas
showed a higher oxidation and consistently exhibited prominent 4-hydroxy-
2-nonenal (4-HNE) -adducts staining in the inner plexiform and ganglion cell
(GCL) layers, and an advanced histopathologic profile. TUNEL staining
appeared in photoreceptor layer as early as 1 month of age, became more
intense also in the inner nuclear layer (INL) and GCL at 4 months, and at 8
months, when complete loss of photoreceptors and atrophy of retinal layers
had occurred, it was restricted to few nuclei in INL and GCL. Along with the
morphological signs, activation caspase-3 was found and its processing
turned out to be higher in females than males. Moreover, an increase in the
expression of Bcl-xl appeared to be consistent with the slow and progressive
nature of retinal cell demise. These results show involvement of oxidation
and apoptotic processes in mnd mouse retinopathy and highlight for the first
time sex-related differences in retinal vulnerability to oxidative stress and
damage. (literal)
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