Late-onset Parkinsonism in NF B/c-Rel-deficient mice

Baiguera, Cristina; Alghisi, Manuela; Pinna, Annalisa; Bellucci, Arianna; Luca, Maria Antonietta De; Frau, Lucia; Morelli, Micaela; Ingrassia, Rosaria; Benarese, Marina; Porrini, Vanessa; Pellitteri, Michele; Bertini, Giuseppe; Fabene, Paolo F.; Sigala, Sandra; Spillantini, Maria Grazia; Liou, Hsiou Chi; Spano, PierFranco; Pizzi, Marina

doi:10.1093/brain/aws193

Cited by 73 publications

(96 citation statements)

References 61 publications

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“…834 chronic neurodegenerative conditions, including severe epileptic seizures , AD (Barger et al, 1995), and HD (Yu et al, 2000). Mice lacking the p65 NF-kB subunit develop a PD-like disease characterized by degeneration of dopaminergic neurons and motor dysfunction as they age, suggesting a critical role for NF-kB in neuronal maintenance during aging (Baiguera et al, 2012). Activation of NF-kB induces the expression of SOD2, which protects mitochondria under conditions of oxidative and metabolic stress (Mattson et al, 1997).…”

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confidence: 99%

Adaptive Cellular Stress Pathways as Therapeutic Targets of Dietary Phytochemicals: Focus on the Nervous System

et al. 2014

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mentioning

confidence: 99%

Adaptive Cellular Stress Pathways as Therapeutic Targets of Dietary Phytochemicals: Focus on the Nervous System

et al. 2014

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“…In systems that detect floor movement, a homecage can be placed on a platform that measures movement allowing specific behaviours including rearing and grooming to be detected Benkhelifa-Ziyyat et al, 2013). Automated video analysis techniques used pattern recognition software to detect specific mouse behaviours including rearing, sniffing and hanging (Steele et al, 2007b;Baiguera et al, 2012;Mochel et al, 2012). RFID-based homecage systems typically focus on identifying the animals' location and movement (Lewejohann et al, 2009;Howerton et al, 2012), can be paired with other monitoring equipment such as lickometers (Krackow et al, 2010;Voikar et al, 2010;Bellmann-Sickert et al, 2011) and some systems can be programmed to respond differentially to each mouse and present stimuli such as mild aversive airpuffs (Rudenko et al, 2009).…”

Section: What Are Automated Behavioural Homecage Technologies?mentioning

confidence: 99%

“…Huntington's disease is characterized by a progressive decline in cognitive and motor functions and patients frequently suffer from sleep disturbances and affective disorders (Rudenko et al, 2009;Mochel et al, 2011;Balci et al, 2013). Other neurodegenerative disease that have been behaviourally characterized using automated technologies include mouse models of prion disease (Steele et al, 2007a(Steele et al, ,b,c, 2008, Parkinson's disease (Baiguera et al, 2012;Paumier et al, 2013), Alzheimer's disease (Lewejohann et al, 2009;Codita et al, 2010) and meningitis (Too et al, 2014). Cognitive impairments and affective disorders occur in all of these neurodegenerative diseases.…”

Section: Assessment Of Disease Progression Using Automated Behaviouramentioning

confidence: 99%

The power of automated behavioural homecage technologies in characterizing disease progression in laboratory mice: A review

Richardson

2015

Applied Animal Behaviour Science

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a b s t r a c tBehavioural changes that occur as animals become sick have been characterized in a number of species and include the less frequent occurrence of 'luxury behaviours' such as playing, grooming and socialization. 'Sickness behaviours' or behavioural changes following exposure to infectious agents, have been particularly well described; animals are typically less active, sleep more, exhibit postural changes and consume less food/water. Disease is frequently induced in laboratory mice to model pathophysiological processes and investigate potential therapies but despite what is known about behavioural changes as animals become sick, behavioural phenotyping of mice involved in disease studies is relatively rare. A detailed understanding of how behaviour changes as mice get sick could be applied to improve welfare of laboratory mice and support the underlying biomedical research. Specifically, characterizing behavioural changes in ill health could help those working with laboratory mice to recognize when refinements should be introduced, when severity limits are being approached and when humane endpoints should be implemented. Understanding how behaviour changes with illness may also help to identify compounds that have a clinical effect as well as when these agents act. There are an increasing number of automated systems to monitor the behaviour of laboratory mice in their homecages incorporating technologies such as the quantification of cage movement, automated video analysis and radiofrequency identification transponders/readers. Mouse models of neurodegenerative diseases particularly Huntington's disease have been well characterized using these systems and behavioural biomarkers of pathology, including changes in the animals' use of environmental enrichment, changes in food/water consumption and alterations in circadian rhythms, are now monitored by laboratories worldwide and used to refine studies and develop therapies. In contrast, automated behavioural technologies have not been used to characterize the behaviour of mice with systemic diseases such as cancer and liver disease. In this review, common behavioural changes that occur in animals with declining health will be discussed with an emphasis on progressive disease studies involving mice. Automated homecage behaviour recording technologies will then be summarized, studies in which these systems have been used to characterize the behaviour of mice with progressive diseases will be reviewed and the potential to apply automated technologies to refine disease studies involving mice will be discussed.

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“…Mice deficient of the c-Rel factor exhibits a marked immunoreactivity for fibrillary α-synuclein in the SNpc, as well as increased expression of divalent metal transporter 1 and iron staining in both SNpc and striatum [104]. Aged c-rel( -/-) mouse brain is characterized by increased microglial reactivity in the basal ganglia.…”

Section: Nfκb/c-rel-deficient Micementioning

confidence: 99%

“…Aged c-rel( -/-) mouse brain is characterized by increased microglial reactivity in the basal ganglia. In addition, c-rel( -/-) mice show agedependent deficits in locomotor and total activity, and various gait-related deficits during a catwalk analysis that is reminiscent of bradykinesia and muscle rigidity [104].…”

Section: Nfκb/c-rel-deficient Micementioning

confidence: 99%

Animal Models of Parkinson's Disease: A Gateway to Therapeutics?

Sayana

Jankovic

2014

Neurotherapeutics

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Parkinson's disease (PD) is a progressive, neurodegenerative disorder of unknown etiology, although a complex interaction between environmental and genetic factors has been implicated as a pathogenic mechanism of selected neuronal loss. A better understanding of the etiology, pathogenesis, and molecular mechanisms underlying the disease process may be gained from research on animal models. While cell and tissue models are helpful in unraveling involved molecular pathways, animal models are much better suited to study the pathogenesis and potential treatment strategies. The animal models most relevant to PD include those generated by neurotoxic chemicals that selectively disrupt the catecholaminergic system such as 6-hydroxydopamine; 1-methyl-1,2,3,6-tetrahydropiridine; agricultural pesticide toxins, such as rotenone and paraquat; the ubiquitin proteasome system inhibitors; inflammatory modulators; and several genetically manipulated models, such as α-synuclein, DJ-1, PINK1, Parkin, and leucine-rich repeat kinase 2 transgenic or knock-out animals. Genetic and nongenetic animal models have their own unique advantages and limitations, which must be considered when they are employed in the study of pathogenesis or treatment approaches. This review provides a summary and a critical review of our current knowledge about various in vivo models of PD used to test novel therapeutic strategies.

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Late-onset Parkinsonism in NF B/c-Rel-deficient mice

Cited by 73 publications

References 61 publications

Adaptive Cellular Stress Pathways as Therapeutic Targets of Dietary Phytochemicals: Focus on the Nervous System

Adaptive Cellular Stress Pathways as Therapeutic Targets of Dietary Phytochemicals: Focus on the Nervous System

The power of automated behavioural homecage technologies in characterizing disease progression in laboratory mice: A review

Animal Models of Parkinson's Disease: A Gateway to Therapeutics?

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