Subject collection: Neurodegenerative Disorders
- SUMOylation by SUMO2 is implicated in the degradation of misfolded ataxin-7 via RNF4 in SCA7 models
Summary: In spinocerebellar ataxia type 7, SUMOylation contributes to the clearance of mutant ATXN7. SUMO2-modified ATXN7 interacts with the SUMO-dependent ubiquitin ligase RNF4, which polyubiquitinates ATXN7, promoting its proteasomal degradation.
- A transgenic minipig model of Huntington's disease shows early signs of behavioral and molecular pathologies
Summary: Here, we show that a minipig model of Huntington's disease mimics human neurodegeneration and holds promise for future intervention studies. However, minipig peripheral blood mononuclear cells express no detectable mutant huntingtin, eliminating their use as monitoring tools.
- Correction of cognitive deficits in mouse models of Down syndrome by a pharmacological inhibitor of DYRK1A
Summary: Normalization of DYRK1A kinase activity can be achieved in three different mouse models of Down syndrome with pharmacological inhibitors of DYRK1A, leading to correction of recognition memory deficits.
- Immunomodulation with minocycline rescues retinal degeneration in juvenile neuronal ceroid lipofuscinosis mice highly susceptible to light damage
Summary: Here, we established a light-damage paradigm to model retinal degeneration in the juvenile neuronal ceroid lipofuscinosis mouse and showed the beneficial effects of minocycline on retinal pathology.
- Modeling Niemann-Pick disease type C1 in zebrafish: a robust platform for in vivo screening of candidate therapeutic compounds
Summary: A zebrafish genetic model of Niemann-Pick disease type C1 is suitable for performing in vivo screening of candidate therapeutic compounds by examining LysoTracker staining intensity in neuromasts.
- Identification of cardioprotective drugs by medium-scale in vivo pharmacological screening on a Drosophila cardiac model of Friedreich's ataxia
Editor's choice: The first medium-scale pharmacological in vivo screen on a Drosophila cardiac model identified 11 compounds with protective effects on Friedreich's ataxia cardiomyopathy.
- United states of amnesia: rescuing memory loss from diverse conditions
Summary: We examine cases of memory loss caused by diverse interventions, diseases and injuries, and summarize how engram investigation and manipulation may help us understand and potentially treat amnesia.
- A guide to using functional magnetic resonance imaging to study Alzheimer's disease in animal models
Summary: This Review examines the use of functional magnetic resonance imaging for studying Alzheimer's disease in animal models, and addresses important considerations concerning model choice, anesthetic use and application of stimuli.
- Molecular biomarkers of Alzheimer's disease: progress and prospects
Summary: This ‘At a glance’ article summarises the molecular biomarkers of Alzheimer's and related diseases, highlighting the challenges and opportunities for diagnostic and prognostic applications.
- Sporadic amyotrophic lateral sclerosis (SALS) – skeletal muscle response to cerebrospinal fluid from SALS patients in a rat model
Editor's choice: In amyotrophic lateral sclerosis, a motor neurodegenerative disease, the pathological changes of the skeletal muscle can significantly damage motor neurons, leading to progressive neurodegeneration.