SPECIAL COLLECTION: NEURODEGENERATION: FROM MODELS TO MECHANISMS TO THERAPIES
Summary: This Editorial introduces a new Special Collection, ‘Neurodegeneration: from models to mechanisms to therapies’, providing a summary of the research and review articles published in this launch issue, as well as highlighting some of DMM's most-read neurodegeneration-related articles from recent issues.
A MODEL FOR LIFE
Summary: Huda Zoghbi discusses her remarkable journey from the clinic to the bench, highlighting the experiences and collaborations that inspired her interest in neurological diseases such as spinocerebellar ataxia type 1 (SCA1) and Rett syndrome.
AT A GLANCE
Summary: In this At a Glance review, Edward Lee and co-authors provide an overview of RNA metabolism defects, including mislocalization of RNA-binding proteins and microRNA biogenesis alterations, that contribute to neurodegenerative disease pathology.
Summary: In this Review, Ludo Van Den Bosch and colleagues discuss the different model systems for studying ALS and how they have contributed to our current understanding of the etiology and pathology of this neurodegenerative disease.
Upregulation of CB2 receptors in reactive astrocytes in canine degenerative myelopathy, a disease model of amyotrophic lateral sclerosis
Editors' choice: CB2 receptors are upregulated in activated astrocytes recruited at the damaged spinal cord in dogs with degenerative myelopathy, a canine model of amyotrophic lateral sclerosis.
Loss of Ranbp2 in motoneurons causes disruption of nucleocytoplasmic and chemokine signaling, proteostasis of hnRNPH3 and Mmp28, and development of amyotrophic lateral sclerosis-like syndromes
Summary: Loss of Ranbp2 in spinal motoneurons drives ALS syndromes in mice and Ranbp2 functions in nucleocytoplasmic trafficking, proteostasis and chemokine signaling uncover novel therapeutic targets and mechanisms for motoneuron disease.
The tyrosine kinase receptor Tyro3 enhances lifespan and neuropeptide Y (Npy) neuron survival in the mouse anorexia (anx) mutation
Summary: The receptor tyrosine kinase Tyro3 improves weight and survival in the anorexia mouse model, and is one of the few factors known to sustain appetite regulatory circuitry.
Schwann cells are activated by ATP released from neurons in an in vitro cellular model of Miller Fisher syndrome
Summary: ATP released by degenerating neurons participates in neuron-Schwann cell communication in an in vitro model of Miller Fisher syndrome and activates Schwann cell pro-regenerative properties.
The familial dysautonomia disease gene IKBKAP is required in the developing and adult mouse central nervous system
Summary: Ikbkap is essential for normal CNS development, neuronal survival and behavior, adding to our understanding of the role of the Elongator complex in the mammalian CNS.
Blood RNA biomarkers in prodromal PARK4 and rapid eye movement sleep behavior disorder show role of complexin 1 loss for risk of Parkinson's disease
Summary: Complexin 1 is a prodromal biomarker and risk factor for REM sleep behavior disorder and PARK4-associated Parkinson's disease.
Genetic mutations linked to Parkinson's disease differentially control nucleolar activity in pre-symptomatic mouse models
Summary: Genetic mutations linked to Parkinson's disease lead to stage-specific deregulation of the nucleolus, a major integrator of the cellular stress response.
A differential autophagy-dependent response to DNA double-strand breaks in bone marrow mesenchymal stem cells from sporadic ALS patients
Summary: A novel endogenous disease mechanism in cells from ALS patients after NCS-induced DNA damage.
Summary: Grb2 has a unique role in protecting the cytoskeletal architecture in AD-like conditions, offering a potential new strategy for controlling neurodegeneration.
Vulnerability of primary neurons derived from Tg2576 Alzheimer mice to oxygen and glucose deprivation: role of intraneuronal amyloid-β accumulation and astrocytes
Summary: In vitro systems derived from AD mice can be used to investigate the vulnerability of AD neurons to different neurotoxic challenges, including oxygen glucose deprivation.