Investigating the Effects of Atlastin-1 on Spastin and REEP1 Using Primary Cultured Neurons from Mice with Motor Neuron Disease
Disruption in axonal transport is a common cause of motor neuron degeneration and is associated with many neurological disorders including the hereditary spastic paraplegias (HSP’s). HSP’s are a group of inherited disorders characterized by weakness and spasticity in the lower limbs that pathologically lead to retrograde degeneration of corticospinal neurons. Over half of all autosomal dominant cases of HSP are caused by mutations in spastin (SPG4), atlastin (SPG3A), and receptor expression enhancing protein (REEP1). These proteins co-localize and bind together in the endoplasmic reticulum and are directly involved in the coordination of ER shaping and microtubule dynamics in corticospinal neurons (Figure 1). The collaboration of these three HSP proteins suggests that mutations may cause impairments in the relationship between ER tubules and microtubule cytoskeleton. This study used primary cortical neuron cultures from adult knockout atlastin mice in an attempt to investigate the consequences of reducing atlastin-1 protein levels in mouse models.
Showing items related by title, author, creator and subject.
Investigating the Effects of Atlastin-1 on Spastin and REEP1 Using Primary Cultured Neurons from Mice with Motor Neuron Disease Edsall, Taryn N. (Kalamazoo College, 2013)Impairment of axonal transport is a common cause of motor neuron degeneration and is associated with many neurological disorders including Alzheimer's disease, Parkinson's disease, Huntington's disease, amyotrophic lateral ...
Tamm, Brendan J. (Kalamazoo, Mich. : Kalamazoo College, 2014)Learning and memory, as well as cognition, are mediated by networks of interconnected neurons. Over time, synapses with high levels of activity will become stronger and more easily stimulated to depolarize the neuron. ...
Analysis of enhanced Green Fluorescent Protein Expression in Corticotropin-Releasing Hormone Neurons in CRH-eGFP BAC Transgenic Mice Cregan, Lindsey (Kalamazoo College, 2007-04-27)The endocrine response to stress is mediated by the hypothalamic-pituitary-adrenal (HPA) axis (Figure 1). The key hypothalamic regulator within the HPA axis is corticotropin-releasing hormone (CRH). Dysregulation within ...