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Persyn, a member of the synuclein family, has a distinct pattern of expression in the developing nervous system.

ABSTRACT: The synucleins are a unique family of small intracellular proteins that have recently attracted considerable attention because of their involvement in human neurodegenerative diseases. We have cloned a new member of the synuclein family called persyn. In contrast to other synucleins, which are presynaptic proteins of CNS neurons, persyn is a cytosolic protein that is expressed predominantly in the cell bodies and axons of primary sensory neurons, sympathetic neurons, and motoneurons. Northern blotting, in situ hybridization, Western blotting, and immunohistochemistry revealed that persyn mRNA and protein are expressed in these neurons from the earliest stages of axonal outgrowth and are maintained at a high level throughout life. Persyn also becomes detectable in evolutionary recent regions of the brain by adulthood.

SUBMITTER: Buchman VL

PROVIDER: S-EPMC6792889 | biostudies-literature | 1998 Nov

REPOSITORIES: biostudies-literature

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Persyn, a member of the synuclein family, has a distinct pattern of expression in the developing nervous system.

Buchman V L VL Hunter H J HJ Pinõn L G LG Thompson J J Privalova E M EM Ninkina N N NN Davies A M AM

The Journal of neuroscience : the official journal of the Society for Neuroscience 19981101 22

The synucleins are a unique family of small intracellular proteins that have recently attracted considerable attention because of their involvement in human neurodegenerative diseases. We have cloned a new member of the synuclein family called persyn. In contrast to other synucleins, which are presynaptic proteins of CNS neurons, persyn is a cytosolic protein that is expressed predominantly in the cell bodies and axons of primary sensory neurons, sympathetic neurons, and motoneurons. Northern bl ...[more]

PMID: 9801372

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Project description:The two main pathological hallmarks of Parkinson's disease are loss of dopamine neurons in the substantia nigra pars compacta and proteinaceous amyloid fibrils composed mostly of ?-synuclein, called Lewy pathology. Levodopa to enhance dopaminergic transmission remains one of the most effective treatment for alleviating the motor symptoms of Parkinson's disease (Olanow, Mov Disord 34:812-815, 2019). In addition, deep brain stimulation (Bronstein et al., Arch Neurol 68:165, 2011) to modulate basal ganglia circuit activity successfully alleviates some motor symptoms. MRI guided focused ultrasound in the subthalamic nucleus is a promising therapeutic strategy as well (Martinez-Fernandez et al., Lancet Neurol 17:54-63, 2018). However, to date, there exists no treatment that stops the progression of this disease. The findings that ?-synuclein can be released from neurons and inherited through interconnected neural networks opened the door for discovering novel treatment strategies to prevent the formation and spread of Lewy pathology with the goal of halting PD in its tracks. This hypothesis is based on discoveries that pathologic aggregates of ?-synuclein induce the endogenous ?-synuclein protein to adopt a similar pathologic conformation, and is thus self-propagating. Phase I clinical trials are currently ongoing to test treatments such as immunotherapy to prevent the neuron to neuron spread of extracellular aggregates. Although tremendous progress has been made in understanding how Lewy pathology forms and spreads throughout the brain, cell intrinsic factors also play a critical role in the formation of pathologic ?-synuclein, such as mechanisms that increase endogenous ?-synuclein levels, selective expression profiles in distinct neuron subtypes, mutations and altered function of proteins involved in ?-synuclein synthesis and degradation, and oxidative stress. Strategies that prevent the formation of pathologic ?-synuclein should consider extracellular release and propagation, as well as neuron intrinsic mechanisms.

Dataset Information

Persyn, a member of the synuclein family, has a distinct pattern of expression in the developing nervous system.

Publications

Persyn, a member of the synuclein family, has a distinct pattern of expression in the developing nervous system.

Similar Datasets

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