Survival of motor neuron
Encyclopedia
The Survival of Motor Neuron (SMN) is a protein involved in the assembly of snRNP
s, the essential components of spliceosomal machinery. A lack of SMN due to SMN1
deletion results in widespread splicing defects, especially in spinal motor neurons, and is one cause of spinal muscular atrophy
.
SMN also functions in transcriptional regulation, telomerase regeneration and cellular trafficking.
A 2004 study using high-throughput screening
found indoprofen
to increase production of the survival of motor neuron protein, suggesting it may provide insight into treatments for spinal muscular atrophies
.
Later research show a possible role of SMN in neuronal migration and/or differentiation
.
SnRNP
snRNPs , or small nuclear ribonucleoproteins, are RNA-protein complexes that combine with unmodified pre-mRNA and various other proteins to form a spliceosome, a large RNA-protein molecular complex upon which splicing of pre-mRNA occurs...
s, the essential components of spliceosomal machinery. A lack of SMN due to SMN1
SMN1
Survival motor neuron protein is a protein that in humans is encoded by the SMN1 gene.-Interactions:SMN1 has been shown to interact with Gem-associated protein 7, GEMIN4, KPNB1, Survival of motor neuron protein-interacting protein 1, DDX20, Coilin, Small nuclear ribonucleoprotein D1, Fibrillarin,...
deletion results in widespread splicing defects, especially in spinal motor neurons, and is one cause of spinal muscular atrophy
Spinal muscular atrophy
Spinal Muscular Atrophy is a neuromuscular disease characterized by degeneration of motor neurons, resulting in progressive muscular atrophy and weakness. The clinical spectrum of SMA ranges from early infant death to normal adult life with only mild weakness...
.
SMN also functions in transcriptional regulation, telomerase regeneration and cellular trafficking.
A 2004 study using high-throughput screening
High-throughput screening
High-throughput screening is a method for scientific experimentation especially used in drug discovery and relevant to the fields of biology and chemistry. Using robotics, data processing and control software, liquid handling devices, and sensitive detectors, High-Throughput Screening allows a...
found indoprofen
Indoprofen
Indoprofen is a non-steroidal anti-inflammatory drug. It was withdrawn worldwide in the 1980s after postmarketing reports of severe gastrointestinal bleeding....
to increase production of the survival of motor neuron protein, suggesting it may provide insight into treatments for spinal muscular atrophies
Spinal muscular atrophy
Spinal Muscular Atrophy is a neuromuscular disease characterized by degeneration of motor neurons, resulting in progressive muscular atrophy and weakness. The clinical spectrum of SMA ranges from early infant death to normal adult life with only mild weakness...
.
Later research show a possible role of SMN in neuronal migration and/or differentiation
Cellular differentiation
In developmental biology, cellular differentiation is the process by which a less specialized cell becomes a more specialized cell type. Differentiation occurs numerous times during the development of a multicellular organism as the organism changes from a simple zygote to a complex system of...
.