Amanda C. Raimer
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View article: Self-oligomerization regulates stability of survival motor neuron protein isoforms by sequestering an SCFSlmb degron
Self-oligomerization regulates stability of survival motor neuron protein isoforms by sequestering an SCFSlmb degron Open
Spinal muscular atrophy (SMA) is caused by homozygous mutations in human SMN1 Expression of a duplicate gene (SMN2) primarily results in skipping of exon 7 and production of an unstable protein isoform, SMNΔ7. Although SMN2 exon skipping i…
View article: Dysregulation of innate immune signaling in animal models of Spinal Muscular Atrophy
Dysregulation of innate immune signaling in animal models of Spinal Muscular Atrophy Open
Background Spinal Muscular Atrophy (SMA) is a devastating neuromuscular disease caused by hypomorphic loss of function in the Survival Motor Neuron (SMN) protein. SMA presents across broad spectrum of disease severity. Unfortunately, verte…
View article: Assembly of higher-order SMN oligomers is essential for metazoan viability and requires an exposed structural motif present in the YG zipper dimer
Assembly of higher-order SMN oligomers is essential for metazoan viability and requires an exposed structural motif present in the YG zipper dimer Open
Protein oligomerization is one mechanism by which homogenous solutions can separate into distinct liquid phases, enabling assembly of membraneless organelles. Survival Motor Neuron (SMN) is the eponymous component of a large macromolecular…
View article: Assembly of higher-order SMN oligomers is essential for metazoan viability and requires an exposed structural motif present in the YG zipper dimer
Assembly of higher-order SMN oligomers is essential for metazoan viability and requires an exposed structural motif present in the YG zipper dimer Open
Protein oligomerization is one mechanism by which homogenous solutions can separate into distinct liquid phases, enabling assembly of membraneless organelles. Survival Motor Neuron (SMN) is the eponymous component of a large macromolecular…
View article: SMN - A chaperone for nuclear RNP social occasions?
SMN - A chaperone for nuclear RNP social occasions? Open
Survival Motor Neuron (SMN) protein localizes to both the nucleus and the cytoplasm. Cytoplasmic SMN is diffusely localized in large oligomeric complexes with core member proteins, called Gemins. Biochemical and cell biological studies hav…
View article: Temperature sensitive SMA-causing point mutations lead to SMN instability, locomotor defects, and premature lethality in <i>Drosophila</i>
Temperature sensitive SMA-causing point mutations lead to SMN instability, locomotor defects, and premature lethality in <i>Drosophila</i> Open
Spinal muscular atrophy (SMA) is the leading genetic cause of death in young children, arising from homozygous deletion or mutation of the SMN1 gene. SMN protein expressed from a paralogous gene, SMN2, is the primary genetic modifier of SM…
View article: Temperature sensitive SMA-causing point mutations lead to SMN instability, locomotor defects, and premature lethality in<i>Drosophila</i>
Temperature sensitive SMA-causing point mutations lead to SMN instability, locomotor defects, and premature lethality in<i>Drosophila</i> Open
Spinal muscular atrophy (SMA) is the leading genetic cause of death in young children, arising from homozygous deletion or mutation of the SMN1 gene. SMN protein expressed from a paralogous gene, SMN2 , is the primary genetic modifier of S…
View article: Comprehensive Modeling of Spinal Muscular Atrophy in Drosophila melanogaster
Comprehensive Modeling of Spinal Muscular Atrophy in Drosophila melanogaster Open
Spinal muscular atrophy (SMA) is a neurodegenerative disorder that affects motor neurons, primarily in young children. SMA is caused by mutations in the Survival Motor Neuron 1 (SMN1) gene. SMN functions in the assembly of spliceosomal RNP…
Composition of the Survival Motor Neuron (SMN) Complex in <i>Drosophila melanogaster</i> Open
Spinal Muscular Atrophy (SMA) is caused by homozygous mutations in the human survival motor neuron 1 (SMN1) gene. SMN protein has a well-characterized role in the biogenesis of small nuclear ribonucleoproteins (snRNPs), core components of …
View article: Composition of the Survival Motor Neuron (SMN) complex in <i>Drosophila melanogaster</i>
Composition of the Survival Motor Neuron (SMN) complex in <i>Drosophila melanogaster</i> Open
Spinal Muscular Atrophy (SMA) is caused by homozygous mutations in the human survival motor neuron 1 ( SMN1 ) gene. SMN protein has a well-characterized role in the biogenesis of small nuclear ribonucleoproteins (snRNPs), core components o…
View article: Comprehensive Modeling of Spinal Muscular Atrophy in <i>Drosophila melanogaster</i>
Comprehensive Modeling of Spinal Muscular Atrophy in <i>Drosophila melanogaster</i> Open
Spinal muscular atrophy (SMA) is a neurodegenerative disorder that affects motor neurons, primarily in young children. SMA is caused by mutations in the Survival Motor Neuron 1 (SMN1) gene. SMN functions in the assembly of spliceosomal RNP…
View article: Self-oligomerization regulates stability of survival motor neuron protein isoforms by sequestering an SCF <sup>Slmb</sup> degron
Self-oligomerization regulates stability of survival motor neuron protein isoforms by sequestering an SCF <sup>Slmb</sup> degron Open
SMN protein levels inversely correlate with the severity of spinal muscular atrophy. The SCF Slmb E3 ligase complex interacts with a degron embedded within the C-terminal self-oligomerization domain of SMN. The findings elucidate a model w…
View article: Self-oligomerization regulates stability of Survival Motor Neuron (SMN) protein isoforms by sequestering an SCF<sup>Slmb</sup>degron
Self-oligomerization regulates stability of Survival Motor Neuron (SMN) protein isoforms by sequestering an SCF<sup>Slmb</sup>degron Open
Spinal muscular atrophy (SMA) is caused by homozygous mutations in human SMN1 . Expression of a duplicate gene ( SMN2 ) primarily results in skipping of exon 7 and production of an unstable protein isoform, SMNΔ7. Although SMN2 exon skippi…
View article: SMN - A chaperone for nuclear RNP social occasions?
SMN - A chaperone for nuclear RNP social occasions? Open
Survival Motor Neuron (SMN) protein localizes to both the nucleus and the cytoplasm. Cytoplasmic SMN is diffusely localized in large oligomeric complexes with core member proteins, called Gemins. Biochemical and cell biological studies hav…