◀ Back to SNRPB
SNRPB — STRAP
Protein-Protein interactions - manually collected from original source literature:
Studies that report less than 10 interactions are marked with *
Text-mined interactions from Literome
Bertrandy et al., Hum Mol Genet 1999
:
SMA is
caused by alterations of the
survival motor neuron ( SMN ) gene which encodes a novel protein of hitherto unclear function
Weinstein et al., Brain Res Dev Brain Res 1999
:
v-Crk also
caused an increase in the number of surviving
spinal motor neurons (SMN) , and interestingly, upon staining of sternomastoid muscle fibers with rhodamine conjugated alpha-bungarotoxin, many muscle fibers displayed an apparent increase in volume of motor end plates, and an increase in complexity of neuromuscular junctions ( NMJ )
Andreassi et al., Amino Acids 2000
:
Immunolocalization studies using SMN monoclonal antibody showed that
SMN is decreased in cultures grown in low K+ or chemically defined medium with respect to cultures grown in high K+ medium and that an increase of
SMN can be
induced by treatment of low K+ cultures with glutamate or N-methyl-D-aspartate
Hofmann et al., Proc Natl Acad Sci U S A 2000
(Muscular Atrophy, Spinal) :
Htra2-beta 1 stimulates an exonic splicing enhancer and can
restore full-length
SMN expression to survival motor neuron 2 (SMN2)
Baron-Delage et al., Mol Med 2000
(Muscular Atrophy, Spinal) :
Interferons and
IRF-1 induce expression of the
survival motor neuron (SMN) genes ...
IRF-1 is, therefore, at least in part
responsible for the induction of
SMN and SMNc by IFNs
Pellizzoni et al., J Cell Biol 2001
:
In vitro experiments suggest that
RHA mediates the association of
SMN with the COOH-terminal domain of pol II
Yong et al., EMBO J 2002
:
The
stem--loop 1 domain of U1 (SL1) is necessary and
sufficient for
SMN complex binding in vivo and in vitro
Mourelatos et al., Genes Dev 2002
:
Reduction in
SMN protein
results in
Spinal muscular atrophy (SMA) , a common neurodegenerative disease
Brichta et al., Hum Mol Genet 2003
(Muscular Atrophy, Spinal) :
Importantly, this up-regulation of SMN could be most likely attributed to increased levels of
Htra2-beta 1 which facilitates the correct splicing of SMN2 RNA as well as to an
SMN gene transcription
activation
Majumder et al., J Biol Chem 2004
:
Identification of a novel cyclic AMP-response element ( CRE-II ) and the
role of
CREB-1 in the cAMP induced expression of the
survival motor neuron (SMN) gene ... Transient overexpression of
CREB1 protein
resulted in a 4-fold increase of the
SMN promoter activity
Zou et al., J Biol Chem 2004
:
SMA results from loss of
survival motor neuron (SMN) expression and subsequent death of motor neuron cells
Brahe et al., Eur J Hum Genet 2005
(Muscular Atrophy) :
Spinal muscular atrophy (SMA) is
caused by insufficient levels of
survival motor neuron (SMN) protein
Lunn et al., Chem Biol 2004
:
Indoprofen, a nonsteroidal anti-inflammatory drug ( NSAID ) and
cyclooxygenase (COX) inhibitor, selectively
increased SMN2-luciferase reporter protein and endogenous
SMN protein and caused a 5-fold increase in the number of nuclear gems in fibroblasts from SMA patients
Wolstencroft et al., Hum Mol Genet 2005
(Muscular Atrophy, Spinal) :
A non-sequence-specific
requirement for
SMN protein activity : the role of aminoglycosides in inducing elevated
SMN protein levels
Wang et al., Mol Genet Metab 2005
(Atrophy...) :
Depletion of
SMN protein by RNA interference in control fibroblasts
increased caspase-3 activity, whereas transfection of SMA fibroblasts with wild-type SMN decreased caspase-3 activity
Sharma et al., Exp Cell Res 2005
(Muscular Atrophy, Spinal) :
Spinal muscular atrophy (SMA) is
caused by reduced levels of
SMN ( survival of motor neurons protein ) and consequent loss of motor neurons
Sumner et al., NeuroRx 2006
(Disease Models, Animal...) :
SMN2 produces reduced amounts of full-length SMN mRNA, and spinal muscular atrophy likely
results from insufficient levels of
SMN protein in motor neurons
Baughan et al., Mol Ther 2006
(Muscular Atrophy, Spinal) :
SMA is
caused by the loss of
survival motor neuron-1 (SMN1)
Mattis et al., Hum Genet 2006
(Muscular Atrophy, Spinal) :
SMA is
caused by the homozygous absence of
survival motor neuron-1 (SMN1)
Pellizzoni et al., EMBO Rep 2007
(Muscular Atrophy, Spinal) :
The survival motor neuron (SMN) protein is part of a macromolecular complex that functions in the biogenesis of small nuclear ribonucleoproteins ( snRNPs ) -- the essential components of the pre-messenger RNA splicing machinery -- as well as probably other RNPs. Reduced levels of
SMN expression
cause the inherited motor neuron disease
spinal muscular atrophy (SMA)
Balabanian et al., Neurol Res 2007
(Disease Models, Animal...) :
Spinal muscular atrophy (SMA) is
caused by
survival of motor neuron (SMN) deficiency, leading to specific motor neuron attrition
Coady et al., Mol Ther 2007
(Muscular Atrophy, Spinal) :
Spinal muscular atrophy (SMA) is
caused by loss of
survival motor neuron-1 (SMN1)
Piazzon et al., J Biol Chem 2008
(Fragile X Syndrome...) :
Spinal muscular atrophy (SMA) is
caused by reduced levels of the
survival of motor neuron (SMN) protein
Gavrilina et al., Hum Mol Genet 2008
(Muscular Atrophy, Spinal) :
The
PrP results in high levels of
SMN in neurons at embryonic day 15
McGovern et al., Hum Mol Genet 2008
(Disease Models, Animal...) :
Spinal muscular atrophy (SMA) is
caused by reduced levels of
survival motor neuron (SMN) protein
Hauke et al., Hum Mol Genet 2009
(Muscular Atrophy, Spinal) :
Spinal muscular atrophy (SMA) , a common neuromuscular disorder, is
caused by homozygous absence of the
survival motor neuron gene 1 (SMN1) , while the disease severity is mainly influenced by the number of SMN2 gene copies
Renvoisé et al., Hum Mol Genet 2009
(Muscular Atrophy, Spinal) :
Indeed, RNA interference knockdown experiments in control fibroblasts demonstrate that
SMN is
required for accumulation of
Nopp140 in CBs ... Conversely, overexpression of
SMN in SMA cells
restores the CB localization of
Nopp140 , whereas SMN mutants found in SMA patients are defective in promoting the association of Nopp140 with CBs
Martins de Araújo et al., RNA 2009
(Muscular Atrophy, Spinal) :
Two trans acting factors implicated in
SMN regulation , SF2/ASF and hnRNP A1, promote and repress, respectively,
U2 snRNP recruitment to both RNAs ... Two trans acting factors implicated in
SMN regulation , SF2/ASF and
hnRNP A1 , promote and repress, respectively, U2 snRNP recruitment to both RNAs ... Two trans acting factors implicated in
SMN regulation ,
SF2/ASF and hnRNP A1, promote and repress, respectively, U2 snRNP recruitment to both RNAs
Turner et al., Neurobiol Dis 2009
(Amyotrophic Lateral Sclerosis...) :
Furthermore, ALS linked mutant
SOD1 expression severely
reduced SMN protein levels, but not transcript, in neuronal culture and mouse models from early presymptomatic disease
Swoboda et al., PloS one 2009
(Muscular Atrophy, Spinal) :
Full length
SMN levels were unchanged and
Delta7SMN levels were significantly
reduced for 2 of 3 treatment visits
Garbes et al., Hum Mol Genet 2009
(Muscular Atrophy, Spinal) :
LBH589 induces up to 10-fold
SMN protein levels by several independent mechanisms and is effective even in cells from SMA patients non-responsive to valproate ... Furthermore,
LBH589 stabilizes
SMN by reducing its ubiquitinylation as well as favouring incorporation into the SMN complex
Dickson et al., Hum Gene Ther 2008
(Muscular Atrophy, Spinal) :
SMA is
caused by the loss of
survival motor neuron-1 (SMN1)
Butchbach et al., Hum Mol Genet 2010
(Disease Models, Animal...) :
SMA is a
consequence of low levels of
survival motor neuron (SMN) protein
Butchbach et al., Biochem Biophys Res Commun 2010
(Disease Models, Animal...) :
SMA is the
result of reduction in
Survival Motor Neuron (SMN) expression
Coady et al., J Neurosci 2010
(Disease Models, Animal...) :
SMA is
caused by the loss of
Survival Motor Neuron-1 (SMN1) , however, all patients retain at least one copy of a nearly identical gene called SMN2
Riessland et al., Hum Mol Genet 2010
(Disease Models, Animal...) :
SMA is
caused by functional loss of the
survival motor neuron gene 1 (SMN1) , whereas disease severity is mainly influenced by the number of SMN2 copies
Dominguez et al., Hum Mol Genet 2011
(Disease Models, Animal...) :
Loss of
SMN1 leads to reduced
SMN protein levels, inducing degeneration of motor neurons ( MN ) and progressive muscle weakness and atrophy
Hammond et al., PloS one 2010
:
Spinal muscular atrophy (SMA) is
caused by low
survival motor neuron (SMN) levels and patients represent a clinical spectrum due primarily to varying copies of the survival motor neuron-2 (SMN2) gene
Peter et al., Hum Mol Genet 2011
(Disease Models, Animal...) :
Spinal muscular atrophy (SMA) , an inherited disease of motor neuron dysfunction,
results from insufficient levels of the
survival motor neuron (SMN) protein
Bebee et al., Genesis 2011
(Disease Models, Animal...) :
Proximal
spinal muscular atrophy (SMA) is
caused by low levels of the
SMN protein, encoded by the Survival Motor Neuron genes ( SMN1 and SMN2 )
Le et al., Hum Mol Genet 2011
(Disease Models, Animal...) :
Spinal muscular atrophy (SMA) is
caused by loss of the
survival motor neuron 1 gene (SMN1) and retention of the SMN2 gene, resulting in reduced
SMN ...
Spinal muscular atrophy (SMA) is
caused by loss of the
survival motor neuron 1 gene (SMN1) and retention of the SMN2 gene, resulting in reduced SMN
Farooq et al., J Clin Invest 2011
(Disease Models, Animal...) :
Prolactin increases
SMN expression and survival in a mouse model of severe spinal muscular atrophy via the STAT5 pathway
Nölle et al., Hum Mol Genet 2011
(Disease Models, Animal...) :
Spinal muscular atrophy (SMA) , a frequent neurodegenerative disease, is
caused by reduced levels of functional
survival of motoneuron (SMN) protein
Glascock et al., Hum Gene Ther 2012
(Disease Models, Animal...) :
SMA is
caused by the homozygous loss of
Survival Motor Neuron-1 (SMN1)
Glascock et al., Biochem Biophys Res Commun 2012
(Disease Models, Animal...) :
SMA is
caused by the homozygous loss of
Survival Motor Neuron-1 (SMN1) ...
SMA , however, is not
due to complete absence of
SMN , rather a low level of functional full-length SMN is produced by a nearly identical copy gene called SMN2
Caraballo-Miralles et al., Mol Cell Neurosci 2012
(Astrocytoma...) :
The present results suggest that in SMN depleted cells, the increase in profilin I expression and the reduction in
SMN inhibitory
action on
profilin could lead to reduced filamentous actin polymerization, thus decreasing cell motility
Locatelli et al., J Neurochem 2012
(Muscular Atrophy, Spinal) :
At present, the
role of
a-SMN in
SMA is unknown
Longhi et al., Cancer 2012
(Bone Neoplasms...) :
The most common
SMN in the osteosarcoma group was breast cancer ( n = 11 ), and the most common
SMN in the Ewing sarcoma group was radiotherapy
induced osteosarcoma ( n = 6 ) ... The most common SMN in the osteosarcoma group was breast cancer ( n = 11 ), and the most common
SMN in the Ewing sarcoma group was radiotherapy
induced osteosarcoma ( n = 6 )
Renvoisé et al., J Cell Sci 2012
(Muscular Atrophy, Spinal) :
A
role for protein phosphatase
PP1? in
SMN complex formation and subnuclear localization to Cajal bodies ... Moreover, depletion of
PP1? by RNA interference
enhances the localization of the
SMN complex and snRNPs to CBs
Hao et al., J Neurosci 2012
(Movement Disorders...) :
This indicates that
SMN affects
PLS3 protein production ... These data show that
PLS3 protein levels are
dependent on
SMN and that PLS3 is able to rescue the neuromuscular defects and corresponding movement phenotypes caused by low levels of Smn suggesting that decreased PLS3 contributes to SMA motor phenotypes
Sarachan et al., Biochem J 2012
:
The SMN-Gemin2 structure explains how
Gemin2 is
stabilized by
SMN and establishes a framework for structure-function studies to investigate snRNP biogenesis as well as biological processes involving Gemin2 that do not involve snRNP assembly
Murdocca et al., Mol Med 2012
(Disease Models, Animal...) :
SMA is caused by low levels of
survival motor neuron (SMN) protein that
induce selective loss of
a-motor neurons (MNs) in the spinal cord, resulting in progressive muscle atrophy and consequent respiratory failure ...
SMA is
caused by low levels of
survival motor neuron (SMN) protein that induce selective loss of a-motor neurons (MNs) in the spinal cord, resulting in progressive muscle atrophy and consequent respiratory failure
Locatelli et al., J Biol Chem 2012
:
a-SMN dependent
induction of
CCL2 and IGF1 mRNAs resulted in increased intracellular levels and secretion of the respective protein products ...
a-SMN dependent induction of CCL2 and
IGF1 mRNAs resulted in increased intracellular levels and secretion of the respective protein products
Martinez et al., J Neurosci 2012
(Muscular Atrophy, Spinal) :
The inherited motor neuron disease
spinal muscular atrophy (SMA) is
caused by deficient expression of
survival motor neuron (SMN) protein and results in severe muscle weakness
Yamazaki et al., Cell reports 2012
(Amyotrophic Lateral Sclerosis...) :
Decreased expression of
SMN causes the fatal childhood motor neuron disorder
spinal muscular atrophy (SMA) ... Here, we report that FUS associates with the
SMN complex ,
mediated by U1 snRNP and by direct interactions between FUS and
SMN
Han et al., J Biol Chem 2012
(Spinal Muscular Atrophies of Childhood) :
Spinal muscular atrophy (SMA) , the leading genetic disorder of infant mortality, is
caused by low levels of
survival motor neuron (SMN) protein
Cobb et al., Hum Mol Genet 2013
(Body Weight...) :
Spinal Muscular Atrophy (SMA) is
due to the loss of the
survival motor neuron gene 1 (SMN1) , resulting in motor neuron ( MN ) degeneration, muscle atrophy and loss of motor function