Molecular determinants of survival motor neuron (SMN) protein cleavage by the calcium-activated protease, calpain

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Molecular determinants of survival motor neuron (SMN) protein cleavage by the calcium-activated protease, calpain is …
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scholarly articleQ13442814

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P819ADS bibcode2010PLoSO...515769F
P356DOI10.1371/JOURNAL.PONE.0015769
P932PMC publication ID3012718
P698PubMed publication ID21209906
P5875ResearchGate publication ID49732317

P50authorA. Gregory MateraQ55154446
P2093author name stringJennifer L Fuentes
Molly S Strayer
P2860cites workAmyloid beta peptide toxicity in differentiated PC12 cells: calpain-calpastatin, caspase, and membrane damageQ46625129
An emerging picture of synapse formation: a balance of two opposing pathwaysQ46757679
Selective vulnerability of motor neurons and dissociation of pre- and post-synaptic pathology at the neuromuscular junction in mouse models of spinal muscular atrophyQ46861362
Survival motor neuron function in motor axons is independent of functions required for small nuclear ribonucleoprotein biogenesis.Q47073257
Intracellular Ca2+-dependent protease (CALPAIN) and its high-molecular-weight endogenous inhibitor (CALPASTATIN)Q47301366
Altered intracellular Ca2+ homeostasis in nerve terminals of severe spinal muscular atrophy mice.Q47807997
Purification and Characterization of a Calcium-Activated Neutral Protease from Monkey Brain and Its Action on Neuropeptides1Q48631663
Non-coding RNAs: lessons from the small nuclear and small nucleolar RNAsQ22121992
Purification of native survival of motor neurons complexes and identification of Gemin6 as a novel componentQ24292040
Gemin8 is required for the architecture and function of the survival motor neuron complexQ24306053
Coupled in vitro import of U snRNPs and SMN, the spinal muscular atrophy proteinQ24307701
The spinal muscular atrophy disease gene product, SMN, and its associated protein SIP1 are in a complex with spliceosomal snRNP proteinsQ24316085
The SMN-SIP1 complex has an essential role in spliceosomal snRNP biogenesisQ24316121
A novel nuclear structure containing the survival of motor neurons proteinQ24324247
Gemin proteins are required for efficient assembly of Sm-class ribonucleoproteins.Q24536283
Symmetrical dimethylation of arginine residues in spliceosomal Sm protein B/B' and the Sm-like protein LSm4, and their interaction with the SMN protein.Q24540057
Coilin forms the bridge between Cajal bodies and SMN, the spinal muscular atrophy proteinQ24600358
Dephosphorylation of survival motor neurons (SMN) by PPM1G/PP2Cgamma governs Cajal body localization and stability of the SMN complexQ24682443
A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye bindingQ25938984
SMN tudor domain structure and its interaction with the Sm proteinsQ27629094
Concerted multi-pronged attack by calpastatin to occlude the catalytic cleft of heterodimeric calpainsQ27652953
Calcium-bound structure of calpain and its mechanism of inhibition by calpastatinQ27652954
Essential role for the tudor domain of SMN in spliceosomal U snRNP assembly: implications for spinal muscular atrophyQ28137718
The exon 2b region of the spinal muscular atrophy protein, SMN, is involved in self-association and SIP1 bindingQ28138530
Muscle cachexia: current concepts of intracellular mechanisms and molecular regulationQ28141769
The calpain systemQ28185062
SMN, the product of the spinal muscular atrophy gene, binds preferentially to dimethylarginine-containing protein targetsQ28199238
Determinants of the interaction of the spinal muscular atrophy disease protein SMN with the dimethylarginine-modified box H/ACA small nucleolar ribonucleoprotein GAR1Q28203025
The SMN complex, an assemblyosome of ribonucleoproteinsQ28204829
Specific interaction of Smn, the spinal muscular atrophy determining gene product, with hnRNP-R and gry-rbp/hnRNP-Q: a role for Smn in RNA processing in motor axons?Q28214361
Spinal muscular atrophyQ28251310
A degron created by SMN2 exon 7 skipping is a principal contributor to spinal muscular atrophy severityQ28274685
Determination of peptide substrate specificity for mu-calpain by a peptide library-based approach: the importance of primed side interactionsQ28276503
Genetic mapping of chronic childhood-onset spinal muscular atrophy to chromosome 5q11.2-13.3Q28280414
Regulation of SMN protein stabilityQ28304878
The Ewing's sarcoma protein interacts with the Tudor domain of the survival motor neuron proteinQ28567835
CaMPDB: a resource for calpain and modulatory proteolysis.Q51713028
Neuromuscular defects and breathing disorders in a new mouse model of spinal muscular atrophy.Q51917443
Involvement of calpains in the destabilization of the acetylcholine receptor clusters in rat myotubes.Q52171980
Gene for chronic proximal spinal muscular atrophies maps to chromosome 5qQ59059683
Neurofilament accumulation at the motor endplate and lack of axonal sprouting in a spinal muscular atrophy mouse modelQ61875385
International SMA consortium meeting. (26-28 June 1992, Bonn, Germany)Q67481178
Cell-penetrating inhibitors of calpain block both membrane fusion and filamin cleavage in chick embryonic myoblastsQ70721504
Phosphorylation modulates calpain-mediated proteolysis and calmodulin binding of the 200-kDa and 160-kDa neurofilament proteinsQ70770912
Molecular analysis of candidate genes on chromosome 5q13 in autosomal recessive spinal muscular atrophy: evidence of homozygous deletions of the SMN gene in unaffected individualsQ70987146
Importance of the ATP-ubiquitin-proteasome pathway in the degradation of soluble and myofibrillar proteins in rabbit muscle extractsQ71742635
An antisense oligodeoxyribonucleotide to m-calpain mRNA inhibits myoblast fusionQ72017235
Comparative specificity and kinetic studies on porcine calpain I and calpain II with naturally occurring peptides and synthetic fluorogenic substratesQ72397802
An exonic enhancer is required for inclusion of an essential exon in the SMA-determining gene SMNQ73295491
A role for polyproline motifs in the spinal muscular atrophy protein SMN. Profilins bind to and colocalize with smn in nuclear gemsQ73297574
SMN oligomerization defect correlates with spinal muscular atrophy severityQ74557706
Spinal muscular atrophy and therapeutic prospectsQ79318646
A comprehensive interaction map of the human survival of motor neuron (SMN) complexQ79441366
Gemin2 plays an important role in stabilizing the survival of motor neuron complexQ79794565
Nuclear calpain regulates Ca2+-dependent signaling via proteolysis of nuclear Ca2+/calmodulin-dependent protein kinase type IV in cultured neuronsQ80987899
Hypomorphic Smn knockdown C2C12 myoblasts reveal intrinsic defects in myoblast fusion and myotube morphologyQ81340087
SMNDelta7, the major product of the centromeric survival motor neuron (SMN2) gene, extends survival in mice with spinal muscular atrophy and associates with full-length SMNQ81385781
Spinal muscular atrophy: the RNP connectionQ82533240
The spinal muscular atrophy gene product regulates neurite outgrowth: importance of the C terminus.Q38304405
A mouse model for spinal muscular atrophyQ38316939
Potential m-calpain substrates during myoblast fusionQ38328892
Epstein-Barr virus nuclear antigen 2 binds via its methylated arginine-glycine repeat to the survival motor neuron protein.Q39741960
Defective Ca2+ channel clustering in axon terminals disturbs excitability in motoneurons in spinal muscular atrophyQ39751507
Calpain small-1 modulates Akt/FoxO3A signaling and apoptosis through PP2A.Q39912728
Refined characterization of the expression and stability of the SMN gene products.Q40089875
Rapsyn interaction with calpain stabilizes AChR clusters at the neuromuscular junction.Q40104987
Phosphorylation regulates the activity of the SMN complex during assembly of spliceosomal U snRNPs.Q40107820
Two PEST-like motifs regulate Ca2+/calpain-mediated cleavage of the CaVbeta3 subunit and provide important determinants for neuronal Ca2+ channel activityQ40278911
Distinct domains of the spinal muscular atrophy protein SMN are required for targeting to Cajal bodies in mammalian cellsQ40322744
Overexpression of calpastatin inhibits L8 myoblast fusionQ40420244
Molecular and functional analysis of intragenic SMN1 mutations in patients with spinal muscular atrophy.Q40484524
m-Calpain implication in cell cycle during muscle precursor cell activationQ40537812
Involvement of survival motor neuron (SMN) protein in cell deathQ40698290
Characterization of functional domains of the SMN protein in vivoQ40778498
Natural history in proximal spinal muscular atrophy. Clinical analysis of 445 patients and suggestions for a modification of existing classificationsQ40986555
Correlation between severity and SMN protein level in spinal muscular atrophyQ41102726
Tudor domains in proteins that interact with RNA.Q41361779
Role of intracellular proteases in differentiation of L6 myoblast cells.Q41484451
Autolysis parallels activation of mu-calpainQ42983783
When is a deletion not a deletion? When it is convertedQ43105061
The human centromeric survival motor neuron gene (SMN2) rescues embryonic lethality in Smn(-/-) mice and results in a mouse with spinal muscular atrophyQ43569750
A multiprotein complex mediates the ATP-dependent assembly of spliceosomal U snRNPsQ43803910
Subcellular localization and in vivo subunit interactions of ubiquitous mu-calpain.Q44319476
Neuromuscular defects in a Drosophila survival motor neuron gene mutant.Q44463867
On the sequential determinants of calpain cleavageQ44779399
Reduced expression of nicotinic AChRs in myotubes from spinal muscular atrophy I patients.Q45025568
Degradation of survival motor neuron (SMN) protein is mediated via the ubiquitin/proteasome pathwayQ45036608
Analysis of point mutations in the SMN1 gene in SMA patients bearing a single SMN1 copy.Q45867775
Calpain activates caspase-8 in neuron-like differentiated PC12 cells via the amyloid-beta-peptide and CD95 pathways.Q45914574
A role for complexes of survival of motor neurons (SMN) protein with gemins and profilin in neurite-like cytoplasmic extensions of cultured nerve cells.Q46564145
Characterization and expression of calpain 10. A novel ubiquitous calpain with nuclear localizationQ28576163
Smn, the spinal muscular atrophy-determining gene product, modulates axon growth and localization of beta-actin mRNA in growth cones of motoneuronsQ28576647
Identification and characterization of a spinal muscular atrophy-determining geneQ29547495
Amino acid sequences common to rapidly degraded proteins: the PEST hypothesisQ29547538
Regulation of cell death: the calcium-apoptosis linkQ29615554
PEST sequences and regulation by proteolysisQ29616394
A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophyQ29617367
Direct interaction of the spinal muscular atrophy disease protein SMN with the small nucleolar RNA-associated protein fibrillarinQ31003752
Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?Q33785933
Survival motor neuron gene transcript analysis in muscles from spinal muscular atrophy patients.Q34302504
Best practice guidelines for molecular analysis in spinal muscular atrophyQ34314037
Quantitative analyses of SMN1 and SMN2 based on real-time lightCycler PCR: fast and highly reliable carrier testing and prediction of severity of spinal muscular atrophyQ34353103
The survival motor neuron protein in spinal muscular atrophyQ34435656
Nuclear bodies: random aggregates of sticky proteins or crucibles of macromolecular assembly?Q35003162
SMN mutants of spinal muscular atrophy patients are defective in binding to snRNP proteins.Q35641208
Interaction of calpastatin with calpain: a reviewQ35837217
A Drosophila melanogaster model of spinal muscular atrophy reveals a function for SMN in striated muscle.Q36118011
When contractile proteins go bad: the sarcomere and skeletal muscle diseaseQ36193679
Calpains in muscle wasting.Q36241898
Regulating cell migration: calpains make the cut.Q36243926
Caspase and calpain function in cell death: bridging the gap between apoptosis and necrosisQ36301734
Intact satellite cells lead to remarkable protection against Smn gene defect in differentiated skeletal muscle.Q36323505
Calpain and synaptic functionQ36586276
Chaperoning ribonucleoprotein biogenesis in health and diseaseQ36777301
Myofibrillar protein turnover: the proteasome and the calpainsQ36914804
SMN complex localizes to the sarcomeric Z-disc and is a proteolytic target of calpainQ36934614
Spinal muscular atrophy and a model for survival of motor neuron protein function in axonal ribonucleoprotein complexesQ37033848
Calpain 3, the "gatekeeper" of proper sarcomere assembly, turnover and maintenanceQ37039715
Calpain-mediated signaling mechanisms in neuronal injury and neurodegenerationQ37237452
Survival motor neuron protein modulates neuron-specific apoptosisQ37292520
Reduced SMN protein impairs maturation of the neuromuscular junctions in mouse models of spinal muscular atrophyQ37294764
Molecular control of mammalian myoblast fusionQ37315586
Impaired synaptic vesicle release and immaturity of neuromuscular junctions in spinal muscular atrophy miceQ37352940
Calpain-1 cleaves and activates caspase-7.Q37375801
KH-type splicing regulatory protein interacts with survival motor neuron protein and is misregulated in spinal muscular atrophy.Q38296508
P275copyright licenseCreative Commons Attribution 4.0 InternationalQ20007257
P6216copyright statuscopyrightedQ50423863
P4510describes a project that usesImageJQ1659584
P433issue12
P407language of work or nameEnglishQ1860
P304page(s)e15769
P577publication date2010-12-30
P1433published inPLOS OneQ564954
P1476titleMolecular determinants of survival motor neuron (SMN) protein cleavage by the calcium-activated protease, calpain
P478volume5

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cites work (P2860)
Q34490238A multi-exon-skipping detection assay reveals surprising diversity of splice isoforms of spinal muscular atrophy genes
Q26781368An eccentric calpain, CAPN3/p94/calpain-3
Q34082155Assays for the identification and prioritization of drug candidates for spinal muscular atrophy
Q57459208Calpain Inhibition Increases SMN Protein in Spinal Cord Motoneurons and Ameliorates the Spinal Muscular Atrophy Phenotype in Mice
Q96685645Calpain system is altered in survival motor neuron-reduced cells from in vitro and in vivo spinal muscular atrophy models
Q31032498Coilin phosphomutants disrupt Cajal body formation, reduce cell proliferation and produce a distinct coilin degradation product
Q35715801Different Stability and Proteasome-Mediated Degradation Rate of SMN Protein Isoforms.
Q64055868Drug screening with human SMN2 reporter identifies SMN protein stabilizers to correct SMA pathology
Q28115047Nuclear Fragile X Mental Retardation Protein is localized to Cajal bodies
Q36460273Regulated specific proteolysis of the Cajal body marker protein coilin
Q27679173Solution structure of the core SMN–Gemin2 complex
Q38073714Spinal muscular atrophy and the antiapoptotic role of survival of motor neuron (SMN) protein

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