scholarly article | Q13442814 |
P50 | author | Utz Fischer | Q52680981 |
P2093 | author name string | Gunter Meister | |
Christoph Winkler | |||
Marieke Giegerich | |||
Doris Wedlich | |||
Dietmar Gradl | |||
Bernhard Laggerbauer | |||
Christian Eggert | |||
P2860 | cites work | A novel function for SMN, the spinal muscular atrophy disease gene product, in pre-mRNA splicing | Q22008514 |
Unique Sm core structure of U7 snRNPs: assembly by a specialized SMN complex and the role of a new component, Lsm11, in histone RNA processing | Q24323271 | ||
pICln inhibits snRNP biogenesis by binding core spliceosomal proteins | Q24517638 | ||
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 | ||
The methylosome, a 20S complex containing JBP1 and pICln, produces dimethylarginine-modified Sm proteins | Q24548244 | ||
Crystal structures of two Sm protein complexes and their implications for the assembly of the spliceosomal snRNPs | Q27639794 | ||
Stages of embryonic development of the zebrafish | Q27860947 | ||
Characterization of a nuclear 20S complex containing the survival of motor neurons (SMN) protein and a specific subset of spliceosomal Sm proteins | Q28143590 | ||
Spliceosomal UsnRNP biogenesis, structure and function | Q28188106 | ||
Co-regulation of survival of motor neuron (SMN) protein and its interactor SIP1 during development and in spinal muscular atrophy | Q28200174 | ||
Methylation of Sm proteins by a complex containing PRMT5 and the putative U snRNP assembly factor pICln | Q28211723 | ||
The SMN complex | Q28259557 | ||
Molecular characterization of a swelling-induced chloride conductance regulatory protein, pICln | Q28567396 | ||
Smn, the spinal muscular atrophy-determining gene product, modulates axon growth and localization of beta-actin mRNA in growth cones of motoneurons | Q28576647 | ||
Identification and characterization of a spinal muscular atrophy-determining gene | Q29547495 | ||
Pre-mRNA splicing and human disease | Q29617335 | ||
A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophy | Q29617367 | ||
The molecular biology of axon guidance | Q29617857 | ||
Identification of survival motor neuron as a transcriptional activator-binding protein. | Q33292104 | ||
Neuronal guidance. A genetic perspective | Q33594307 | ||
Conservation and divergence of axon guidance mechanisms. | Q33745357 | ||
Purification of the individual snRNPs U1, U2, U5 and U4/U6 from HeLa cells and characterization of their protein constituents | Q33881222 | ||
Gene targeting of Gemin2 in mice reveals a correlation between defects in the biogenesis of U snRNPs and motoneuron cell death | Q34099265 | ||
Control of motor axon guidance in the zebrafish embryo | Q34134037 | ||
Geminin deficiency causes a Chk1-dependent G2 arrest in Xenopus | Q34168520 | ||
Circadian rhythm and light regulate opsin mRNA in rod photoreceptors | Q34438287 | ||
Essential role for the SMN complex in the specificity of snRNP assembly | Q34529450 | ||
Translating axon guidance cues. | Q34856370 | ||
Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding. | Q35567371 | ||
Developmental regulation of zebrafish MyoD in wild-type, no tail and spadetail embryos | Q38287447 | ||
A mouse model for spinal muscular atrophy | Q38316939 | ||
Assisted RNP assembly: SMN and PRMT5 complexes cooperate in the formation of spliceosomal UsnRNPs | Q39665747 | ||
Phosphorylation regulates the activity of the SMN complex during assembly of spliceosomal U snRNPs. | Q40107820 | ||
Disruption of an SF2/ASF-dependent exonic splicing enhancer in SMN2 causes spinal muscular atrophy in the absence of SMN1. | Q40741164 | ||
Functional divergence of two zebrafish midkine growth factors following fish-specific gene duplication | Q40829871 | ||
Correlation between severity and SMN protein level in spinal muscular atrophy | Q41102726 | ||
Dominant negative expression of a cytoplasmically deleted mutant of XB/U-cadherin disturbs mesoderm migration during gastrulation in Xenopus laevis | Q41249065 | ||
The human centromeric survival motor neuron gene (SMN2) rescues embryonic lethality in Smn(-/-) mice and results in a mouse with spinal muscular atrophy | Q43569750 | ||
A multiprotein complex mediates the ATP-dependent assembly of spliceosomal U snRNPs | Q43803910 | ||
Motoneuron fate specification revealed by patterned LIM homeobox gene expression in embryonic zebrafish. | Q48068850 | ||
Active transport of the survival motor neuron protein and the role of exon-7 in cytoplasmic localization. | Q48586550 | ||
PRMT5 (Janus Kinase-binding Protein 1) Catalyzes the Formation of Symmetric Dimethylarginine Residues in Proteins | Q56227906 | ||
An exonic enhancer is required for inclusion of an essential exon in the SMA-determining gene SMN | Q73295491 | ||
A negative element in SMN2 exon 7 inhibits splicing in spinal muscular atrophy | Q73588971 | ||
Pathfinding by identified zebrafish motoneurons in the absence of muscle pioneers | Q73745150 | ||
P433 | issue | 19 | |
P921 | main subject | spinal muscular atrophy | Q580290 |
Survival of motor neuron 1, telomeric | Q29827572 | ||
P304 | page(s) | 2320-2330 | |
P577 | publication date | 2005-10-01 | |
P1433 | published in | Genes & Development | Q1524533 |
P1476 | title | Reduced U snRNP assembly causes motor axon degeneration in an animal model for spinal muscular atrophy | |
P478 | volume | 19 |
Q36118011 | A Drosophila melanogaster model of spinal muscular atrophy reveals a function for SMN in striated muscle. |
Q24657021 | A SMN missense mutation complements SMN2 restoring snRNPs and rescuing SMA mice |
Q34964785 | A cell system for phenotypic screening of modifiers of SMN2 gene expression and function |
Q34490238 | A multi-exon-skipping detection assay reveals surprising diversity of splice isoforms of spinal muscular atrophy genes |
Q28586960 | A role for SMN exon 7 splicing in the selective vulnerability of motor neurons in spinal muscular atrophy |
Q39150934 | ALS-associated mutations in FUS disrupt the axonal distribution and function of SMN. |
Q35114564 | ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP. |
Q104459050 | Aberrant interaction of FUS with the U1 snRNA provides a molecular mechanism of FUS induced amyotrophic lateral sclerosis |
Q38118935 | Aberrant splicing in neurological diseases. |
Q21144988 | Alternative splicing events are a late feature of pathology in a mouse model of spinal muscular atrophy |
Q35578535 | Alternative splicing in spinal muscular atrophy underscores the role of an intron definition model |
Q41856470 | An SMN-dependent U12 splicing event essential for motor circuit function |
Q24311843 | An assembly chaperone collaborates with the SMN complex to generate spliceosomal SnRNPs |
Q29615723 | Animal models of human disease: zebrafish swim into view |
Q37913270 | Biogenesis of spliceosomal small nuclear ribonucleoproteins |
Q36777301 | Chaperoning ribonucleoprotein biogenesis in health and disease |
Q41908770 | Characterization and in vivo functional analysis of the Schizosaccharomyces pombe ICLN gene |
Q42242558 | Characterization of the angiogenic activity of zebrafish ribonucleases |
Q28506113 | Chondrolectin affects cell survival and neuronal outgrowth in in vitro and in vivo models of spinal muscular atrophy |
Q47073237 | Coilin-dependent snRNP assembly is essential for zebrafish embryogenesis |
Q64912689 | Comprehensive Modeling of Spinal Muscular Atrophy in Drosophila melanogaster. |
Q39751507 | Defective Ca2+ channel clustering in axon terminals disturbs excitability in motoneurons in spinal muscular atrophy |
Q36740997 | Deficiency of the Survival of Motor Neuron Protein Impairs mRNA Localization and Local Translation in the Growth Cone of Motor Neurons. |
Q34658141 | Depletion of SMN by RNA interference in HeLa cells induces defects in Cajal body formation. |
Q37365336 | Developmental arrest of Drosophila survival motor neuron (Smn) mutants accounts for differences in expression of minor intron-containing genes |
Q35707832 | Disease mechanisms and therapeutic approaches in spinal muscular atrophy |
Q47070454 | Drosophila SMN complex proteins Gemin2, Gemin3, and Gemin5 are components of U bodies. |
Q58826477 | Drug discovery for spinal muscular atrophy |
Q30498123 | Dynamic control of Cajal body number during zebrafish embryogenesis |
Q37294791 | Embryonic motor axon development in the severe SMA mouse |
Q38039409 | Emerging Roles of Alternative Pre-mRNA Splicing Regulation in Neuronal Development and Function |
Q34673242 | Fishing for a mechanism: using zebrafish to understand spinal muscular atrophy |
Q34342044 | GEMINs: potential therapeutic targets for spinal muscular atrophy? |
Q38197233 | Gem depletion: amyotrophic lateral sclerosis and spinal muscular atrophy crossover. |
Q24536283 | Gemin proteins are required for efficient assembly of Sm-class ribonucleoproteins. |
Q37035061 | Gemin3 is an essential gene required for larval motor function and pupation in Drosophila |
Q28115127 | Gemin5 Binds to the Survival Motor Neuron mRNA to Regulate SMN Expression |
Q24306053 | Gemin8 is required for the architecture and function of the survival motor neuron complex |
Q35670763 | Genetic Interactions between the Members of the SMN-Gemins Complex in Drosophila |
Q39072801 | Genome-wide identification of mRNAs associated with the protein SMN whose depletion decreases their axonal localization |
Q39418520 | Global detection and identification of developmental stage specific transcripts in mouse brain using subtractive cross-screening algorithm |
Q38855284 | Hypo- and Hyper-Assembly Diseases of RNA-Protein Complexes |
Q44501110 | Impaired spliceosomal UsnRNP assembly leads to Sm mRNA down-regulation and Sm protein degradation |
Q36559321 | Implication of the SMN complex in the biogenesis and steady state level of the signal recognition particle |
Q28116617 | In vitro and in cellulo evidences for association of the survival of motor neuron complex with the fragile X mental retardation protein |
Q42163950 | In vivo mutation of pre-mRNA processing factor 8 (Prpf8) affects transcript splicing, cell survival and myeloid differentiation |
Q33355418 | Inactivation of the SMN complex by oxidative stress |
Q37218543 | Increased susceptibility of spinal muscular atrophy fibroblasts to camptothecin is p53-independent |
Q35758087 | Inhibition of U snRNP assembly by a virus-encoded proteinase |
Q24608490 | Intronic miR-26b controls neuronal differentiation by repressing its host transcript, ctdsp2 |
Q39039061 | Linking amyotrophic lateral sclerosis and spinal muscular atrophy through RNA-transcriptome homeostasis: a genomics perspective |
Q36924730 | Molecular functions of the SMN complex |
Q36924727 | Molecular mechanisms of spinal muscular atrophy |
Q83227267 | Multifaceted roles of microRNAs: From motor neuron generation in embryos to degeneration in spinal muscular atrophy |
Q91594404 | Multiparametric rapid screening of neuronal process pathology for drug target identification in HSP patient-specific neurons |
Q57924709 | Mutations in the Survival Motor Neuron (SMN) Protein Alter the Dynamic Nature of Nuclear Bodies |
Q91678686 | Negative cooperativity between Gemin2 and RNA provides insights into RNA selection and the SMN complex's release in snRNP assembly |
Q38222192 | Nervous translation, do you get the message? A review of mRNPs, mRNA-protein interactions and translational control within cells of the nervous system |
Q51744183 | Neurochondrin interacts with the SMN protein suggesting a novel mechanism for Spinal Muscular Atrophy pathology. |
Q39521045 | Neuronal SMN expression corrects spinal muscular atrophy in severe SMA mice while muscle-specific SMN expression has no phenotypic effect |
Q28261807 | Novel aminoglycosides increase SMN levels in spinal muscular atrophy fibroblasts |
Q24336921 | Ongoing U snRNP biogenesis is required for the integrity of Cajal bodies |
Q33840295 | Proteomic assessment of a cell model of spinal muscular atrophy |
Q34060813 | Quality control of assembly-defective U1 snRNAs by decapping and 5'-to-3' exonucleolytic digestion. |
Q29615183 | RNA and disease |
Q36827345 | RNA transport and localized protein synthesis in neurological disorders and neural repair |
Q34993977 | RNA-Seq analysis in mutant zebrafish reveals role of U1C protein in alternative splicing regulation |
Q37577398 | RNA-sequencing of a mouse-model of spinal muscular atrophy reveals tissue-wide changes in splicing of U12-dependent introns |
Q39233232 | Recapitulation of spinal motor neuron-specific disease phenotypes in a human cell model of spinal muscular atrophy. |
Q45973869 | Reduced levels of survival motor neuron protein leads to aberrant motoneuron growth in a Xenopus model of muscular atrophy. |
Q40089875 | Refined characterization of the expression and stability of the SMN gene products. |
Q21142695 | Restoration of full-length SMN promoted by adenoviral vectors expressing RNA antisense oligonucleotides embedded in U7 snRNAs |
Q33300324 | Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs |
Q41864190 | RioK1, a new interactor of protein arginine methyltransferase 5 (PRMT5), competes with pICln for binding and modulates PRMT5 complex composition and substrate specificity. |
Q42575278 | Role of pICLn in methylation of Sm proteins by PRMT5. |
Q41835189 | SMA-causing missense mutations in survival motor neuron (Smn) display a wide range of phenotypes when modeled in Drosophila. |
Q91854567 | SMN complex member Gemin3 self-interacts and has a functional relationship with ALS-linked proteins TDP-43, FUS and Sod1 |
Q38207417 | SMN control of RNP assembly: from post-transcriptional gene regulation to motor neuron disease. |
Q35039158 | SMN deficiency alters Nrxn2 expression and splicing in zebrafish and mouse models of spinal muscular atrophy |
Q36746476 | SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing |
Q29013847 | SMN in spinal muscular atrophy and snRNP biogenesis |
Q47885235 | SMN regulation in SMA and in response to stress: new paradigms and therapeutic possibilities. |
Q34056100 | SMN requirement for synaptic vesicle, active zone and microtubule postnatal organization in motor nerve terminals |
Q37272307 | SMN-assisted assembly of snRNP-specific Sm cores in trypanosomes |
Q36575678 | Sm protein methylation is dispensable for snRNP assembly in Drosophila melanogaster. |
Q49389328 | Small-molecule flunarizine increases SMN protein in nuclear Cajal bodies and motor function in a mouse model of spinal muscular atrophy |
Q81303651 | Smn depletion alters profilin II expression and leads to upregulation of the RhoA/ROCK pathway and defects in neuronal integrity |
Q33914362 | Specific splicing defects in S. pombe carrying a degron allele of the Survival of Motor Neuron gene |
Q39084725 | Spectrum of neuropathophysiology in spinal muscular atrophy type I |
Q33775770 | Spinal Muscular Atrophy: From Defective Chaperoning of snRNP Assembly to Neuromuscular Dysfunction |
Q37033848 | Spinal muscular atrophy and a model for survival of motor neuron protein function in axonal ribonucleoprotein complexes |
Q33345749 | Spinal muscular atrophy. |
Q28302547 | Spinal muscular atrophy: SMN2 pre-mRNA splicing corrected by a U7 snRNA derivative carrying a splicing enhancer sequence |
Q38123249 | Spinal muscular atrophy: a motor neuron disorder or a multi-organ disease? |
Q35990221 | Spinal muscular atrophy: the role of SMN in axonal mRNA regulation. |
Q33785933 | Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick? |
Q37100836 | Splice-site pairing is an intrinsically high fidelity process. |
Q37994369 | Spliceosomal small nuclear ribonucleoprotein biogenesis defects and motor neuron selectivity in spinal muscular atrophy |
Q35563254 | Splicing changes in SMA mouse motoneurons and SMN-depleted neuroblastoma cells: evidence for involvement of splicing regulatory proteins |
Q34582131 | Splicing in disease: disruption of the splicing code and the decoding machinery |
Q47073257 | Survival motor neuron function in motor axons is independent of functions required for small nuclear ribonucleoprotein biogenesis. |
Q21563387 | Survival motor neuron protein regulates stem cell division, proliferation, and differentiation in Drosophila |
Q37875033 | Synaptic defects in spinal muscular atrophy animal models |
Q36174725 | TIS7 induces transcriptional cascade of methylosome components required for muscle differentiation |
Q30540382 | Temporal requirement for SMN in motoneuron development. |
Q41120778 | The C-terminus of ICln is natively disordered but displays local structural preformation |
Q35082654 | The Gemin associates of survival motor neuron are required for motor function in Drosophila |
Q79828985 | The SMN binding protein Gemin2 is not involved in motor axon outgrowth |
Q39802654 | The SMN protein is a key regulator of nuclear architecture in differentiating neuroblastoma cells. |
Q24604444 | The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy |
Q42439185 | The craniosacral progression of muscle development influences the emergence of neuromuscular junction alterations in a severe murine model for spinal muscular atrophy. |
Q47073239 | The heterogeneous nuclear ribonucleoprotein-R is necessary for axonal beta-actin mRNA translocation in spinal motor neurons |
Q37774045 | The role of RNA processing in the pathogenesis of motor neuron degeneration. |
Q64444095 | The role of survival motor neuron protein (SMN) in protein homeostasis |
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Q37935721 | The search for evolutionary developmental origins of aging in zebrafish: a novel intersection of developmental and senescence biology in the zebrafish model system |
Q35910079 | The spliceosome assembly factor GEMIN2 attenuates the effects of temperature on alternative splicing and circadian rhythms. |
Q36190026 | The survival motor neuron gene smn-1 interacts with the U2AF large subunit gene uaf-1 to regulate Caenorhabditis elegans lifespan and motor functions |
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Q36428761 | Therapeutics development for spinal muscular atrophy |
Q36974709 | Transcriptional enhancement of Smn levels in motoneurons is crucial for proper axon morphology in zebrafish |
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