| scholarly article | Q13442814 |
| P2093 | author name string | Jin Wang | |
| Gideon Dreyfuss | |||
| Lili Wan | |||
| Jeongsik Yong | |||
| Daniel J Battle | |||
| Stephen J Kolb | |||
| Amelie K Gubitz | |||
| P2860 | cites work | A novel function for SMN, the spinal muscular atrophy disease gene product, in pre-mRNA splicing | Q22008514 |
| Gemin3: A novel DEAD box protein that interacts with SMN, the spinal muscular atrophy gene product, and is a component of gems | Q22010941 | ||
| Gemin4. A novel component of the SMN complex that is found in both gems and nucleoli | Q22253358 | ||
| Gemin5, a novel WD repeat protein component of the SMN complex that binds Sm proteins | Q24291913 | ||
| The spinal muscular atrophy disease gene product, SMN, and its associated protein SIP1 are in a complex with spliceosomal snRNP proteins | Q24316085 | ||
| The SMN-SIP1 complex has an essential role in spliceosomal snRNP biogenesis | Q24316121 | ||
| A novel nuclear structure containing the survival of motor neurons protein | Q24324247 | ||
| The Sm domain is an ancient RNA-binding motif with oligo(U) specificity | Q24618608 | ||
| A functional interaction between the survival motor neuron complex and RNA polymerase II | Q24674951 | ||
| Crystal structures of two Sm protein complexes and their implications for the assembly of the spliceosomal snRNPs | Q27639794 | ||
| The economics of ribosome biosynthesis in yeast | Q28131645 | ||
| Essential role for the tudor domain of SMN in spliceosomal U snRNP assembly: implications for spinal muscular atrophy | Q28137718 | ||
| Structure and assembly of the spliceosomal small nuclear ribonucleoprotein particles | Q28143806 | ||
| Spliceosomal UsnRNP biogenesis, structure and function | Q28188106 | ||
| Arrangement of RNA and proteins in the spliceosomal U1 small nuclear ribonucleoprotein particle | Q28201289 | ||
| Why do cells need an assembly machine for RNA-protein complexes? | Q28260526 | ||
| Cap trimethylation of U snRNA is cytoplasmic and dependent on U snRNP protein binding | Q28622300 | ||
| m3G cap hypermethylation of U1 small nuclear ribonucleoprotein (snRNP) in vitro: evidence that the U1 small nuclear RNA-(guanosine-N2)-methyltransferase is a non-snRNP cytoplasmic protein that requires a binding site on the Sm core domain | Q28678756 | ||
| Identification and characterization of a spinal muscular atrophy-determining gene | Q29547495 | ||
| Monoclonal antibodies to nucleic acid-containing cellular constituents: probes for molecular biology and autoimmune disease | Q29616423 | ||
| A cell system with targeted disruption of the SMN gene: functional conservation of the SMN protein and dependence of Gemin2 on SMN. | Q30976876 | ||
| U2 RNA shares a structural domain with U1, U4, and U5 RNAs | Q33928202 | ||
| In vitro assembly of U1 snRNPs | Q33929868 | ||
| The Y14 protein communicates to the cytoplasm the position of exon-exon junctions | Q34077558 | ||
| Sequence-specific interaction of U1 snRNA with the SMN complex | Q34086103 | ||
| The spliceosome: the most complex macromolecular machine in the cell? | Q34278742 | ||
| How cells coordinate growth and division | Q34374885 | ||
| The survival motor neuron protein in spinal muscular atrophy | Q34435656 | ||
| Essential role for the SMN complex in the specificity of snRNP assembly | Q34529450 | ||
| Functions of U-snRNPs | Q37854929 | ||
| Structure of spliceosomal snRNPs and their role in pre-mRNA splicing | Q37862777 | ||
| Spliceosomal U snRNP core assembly: Sm proteins assemble onto an Sm site RNA nonanucleotide in a specific and thermodynamically stable manner | Q38320484 | ||
| The determinants for Sm protein binding to Xenopus U1 and U5 snRNAs are complex and non-identical. | Q38322971 | ||
| The U2 small nuclear ribonucleoprotein particle associates with nuclear factors in a pre-mRNA independent reaction. | Q38332855 | ||
| Assisted RNP assembly: SMN and PRMT5 complexes cooperate in the formation of spliceosomal UsnRNPs | Q39665747 | ||
| U2 small nuclear RNP assembly in vitro | Q40450724 | ||
| Valproic acid increases SMN levels in spinal muscular atrophy patient cells. | Q40620622 | ||
| Nucleocytoplasmic transport and snRNP assembly | Q40776319 | ||
| Nucleo-cytoplasmic transport of U snRNPs: definition of a nuclear location signal in the Sm core domain that binds a transport receptor independently of the m3G cap | Q40871968 | ||
| The snRNP core assembly pathway: identification of stable core protein heteromeric complexes and an snRNP subcore particle in vitro | Q41064906 | ||
| Correlation between severity and SMN protein level in spinal muscular atrophy | Q41102726 | ||
| The neurobiology of childhood spinal muscular atrophy. | Q41489423 | ||
| A multiprotein complex mediates the ATP-dependent assembly of spliceosomal U snRNPs | Q43803910 | ||
| U1 small nuclear RNA genes are subject to dosage compensation in mouse cells | Q45071327 | ||
| The trimethylguanosine cap structure of U1 snRNA is a component of a bipartite nuclear targeting signal | Q57378192 | ||
| In vitro reconstitution of U1 and U2 snRNPs from isolated proteins and snRNA | Q67595512 | ||
| An essential signaling role for the m3G cap in the transport of U1 snRNP to the nucleus | Q68425864 | ||
| Spinal muscular atrophy | Q74515361 | ||
| Spinal muscular atrophy | Q75190358 | ||
| P433 | issue | 13 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | spinal muscular atrophy | Q580290 |
| muscular atrophy | Q2844600 | ||
| P304 | page(s) | 5543-5551 | |
| P577 | publication date | 2005-07-01 | |
| P1433 | published in | Molecular and Cellular Biology | Q3319478 |
| P1476 | title | The survival of motor neurons protein determines the capacity for snRNP assembly: biochemical deficiency in spinal muscular atrophy | |
| P478 | volume | 25 |
| 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 |
| Q36806948 | A U1 snRNP-specific assembly pathway reveals the SMN complex as a versatile hub for RNP exchange |
| Q28274685 | A degron created by SMN2 exon 7 skipping is a principal contributor to spinal muscular atrophy severity |
| Q44430737 | A new communication hub in the RNA world |
| Q33232855 | A novel cell immunoassay to measure survival of motor neurons protein in blood cells |
| Q36995340 | A novel magnetic bead-based assay with high sensitivity and selectivity for analysis of telomerase in exfoliated cells from patients with bladder and colon cancer. |
| Q35826664 | A quantitative high-throughput in vitro splicing assay identifies inhibitors of spliceosome catalysis |
| Q35114564 | ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP. |
| Q34427456 | Abnormal interaction of motor neuropathy-associated mutant HspB8 (Hsp22) forms with the RNA helicase Ddx20 (gemin3). |
| Q37236684 | Abnormal motor phenotype in the SMNDelta7 mouse model of spinal muscular atrophy |
| Q41856470 | An SMN-dependent U12 splicing event essential for motor circuit function |
| Q34082155 | Assays for the identification and prioritization of drug candidates for spinal muscular atrophy |
| Q36409666 | Behavioral and electrophysiological outcomes of tissue-specific Smn knockdown in Drosophila melanogaster |
| Q37913270 | Biogenesis of spliceosomal small nuclear ribonucleoproteins |
| Q42144658 | Changes in intranuclear mobility of mature snRNPs provide a mechanism for splicing defects in spinal muscular atrophy. |
| Q36777301 | Chaperoning ribonucleoprotein biogenesis in health and disease |
| Q82029153 | Chapter 7 Spinal muscular atrophies and hereditary motor neuropathies |
| Q28506113 | Chondrolectin affects cell survival and neuronal outgrowth in in vitro and in vivo models of spinal muscular atrophy |
| Q26750803 | Copy Number Variations in the Survival Motor Neuron Genes: Implications for Spinal Muscular Atrophy and Other Neurodegenerative Diseases |
| Q35715801 | Different Stability and Proteasome-Mediated Degradation Rate of SMN Protein Isoforms. |
| Q93178436 | Disruption of RNA Metabolism in Neurological Diseases and Emerging Therapeutic Interventions |
| Q58826477 | Drug discovery for spinal muscular atrophy |
| Q37353218 | Dysregulation of synaptogenesis genes antecedes motor neuron pathology in spinal muscular atrophy. |
| Q33245954 | EST-based identification of genes expressed in brain and spinal cord of Gekko japonicus, a species demonstrating intrinsic capacity of spinal cord regeneration |
| Q30492560 | Effects of 2,4-diaminoquinazoline derivatives on SMN expression and phenotype in a mouse model for spinal muscular atrophy |
| Q37157517 | El Sur también existe: processing RNA in the Argentine Patagonia. Meetings on 'Cell Biology, Signaling and Alternative Splicing' and 'Gene Expression and RNA Processing' |
| Q38712609 | Establishing a reference dataset for the authentication of spinal muscular atrophy cell lines using STR profiling and digital PCR. |
| Q46793951 | Exercise-induced activation of NMDA receptor promotes motor unit development and survival in a type 2 spinal muscular atrophy model mouse. |
| 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 |
| Q28115894 | Gemin5 delivers snRNA precursors to the SMN complex for snRNP biogenesis |
| Q28117609 | Gemin5-snRNA interaction reveals an RNA binding function for WD repeat domains |
| Q24306053 | Gemin8 is required for the architecture and function of the survival motor neuron complex |
| Q39072801 | Genome-wide identification of mRNAs associated with the protein SMN whose depletion decreases their axonal localization |
| Q24307755 | Gle1 functions during mRNA export in an oligomeric complex that is altered in human disease |
| Q52605306 | Impaired minor tri-snRNP assembly generates differential splicing defects of U12-type introns in lymphoblasts derived from a type I SMA patient |
| 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 |
| Q33355418 | Inactivation of the SMN complex by oxidative stress |
| Q37218543 | Increased susceptibility of spinal muscular atrophy fibroblasts to camptothecin is p53-independent |
| Q49304342 | Increasing Agrin Function Antagonizes Muscle Atrophy and Motor Impairment in Spinal Muscular Atrophy. |
| Q35758087 | Inhibition of U snRNP assembly by a virus-encoded proteinase |
| Q21562542 | Modeling spinal muscular atrophy in Drosophila |
| Q35003601 | Modeling spinal muscular atrophy in Drosophila links Smn to FGF signaling |
| Q36924730 | Molecular functions of the SMN complex |
| Q36924727 | Molecular mechanisms of spinal muscular atrophy |
| Q37400655 | More than a bystander: the contributions of intrinsic skeletal muscle defects in motor neuron diseases. |
| Q92510092 | Motor neuron biology and disease: A current perspective on infantile-onset spinal muscular atrophy |
| Q51744183 | Neurochondrin interacts with the SMN protein suggesting a novel mechanism for Spinal Muscular Atrophy pathology. |
| Q42852012 | Of SMN in mice and men: a therapeutic opportunity |
| Q104567661 | Pathogenesis and therapeutic targets in spinal muscular atrophy (SMA) |
| Q28475498 | Phase II open label study of valproic acid in spinal muscular atrophy |
| Q40520534 | Post-transcriptional modification of spliceosomal RNAs is normal in SMN-deficient cells |
| Q35122217 | Quality assessment and control of tissue specific RNA-seq libraries of Drosophila transgenic RNAi models |
| Q34060813 | Quality control of assembly-defective U1 snRNAs by decapping and 5'-to-3' exonucleolytic digestion. |
| Q29615183 | RNA and disease |
| Q34360790 | RNA exodus to Israel: RNA controlling function in the far reaches of the neuron. Workshop on RNA control on neuronal function |
| Q36846566 | RNA processing defects associated with diseases of the motor neuron |
| Q41846402 | RNA reigns in neurons |
| Q35991646 | Reconstitution of the human U snRNP assembly machinery reveals stepwise Sm protein organization |
| Q40089875 | Refined characterization of the expression and stability of the SMN gene products. |
| Q33300324 | Ribonucleoprotein assembly defects correlate with spinal muscular atrophy severity and preferentially affect a subset of spliceosomal snRNPs |
| Q21562176 | SMA CARNI-VAL trial part I: double-blind, randomized, placebo-controlled trial of L-carnitine and valproic acid in spinal muscular atrophy |
| Q21560963 | SMA CARNIVAL TRIAL PART II: a prospective, single-armed trial of L-carnitine and valproic acid in ambulatory children with spinal muscular atrophy |
| Q38207417 | SMN control of RNP assembly: from post-transcriptional gene regulation to motor neuron disease. |
| Q36746476 | SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing |
| 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 |
| Q28237155 | SMN-independent subunits of the SMN complex. Identification of a small nuclear ribonucleoprotein assembly intermediate |
| 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 |
| Q37610527 | Spinal Muscular Atrophy Biomarker Measurements from Blood Samples in a Clinical Trial of Valproic Acid in Ambulatory Adults |
| Q33775770 | Spinal Muscular Atrophy: From Defective Chaperoning of snRNP Assembly to Neuromuscular Dysfunction |
| Q26861377 | Spinal muscular atrophy: development and implementation of potential treatments |
| Q34945009 | Spinal muscular atrophy: diagnosis and management in a new therapeutic era |
| Q33785933 | Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick? |
| Q37994369 | Spliceosomal small nuclear ribonucleoprotein biogenesis defects and motor neuron selectivity in spinal muscular atrophy |
| Q36602121 | Spliceosome integrity is defective in the motor neuron diseases ALS and SMA. |
| Q34353625 | Splicing of a critical exon of human Survival Motor Neuron is regulated by a unique silencer element located in the last intron |
| Q24319038 | Structure of a key intermediate of the SMN complex reveals Gemin2's crucial function in snRNP assembly |
| Q47073257 | Survival motor neuron function in motor axons is independent of functions required for small nuclear ribonucleoprotein biogenesis. |
| Q37875033 | Synaptic defects in spinal muscular atrophy animal models |
| Q36298224 | TDRD6 mediates early steps of spliceosome maturation in primary spermatocytes. |
| Q24297645 | The Gemin5 protein of the SMN complex identifies snRNAs |
| Q79828985 | The SMN binding protein Gemin2 is not involved in motor axon outgrowth |
| Q28294580 | The SMN complex: an assembly machine for RNPs |
| Q94503668 | The Small-Molecule Flunarizine in Spinal Muscular Atrophy Patient Fibroblasts Impacts on the Gemin Components of the SMN Complex and TDP43, an RNA-Binding Protein Relevant to Motor Neuron Diseases |
| Q92756257 | The Ubiquitin Proteasome System Is a Key Regulator of Pluripotent Stem Cell Survival and Motor Neuron Differentiation |
| Q24604444 | The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy |
| Q64444095 | The role of survival motor neuron protein (SMN) in protein homeostasis |
| 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 |
| Q24600623 | The survival motor neuron protein forms soluble glycine zipper oligomers |
| Q37363171 | Therapeutic potential of splice-switching oligonucleotides |
| Q36428761 | Therapeutics development for spinal muscular atrophy |
| Q34142599 | Transcriptome profiling of spinal muscular atrophy motor neurons derived from mouse embryonic stem cells |
| Q28289793 | Trichostatin A increases SMN expression and survival in a mouse model of spinal muscular atrophy |
| Q24337867 | Two distinct arginine methyltransferases are required for biogenesis of Sm-class ribonucleoproteins |
| Q29032058 | U1 snRNP protects pre-mRNAs from premature cleavage and polyadenylation |
| Q37563561 | U1A regulates 3' processing of the survival motor neuron mRNA. |
| Q21145787 | WRAP53 is essential for Cajal body formation and for targeting the survival of motor neuron complex to Cajal bodies |
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