scholarly article | Q13442814 |
P50 | author | Clemens Steegborn | Q28320688 |
Michael Sendtner | Q32426109 | ||
P2093 | author name string | Sibylle Jablonka | |
Wilfried Rossoll | |||
Ann-Kathrin Kröning | |||
Uta-Maria Ohndorf | |||
P433 | issue | 1 | |
P407 | language of work or name | English | Q1860 |
P921 | main subject | muscular atrophy | Q2844600 |
spinal muscular atrophy | Q580290 | ||
P304 | page(s) | 93-105 | |
P577 | publication date | 2002-01-01 | |
P1433 | published in | Human Molecular Genetics | Q2720965 |
P1476 | title | 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? | |
P478 | volume | 11 |
Q34490238 | A Multi-Exon-Skipping Detection Assay Reveals Surprising Diversity of Splice Isoforms of Spinal Muscular Atrophy Genes |
Q24657021 | A SMN missense mutation complements SMN2 restoring snRNPs and rescuing SMA mice |
Q34341178 | A feedback loop regulates splicing of the spinal muscular atrophy-modifying gene, SMN2. |
Q47786052 | A new cis-acting motif is required for the axonal SMN-dependent Anxa2 mRNA localization. |
Q58379271 | A novel function for the survival motoneuron protein as a translational regulator |
Q38812591 | A novel role for CARM1 in promoting nonsense-mediated mRNA decay: potential implications for spinal muscular atrophy. |
Q28244480 | A short antisense oligonucleotide masking a unique intronic motif prevents skipping of a critical exon in spinal muscular atrophy |
Q34241874 | A single administration of morpholino antisense oligomer rescues spinal muscular atrophy in mouse |
Q36323111 | A transgene carrying an A2G missense mutation in the SMN gene modulates phenotypic severity in mice with severe (type I) spinal muscular atrophy. |
Q36506599 | Abnormal mitochondrial transport and morphology as early pathological changes in human models of spinal muscular atrophy. |
Q38073951 | Actin Isoforms in Neuronal Development and Function |
Q36558108 | Activation of the lifespan regulator p66Shc through reversible disulfide bond formation |
Q48586550 | Active transport of the survival motor neuron protein and the role of exon-7 in cytoplasmic localization. |
Q35167319 | Advances in therapeutic development for spinal muscular atrophy |
Q38668630 | Advances in understanding the role of disease-associated proteins in spinal muscular atrophy. |
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 |
Q47406275 | Artificial intelligence in neurodegenerative disease research: use of IBM Watson to identify additional RNA-binding proteins altered in amyotrophic lateral sclerosis |
Q34082155 | Assays for the identification and prioritization of drug candidates for spinal muscular atrophy |
Q39665747 | Assisted RNP assembly: SMN and PRMT5 complexes cooperate in the formation of spliceosomal UsnRNPs |
Q37264930 | Autophagy negatively regulates early axon growth in cortical neurons |
Q34708286 | Axonal Regeneration and Neuronal Function Are Preserved in Motor Neurons Lacking ß-Actin In Vivo |
Q52321880 | Axonal mRNA transport and translation at a glance. |
Q35629448 | Axonal-SMN (a-SMN), a protein isoform of the survival motor neuron gene, is specifically involved in axonogenesis. |
Q38780715 | COPI transport complexes bind to specific RNAs in neuronal cells. |
Q36659685 | Cell-autonomous axon growth of young motoneurons is triggered by a voltage-gated sodium channel |
Q82029153 | Chapter 7 Spinal muscular atrophies and hereditary motor neuropathies |
Q24307701 | Coupled in vitro import of U snRNPs and SMN, the spinal muscular atrophy protein |
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. |
Q28203025 | Determinants of the interaction of the spinal muscular atrophy disease protein SMN with the dimethylarginine-modified box H/ACA small nucleolar ribonucleoprotein GAR1 |
Q39337972 | Developmental regulation of SMN expression: pathophysiological implications and perspectives for therapy development in spinal muscular atrophy |
Q36002482 | Different motif requirements for the localization zipcode element of β-actin mRNA binding by HuD and ZBP1. |
Q93178436 | Disruption of RNA Metabolism in Neurological Diseases and Emerging Therapeutic Interventions |
Q39092025 | Diverse role of survival motor neuron protein |
Q34240890 | Drosophila Syncrip modulates the expression of mRNAs encoding key synaptic proteins required for morphology at the neuromuscular junction |
Q34172502 | Drug Discovery and Development for Spinal Muscular Atrophy: Lessons from Screening Approaches and Future Challenges for Clinical Development |
Q58826477 | Drug discovery for spinal muscular atrophy |
Q42414886 | Dynamic remodelling of human 7SK snRNP controls the nuclear level of active P-TEFb |
Q24604173 | Dynamics of axonal mRNA transport and implications for peripheral nerve regeneration |
Q37529892 | Dynamics of survival of motor neuron (SMN) protein interaction with the mRNA-binding protein IMP1 facilitates its trafficking into motor neuron axons |
Q97531615 | Efficient recovery of the RNA-bound proteome and protein-bound transcriptome using phase separation (OOPS) |
Q92513205 | Emerging roles of histone modifications and HDACs in RNA splicing |
Q28206060 | Evidence for a modifying pathway in SMA discordant families: reduced SMN level decreases the amount of its interacting partners and Htra2-beta1 |
Q39359080 | Expanding Axonal Transcriptome Brings New Functions for Axonally Synthesized Proteins in Health and Disease |
Q34673242 | Fishing for a mechanism: using zebrafish to understand spinal muscular atrophy |
Q37783316 | Functional diversity of the hnRNPs: past, present and perspectives |
Q24536283 | Gemin proteins are required for efficient assembly of Sm-class ribonucleoproteins. |
Q36967887 | Genetic circuitry of Survival motor neuron, the gene underlying spinal muscular atrophy |
Q99556544 | Genetic modifiers ameliorate endocytic and neuromuscular defects in a model of spinal muscular atrophy |
Q34576428 | Genome-Wide Pathway Analysis Reveals Different Signaling Pathways between Secreted Lactoferrin and Intracellular Delta-Lactoferrin |
Q39072801 | Genome-wide identification of mRNAs associated with the protein SMN whose depletion decreases their axonal localization |
Q37431043 | HRP-2, the Caenorhabditis elegans homolog of mammalian heterogeneous nuclear ribonucleoproteins Q and R, is an alternative splicing factor that binds to UCUAUC splicing regulatory elements |
Q64237309 | Heterogeneous nuclear ribonucleoproteins R and Q accumulate in pathological inclusions in FTLD-FUS |
Q28303272 | Hormone response element binding proteins: novel regulators of vitamin D and estrogen signaling |
Q28239705 | Human hnRNP Q re-localizes to cytoplasmic granules upon PMA, thapsigargin, arsenite and heat-shock treatments |
Q36972709 | Human pathologies associated with defective RNA transport and localization in the nervous system |
Q28207148 | Hyperphosphorylated C-terminal repeat domain-associating proteins in the nuclear proteome link transcription to DNA/chromatin modification and RNA processing |
Q36327383 | IPLEX Administration Improves Motor Neuron Survival and Ameliorates Motor Functions in a Severe Mouse Model of Spinal Muscular Atrophy |
Q24299793 | Identification and characterization of Gemin7, a novel component of the survival of motor neuron complex |
Q35567374 | 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 |
Q41783290 | Identifying specific protein interaction partners using quantitative mass spectrometry and bead proteomes |
Q28116617 | In vitro and in cellulo evidences for association of the survival of motor neuron complex with the fragile X mental retardation protein |
Q37218543 | Increased susceptibility of spinal muscular atrophy fibroblasts to camptothecin is p53-independent |
Q34284670 | Infantile spinal muscular atrophy with respiratory distress type 1 (SMARD1). |
Q58621285 | Interaction modulation through arrays of clustered methyl-arginine protein modifications |
Q35064376 | Interaction of survival of motor neuron (SMN) and HuD proteins with mRNA cpg15 rescues motor neuron axonal deficits. |
Q55354265 | Interactome analyses revealed that the U1 snRNP machinery overlaps extensively with the RNAP II machinery and contains multiple ALS/SMA-causative proteins. |
Q60215185 | Isolation and enrichment of embryonic mouse motoneurons from the lumbar spinal cord of individual mouse embryos |
Q61816592 | Key role of SMN/SYNCRIP and RNA-Motif 7 in spinal muscular atrophy: RNA-Seq and motif analysis of human motor neurons |
Q35567371 | Knockdown of the survival motor neuron (Smn) protein in zebrafish causes defects in motor axon outgrowth and pathfinding. |
Q36142393 | Low levels of Survival Motor Neuron protein are sufficient for normal muscle function in the SMNΔ7 mouse model of SMA. |
Q38134411 | Mechanisms for axon maintenance and plasticity in motoneurons: alterations in motoneuron disease |
Q34339435 | Minute virus of mice small nonstructural protein NS2 interacts and colocalizes with the Smn protein |
Q90674893 | Molecular Factors Involved in Spinal Muscular Atrophy Pathways as Possible Disease-modifying Candidates |
Q35226376 | Molecular and cellular basis of spinal muscular atrophy |
Q30994078 | Molecular determinants of survival motor neuron (SMN) protein cleavage by the calcium-activated protease, calpain |
Q36457297 | Motoneuron injury and repair: New perspectives on gonadal steroids as neurotherapeutics |
Q92510092 | Motor neuron biology and disease: A current perspective on infantile-onset spinal muscular atrophy |
Q42123203 | Multiprotein complexes of the survival of motor neuron protein SMN with Gemins traffic to neuronal processes and growth cones of motor neurons |
Q35891195 | Negative regulation of RhoA translation and signaling by hnRNP-Q1 affects cellular morphogenesis |
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 |
Q38197609 | Neurodegeneration in spinal muscular atrophy: from disease phenotype and animal models to therapeutic strategies and beyond |
Q36013982 | Neurogenic and Myogenic Contributions to Hereditary Motor Neuron Disease |
Q39521045 | Neuronal SMN expression corrects spinal muscular atrophy in severe SMA mice while muscle-specific SMN expression has no phenotypic effect |
Q35095113 | Poly(A) RNA-binding proteins and polyadenosine RNA: new members and novel functions |
Q47755072 | Predominant expression of exon 7 skipped SMN mRNAs in lung based on analysis of transcriptome sequencing datasets |
Q34388403 | Presynaptic localization of Smn and hnRNP R in axon terminals of embryonic and postnatal mouse motoneurons |
Q28187838 | Protein phosphatase 4 interacts with the Survival of Motor Neurons complex and enhances the temporal localisation of snRNPs |
Q37720436 | RNA processing pathways in amyotrophic lateral sclerosis |
Q36827345 | RNA transport and localized protein synthesis in neurological disorders and neural repair |
Q38111759 | RNA-binding proteins and translational regulation in axons and growth cones |
Q37577398 | RNA-sequencing of a mouse-model of spinal muscular atrophy reveals tissue-wide changes in splicing of U12-dependent introns |
Q34762623 | Repair of pre-mRNA splicing: prospects for a therapy for spinal muscular atrophy. |
Q24520629 | Rhythmic serotonin N-acetyltransferase mRNA degradation is essential for the maintenance of its circadian oscillation |
Q35039158 | SMN deficiency alters Nrxn2 expression and splicing in zebrafish and mouse models of spinal muscular atrophy |
Q55053799 | SMN deficiency reduces cellular ability to form stress granules, sensitizing cells to stress. |
Q29013847 | SMN in spinal muscular atrophy and snRNP biogenesis |
Q40314348 | SMN, the product of the spinal muscular atrophy-determining gene, is expressed widely but selectively in the developing human forebrain |
Q34312156 | SMN-Inducing Compounds For The Treatment Of Spinal Muscular Atrophy |
Q51969285 | SYNCRIP, a component of dendritically localized mRNPs, binds to the translation regulator BC200 RNA. |
Q31127282 | SYNCRIP, a member of the heterogeneous nuclear ribonucleoprotein family, is involved in mouse hepatitis virus RNA synthesis |
Q33356209 | Small nuclear RNAs and mRNAs: linking RNA processing and transport to spinal muscular atrophy |
Q81303651 | Smn depletion alters profilin II expression and leads to upregulation of the RhoA/ROCK pathway and defects in neuronal integrity |
Q28576647 | Smn, the spinal muscular atrophy-determining gene product, modulates axon growth and localization of beta-actin mRNA in growth cones of motoneurons |
Q54765007 | Solving the puzzle of spinal muscular atrophy: what are the missing pieces? |
Q37033848 | Spinal muscular atrophy and a model for survival of motor neuron protein function in axonal ribonucleoprotein complexes |
Q39398228 | Spinal muscular atrophy pathogenic mutations impair the axonogenic properties of axonal-survival of motor neuron. |
Q38489298 | Spinal muscular atrophy--recent therapeutic advances for an old challenge. |
Q33345749 | Spinal muscular atrophy. |
Q37302832 | Spinal muscular atrophy: an update on therapeutic progress |
Q26824894 | Spinal muscular atrophy: from gene discovery to clinical trials |
Q35340133 | Spinal muscular atrophy: journeying from bench to bedside |
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? |
Q27009581 | Splicing therapy for neuromuscular disease |
Q37805736 | Subcellular communication through RNA transport and localized protein synthesis |
Q34363918 | Subcellular transcriptome alterations in a cell culture model of spinal muscular atrophy point to widespread defects in axonal growth and presynaptic differentiation |
Q39125261 | Supraphysiological expression of survival motor neuron protein from an adenovirus vector does not adversely affect cell function. |
Q21563387 | Survival Motor Neuron Protein Regulates Stem Cell Division, Proliferation, and Differentiation in Drosophila |
Q47073257 | Survival motor neuron function in motor axons is independent of functions required for small nuclear ribonucleoprotein biogenesis. |
Q30586954 | Syncrip/hnRNP Q influences synaptic transmission and regulates BMP signaling at the Drosophila neuromuscular synapse |
Q58454162 | Systematic Analysis of Gene Expression Profiles Controlled by hnRNP Q and hnRNP R, Two Closely Related Human RNA Binding Proteins Implicated in mRNA Processing Mechanisms |
Q39274901 | The Actin Cytoskeleton in SMA and ALS: How Does It Contribute to Motoneuron Degeneration? |
Q30499358 | The COPI vesicle complex binds and moves with survival motor neuron within axons |
Q24648050 | The RNA binding protein hnRNP Q modulates the utilization of exon 7 in the survival motor neuron 2 (SMN2) gene |
Q28204829 | The SMN complex, an assemblyosome of ribonucleoproteins |
Q27329644 | The Spinal Muscular Atrophy Disease Protein SMN Is Linked to the Golgi Network |
Q33858798 | The Survival of Motor Neuron Protein Acts as a Molecular Chaperone for mRNP Assembly |
Q28242279 | The benzamide M344, a novel histone deacetylase inhibitor, significantly increases SMN2 RNA/protein levels in spinal muscular atrophy cells |
Q28572959 | The expression pattern of heterogeneous nuclear ribonucleoprotein R in rat retina |
Q104111385 | The functional organization of axonal mRNA transport and translation |
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. |
Q54560146 | The spinal muscular atrophy disease protein SMN is linked to the Rho-kinase pathway via profilin |
Q28570605 | The survival of motor neuron (SMN) protein interacts with the mRNA-binding protein HuD and regulates localization of poly(A) mRNA in primary motor neuron axons. |
Q34122981 | Therapy development in spinal muscular atrophy |
Q47427810 | To the end of the line: Axonal mRNA transport and local translation in health and neurodegenerative disease. |
Q36974709 | Transcriptional enhancement of Smn levels in motoneurons is crucial for proper axon morphology in zebrafish |
Q40727142 | Ultrastructure of nuclear aggregates formed by expressing an expanded polyglutamine |
Q58800979 | Unraveling the Pathways to Neuronal Homeostasis and Disease: Mechanistic Insights into the Role of RNA-Binding Proteins and Associated Factors |
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Q52366535 | hnRNP R and its main interactor, the noncoding RNA 7SK, coregulate the axonal transcriptome of motoneurons |
Q36574685 | hnRNP-Q1 represses nascent axon growth in cortical neurons by inhibiting Gap-43 mRNA translation |
Q40024849 | hnRNP-R regulates the PMA-induced c-fos expression in retinal cells. |
Q38469185 | mRNA metabolism and neuronal disease |
Q49808601 | mRNP assembly, axonal transport, and local translation in neurodegenerative diseases |
Q36331590 | α-COP binding to the survival motor neuron protein SMN is required for neuronal process outgrowth |
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