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*  JCI - Blocking p62-dependent SMN degradation ameliorates spinal muscular atrophy disease phenotypes
Here, we show that SMN protein is degraded by autophagy. Pharmacological and genetic inhibition of autophagy increases SMN ... showing the levels of the SMN-binding partner gemin2 (member of the SMN complex) (. n. = 3 independent experiments). *. P. , ... The ubiquitin/proteasome system is known to regulate SMN protein levels; however, whether autophagy controls SMN levels remains ... SMN) protein due to SMN1 gene mutations, is a leading cause of infant mortality. Increasing SMN levels ameliorates the disease ...
https://oktaikong.com.www.mobile.jci.org/articles/view/95231/figure/1
*  Konstantinos Tripsianes, Ph.D. | CEITEC - výzkumné centrum
A Novel Protein-Protein Interaction in the RES (REtention and Splicing) Complex. JOURNAL OF BIOLOGICAL CHEMISTRY 289(41), p. ... Structural basis for dimethylarginine recognition by the Tudor domains of human SMN and SPF30 proteins. NATURE STRUCTURAL & ... 2015-2017 Principal investigator, GAČR GJ15-22380Y, "Molecular architecture of protein-DNA complexes involved in Nucleotide ... The multiple Tudor domain-containing protein TDRD1 is a molecular scaffold for mouse Piwi proteins and piRNA biogenesis factors ...
https://www.ceitec.eu/konstantinos-tripsianes-ph-d/u17552
*  Treatment strategies for spinal muscular atrophy : Translational Neuroscience
A nonsequence-specific requirement for SMN protein activity: the role of aminoglycosides in inducing elevated SMN protein ... SMN complexes of nucleus and cytoplasm: A proteomic study for SMA therapy ... stabilizing SMN mRNA and SMN protein, 4) development of neurotrophic, neuroprotective and anabolic compounds and 5) stem cell ... 87] Chang H. C., Hung W. C., Chuang Y. J., Jong Y. J., Degradation of survival motor neuron (SMN) protein is mediated via the ...
https://www.degruyter.com/view/j/tnsci.2010.1.issue-4/v10134-010-0045-4/v10134-010-0045-4.xml
*  Best Advances of 2017: Picks from the Neurology Today Editor... : Neurology Today
... received a single dose of intravenous adeno-associated virus serotype 9 encoding the missing SMN protein, resulting in longer ... For example, the term "complex partial seizure" has now become "focal seizure with impaired awareness." And epilepsy is now ... protein. At approximately two years, 15 of the 17 patients were alive and free of major functional disability, with minimal ...
https://journals.lww.com/neurotodayonline/Fulltext/2018/01250/Best_Advances_of_2017__Picks_from_the_Neurology.1.aspx
*  Most recent papers with the keyword Nusinersen | Read by QxMD
... leading to low levels of SMN protein. However, a second SMN gene (SMN2) exists, which can be therapeutically targeted to ... Compared to fluoroscopy, CT affords superior guidance for complex needle placements.... 29130140 ... SMA is caused by the lack of survival motor neuron (SMN) protein due to mutations, which are often deletions, in the SMN1 gene ... reduce the expression of a toxic protein, modify functional effects of proteins, or reduce toxicity of mutant proteins... ...
https://www.readbyqxmd.com/keyword/104001
*  Frontiers | Integrated Analysis of DNA Methylation and mRNA Expression Profiles to Identify Key Genes in Severe Oligozoospermia...
Integrated pathways analysis indicates that these genes are involved in response to hormone stimulus, activation of protein ... Integrated pathways analysis indicates that these genes are involved in response to hormone stimulus, activation of protein ... is a complex disorder, whose etiology is the combined effect of genetic factors and epigenetic conditions. In this study, we ... is a complex disorder, whose etiology is the combined effect of genetic factors and epigenetic conditions. In this study, we ...
https://www.frontiersin.org/articles/10.3389/fphys.2017.00261/full
*  Items where Community is "CSHL labs | Krainer lab" - CSHL Scientific Digital Repository
Binding to SMN2 pre-mRNA-protein complex elicits specificity for small molecule splicing modifiers. Nat Commun, 8 (1). p. 1476 ... Hua, Y., Sahashi, K., Rigo, F., Hung, G., Horev, G., Bennett, C. F., Krainer, A. R. (2011) Peripheral SMN restoration is ... Manley, J. L., Krainer, A. R. (2010) A rational nomenclature for serine/arginine-rich protein splicing factors (SR proteins). ... complex) disease. In: Genetics of Complex Human Diseases: A Laboratory Manual. Cold Spring Harbor Laboratory Press, pp. 165-182 ...
http://repository.cshl.edu/view/divisions/Krainer=5Flab.default.html
*  anti-ITCH Primary Antibodies
... atrophin 1 interacting protein 4 (AIP4)) , itchy E3 ubiquitin protein ligase homolog , E3 ubiquitin-protein ligase Itchy ... Itch monoubiquitinates SMN and monoubiquitination of SMN plays an important role in regulating its cellular localization. ... Itch/beta-arrestin2 complex binds SuFu and induces its Lys63-linked polyubiquitylation without affecting its stability. ... Protein level used designations for ITCH E3 ubiquitin-protein ligase Itchy homolog , NFE2-associated polypeptide 1 , atrophin-1 ...
https://www.anticorps-enligne.fr/activation-of-innate-immune-response-pathway-52/itch-antibody-297/
*  FENS Forum 2004 - Abstracts
SPECIFIC ASSOCIATED OF THE SMN PROTEIN WITH THE CYTOSKELETON IN THE AXONAL COMPARTMENT OF CULTURED MOTOR NEURONS.. A115.4. ... NMDA RECEPTOR COMPLEX IMPAIRMENT IN HUMAN CEREBRAL HETEROTOPIA.. A069.4. Symposium SS1-4 (session 069). Sun 11/07 15:45 - Room ... ROLE OF SCAFFOLD PROTEINS IN AMPA RECEPTOR LATERAL MOBILITY REGULATION.. A120.2. Poster 227 - Mon 12/07, 16:45 - Hall I. ... PROTEIN KINASE CMU (PKCMU) IS EXPRESSED BY HUMAN IMMORTALIZED MICROGLIA (C13NJ).. A004.3. Poster 9 - Sun 11/07, 12:15 - Hall I ...
http://fens2004.neurosciences.asso.fr/posters/Let/LetB.html
*  JCI - Volume 124, Issue 2
and resulting SMN protein deficiency manifests predominantly with motor neuron degeneration; however, a wealth of emerging data ... interacts with SMAD3 to sustain activation of the SMAD3/SMAD4 complex in the nucleus. FOXM1 prevented the E3 ubiquitin-protein ... Spinal muscular atrophy is a common motor neuron disease caused by low survival motoneuron (SMN), a key protein in the proper ... Our results demonstrate that relative maturity of the NMJ determines the temporal requirement for the SMN protein. These ...
https://www.jci.org/124/2
*  0601 - Biochemistry and Cell Biology
The SMN interactome includes Myb-binding protein 1a  Fuller, Heidi; Thanh, Le; Asperger, Arndt; Morris, Glenn; Man, Nguyen; ... Human immunodeficiency virus 1 (HIV-1) virion infectivity factor (Vif) is part of reverse transcription complexes and acts as ... p160 Myb-binding protein interacts with Prep1 and inhibits its transcriptional activity  Díaz, Víctor; Mori, Silvia; ... Vif-deficient HIV reverse transcription complexes (RTCs) are subject to APOBEC3G-mediated structural changes and mutation of ...
https://dspace.flinders.edu.au/xmlui/handle/2328/27321
*  Categorical Spending - Project Listing - NIH Research Portfolio Online Reporting Tools (RePORT)
Spinal muscular atrophy therapy using recombinant SMN proteins ZHAO, RUI UNIVERSITY OF COLORADO DENVER CO $233,188 ... Characterization of a complex regulatory element of Spinal Muscular Atrophy genes SINGH, RAVINDRA IOWA STATE UNIVERSITY IA $ ... The COPA vesicle protein and pathogenesis of spinal muscular atrophy ANDROPHY, ELLIOT INDIANA UNIV-PURDUE UNIV AT INDIANAPOLIS ... Survival Motor Neuron (SMN) function in motoneuron development BEATTIE, CHRISTINE OHIO STATE UNIVERSITY OH $385,000 ...
https://report.nih.gov/categorical_spending_project_listing.aspx?FY=2015&ARRA=N&DCat=Spinal%20Muscular%20Atrophy
*  JPM | Free Full-Text | Antisense Therapy in Neurology | HTML
... some full-length SMN transcripts (~10%) and functional SMN proteins are still produced. The SMN2 gene differs from SMN1 by only ... Schoser, B.; Timchenko, L. Myotonic dystrophies 1 and 2: Complex diseases with complex mechanisms. Curr. Genomics 2010, 11, 77- ... The sarcolemmal protein dysferlin is a transmembrane protein that is ubiquitously expressed and is found abundantly in cardiac ... impairing production of normal SMN protein. AOs targeted to the intronic splice silencer site (ISS) in intron 7 of SMN2 ...
https://www.mdpi.com/2075-4426/3/3/144/htm
*  Journal of Neuromuscular Diseases - Volume 4, issue 1 - Journals - IOS Press
SMN Blood Levels in a Porcine Model of Spinal Muscular Atrophy Authors: Iyer, Chitra , Wang, Xueqian , Renusch, Samantha R. , ... Abstract: Myofibrillar myopathies (MFMs) are muscular disorders involving proteins that play a role in the structure, ... If you need to make more complex queries, use the tips below to guide you. ... The measurement of SMN mRNA levels in peripheral blood samples has been used in SMA clinical studies as a pharmacodynamic ...
https://content.iospress.com/journals/journal-of-neuromuscular-diseases/4/1
*  EB病毒核抗原3C提高Gemin3基因的表达
Gemin3: A novel DEAD box protein that interacts with SMN, the spinal muscular atrophy gene product, and is a component of gems[ ... SCFSkp2 complex targeted by Epstein-Barr virus essential nuclear antigen[J]. Mol Cell Biol, 2005, 25(5):1749-1763.. [17] Bajaj ... The DEAD-box protein family of RNA helicases[J]. Gene, 2006, 367(15):17-37.. [6] Rocak S, Linder P. DEAD-box proteins: the ... Characterization of DP103, a novel DEAD box protein that binds to the Epstein-Barr virus nuclear proteins EBNA2 and EBNA3C[J]. ...
http://journal.cmu.edu.cn/CN/Y2012/V41/I6/497
*  Gene coexpression networks reveal key drivers of phenotypic divergence in porcine muscle | BMC Genomics | Full Text
Our results showed the regulation of muscle development might be more complex than had been previously acknowledged, and is ... Survival motor neuron (SMN) protein: role in neurite outgrowth and neuromuscular maturation during neuronal differentiation and ... The complex coexpression networks responsible for the different muscle phenotypes are likely to have been generated by ... SMN1 encodes a protein that is crucial in neuromuscular maturation [24]. GNB2L1 [25] and SBDS [26] encode proteins that are ...
https://bmcgenomics.biomedcentral.com/articles/10.1186/s12864-015-1238-5
*  Apoptosis Database
Naip5 encodes a protein that is homologous to plant innate immunity (so-called resistance) proteins and has been implicated in ... A second SMA candidate gene encoding survival motor neuron (SMN), which is contiguous with the NAIP locus on 5q13.1, was also ... Inflammasomes are cytosolic multiprotein complexes that sense microbial infection and trigger cytokine production and cell ... Short gene description: Baculoviral IAP repeat-containing protein 1 (Neuronal apoptosis inhibitory protein) [Source:UniProtKB/ ...
http://deathbase.org/protein_report.php?id=H_sapiens_ENSP00000194097
*  Methods and Compositions Related to Delivery of Chemical Compounds to Invertebrate Embryos - Patent application
This protein forms heteromeric complexes with proteins such as SIP1 and GEMIN4, and also interacts with several proteins known ... For example, in SMN (Spinal motorneuronal atrophy), the human gene exists as an almost perfect duplication, known as SMN1 and ... 0250]Gamma secretase is an enzyme complex that cleaves type I transmembrane proteins. Inhibitors of this complex inhibit the ... protein RNA complex which is cable of degrading RNA, the nuclease complex, which has been called the RNA-induced silencing ...
http://www.patentsencyclopedia.com/app/20080276327
*  RNAi knockdown of hPrp31 leads to an accumulation of U4/U6 di‐snRNPs in Cajal bodies | The EMBO Journal
To determine whether U4 snRNP, U6 snRNP and the protein complex CypH/hPrp4/hPrp3 accumulate in Cajal bodies separately, or as ... coilin and SMN. J Cell Sci 114: 4407-4419. ... 220kD protein (hPrp8) forms a stable RNA‐free complex with ... An important protein for the interaction between the U4/U6 di‐snRNP and the U5 snRNP is the U4/U6‐specific hPrp31 protein, ... The subsequent integration of the proteins CypH (20K or USACyp), hPrp4 (60K) and hPrp3 (90K), which form a heteromeric complex ...
http://emboj.embopress.org/content/23/15/3000
*  Biogen Idec and Isis Pharmaceuticals Announce Global Collaboration for Antisense Program Targeting Myotonic Dystrophy - Drugs...
It is caused by a genetic defect in the dystrophia myotonica-protein kinase (DMPK) gene in which a sequence of three ... Prevymis Prevymis (letermovir) a CMV DNA terminase complex inhibitor indicated for prophylaxis of cytomegalovirus... ... ISIS-SMNRx, for the treatment of spinal muscular atrophy (SMA). ... drugs can reduce the production of disease-causing proteins ... The genetic defect in the DMPK gene creates a toxic RNA rather than a disease-causing protein. The toxic RNA accumulates within ...
https://www.drugs.com/news/biogen-idec-isis-pharmaceuticals-announce-global-collaboration-antisense-program-targeting-myotonic-39023.html
*  Cell nucleus
Most proteins, ribosomal subunits, and some RNAs are transported through the pore complexes in a process mediated by a family ... but do contain a protein called survivor of motor neurons (SMN) whose function relates to snRNP biogenesis. Gems are believed ... During most of the cell cycle these are organized in a DNA-protein complex known as chromatin, and during cell division the ... While in the nucleus, pre-mRNA is associated with a variety of proteins in complexes known as heterogeneous ribonucleoprotein ...
https://www.bionity.com/en/encyclopedia/Cell_nucleus.html
*  Reciprocal Benefits of Genetic Disease Research - TUFTSBG2007 - VIN
The SMN function that is crucial to motor neuron survival remains obscure, despite intense investigation by many laboratories ... T mutation that predicted a premature stop codon at amino acid residue 111 of the protein coding sequence (p.R111X). The ... but for those in medical research they are windows that open onto the complex physiology underlying normal health. ... is typically made because the gene has a known function that is important in a metabolic pathway or structural complex that is ...
https://www.vin.com/apputil/content/defaultadv1.aspx?pId=11243&meta=Generic&catId=31943&id=3861253&ind=21&objTypeID=17
*  Clarks Drugstore: Order zithromax online trusted suppliers!
Hyperplasia is a more centromeric smn pseudogene. She wont make it more protractile. The finding of a shunt is the platelet ... protein syn-thesis, reproduction, and movement. Oliguria. In patients with lightning injuries are common in septic shock. ... hypertension and decreased pulmonary blood flow and urinary retention or exces-sive mobilization of calcium phosphate complexes ...
https://nrha.org/research/order-zithromax-online/51/
*  A Third of Mars Could Have Been Underwater - Slashdot
We know very little of what situations complex multicellular life can really adapt to, but we can say that Earth would no ... A temperature above which proteins in most organisms coagulate would bring us down to archea. Photosynthesis in its current ... http://smn.klm.net.pl/kosmita3.pdf -- it's in Polish, and the book has been released just a few weeks ago (and it's an alpha ... http://smn.klm.net.pl/kosmita3.pdf. Go on if you speak Polish. ...
https://science.slashdot.org/story/08/11/18/146252/a-third-of-mars-could-have-been-underwater?sdsrc=next
*  Systematic and single cell analysis of Xenopus Piwi‐interacting RNAs and Xiwi | The EMBO Journal
Each of the above antibodies was pre‐complexed with protein A or G Dynabeads and used to precipitate the relevant protein from ... Sprangers R, Groves MR, Sinning I, Sattler M (2003) High‐resolution X‐ray and NMR structures of the SMN Tudor domain: ... to stabilize protein-protein and protein-RNA interactions (Figure 5B). Several proteins consistently co‐IPed with Xiwi using ... Associated proteins were excised from the gel and identified by mass spectrometry. Protein identities of the top hits are ...
http://emboj.embopress.org/content/28/19/2945