TY - JOUR. T1 - Expression of the neural intermediate filament proteins peripherin and neurofilament-66/α-internexin in neuroblastoma. AU - Foley, John. AU - Witte, D.. AU - Chiu, F. C.. AU - Parysek, L. M.. PY - 1994. Y1 - 1994. N2 - BACKGROUND: Peripherin and neurofilament (NF)-66/α-internexin are recently characterized, neuron-specific intermediate filament proteins that are expressed in the developing peripheral nervous system. Peripherin, in particular, is highly enriched in neuronal derivatives of the neural crest. We speculated that these intermediate filament proteins would be expressed in neuroblastoma (NB), a neural crest-derived tumor with many neuronal features. EXPERIMENTAL DESIGN: By use of antibodies specific to peripherin and NF- 66/α-internexin, we detected these proteins on Western blots of NB tissue extracts and in paraffin sections of NBs. RESULTS: Western blotting indicated that NB tumor extracts contained immunoreactive proteins that co-migrated with rat peripherin and ...
en] Peripherin is an intermediate filament protein that is expressed in peripheral and enteric neurons. In the cochlear nervous system, peripherin expression has been extensively used as a differentiation marker by preferentially labeling the type II neuronal population at adulthood, but yet without knowing its function. Since the expression of peripherin has been associated in time with the process of axonal extension and during regeneration of nerve fibers in other systems, it was of interest to determine whether peripherin expression in cochlear neurons was a static phenotypic trait or rather prone to modifications following nerve injury. In the present study, we first compared the expression pattern of peripherin and beta III-tubulin from late embryonic stages to the adult in rat cochlea. The staining for both proteins was seen before birth within all cochlear neurons. By birth, and for 2 or 3 days, peripherin expression was gradually restricted to the type II neuronal population and their ...
PURPOSE: To report the characteristic findings of a macular pattern dystrophy in patients with diabetes and deafness resulting from the mitochondrial point mutation at position 3243 and to expand the clinical spectrum of this condition by describing functional testing results. METHODS: Four diabetic patients who were referred to the eye department for diabetic fundus examination were found to harbor a macular pattern dystrophy. Further examination of visual fields; color contrast sensitivity; and the ear, nose, and throat; and molecular analysis of the mitochondrial genome were performed. Two of our patients were sisters. Their relatives also were examined. RESULTS: All four patients were found to harbor the mitochondrial point mutation at position 3243 and presented clinically with the phenotype of diabetes and deafness. The macular pattern dystrophy described in these patients seems to be typical for this condition. Results of a 9-year follow-up study of one of the patients showed mild ...
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Macular Pattern Dystrophy Symptom Checker: Possible causes include Retinitis Pigmentosa 7. Check the full list of possible causes and conditions now! Talk to our Chatbot to narrow down your search.
The natural history of subfoveal CNV secondary to Pattern Dystrophy is little understood, but it seems that visual function may be preserved only in the short-term follow-up, with progressive deterioration thereafter.. Photodynamic therapy with verteporfin is the most studied therapeuthic option for this kindf of lesion, but unfortunately, this approach does not appear to guarantee long-term vision stabilization, and alternative therapies should be investigated. ...
Purpose : The tetraspanins peripherin-2 and Rom-1 can form homo- and heteromeric complexes in photoreceptor outer segments (OS). Mutations in peripherin-2, but not in Rom-1, lead to autosomal dominant retinitis pigmentosa (adRP) and the vast majority of these mutations is located in the D2 loop domain. How different complexes assembled by various combinations of wild type (perWT), mutant peripherin-2 (perMT) or Rom-1, which can be formed in heterozygous patients, contribute to the pathophysiology of adRP remained elusive so far. Here, focusing on the two adRP-linked peripherin-2 D2 loop mutants, perP210L and perC214S, we analyzed the binding characteristics, subunit assembly, subcellular localization, and rod OS targeting of perWT-perMT, and Rom-1-perMT complexes in HEK293 cells and in murine rod photoreceptors. Methods : Binding characteristics were analyzed by co-immunoprecipitation, protease accessibility assay, peptide competition assay, and quantitative FRET measurements. Subunit assembly ...
Peripherin was discovered as being the major intermediate filament in neuroblastoma cell lines and in rat pheochromocytoma cells. It is classified by gene structure and coding sequence as a type III intermediate filament protein because of its homology with vimentin, glial fibrillary acidic protein, and desmin.[5] All intermediate filament proteins share a common secondary structure consisting of three main domains, the most conserved of which is the central α-helical rod domain. This central coil is capped by non-helical head (N-terminal) and tail (C-terminal) domains. The α-helical rod domain contains repeating segments of hydrophobic amino acids, such that the first and fourth residues of every set of seven amino acids are usually nonpolar. This specific structure enables two intermediate filament polypeptides to coil together and create a hydrophobic seal.[6] The rod also contains specific placement of alternating acidic and basic residues, many of which are spaced 4 amino acids apart. ...
Purpose: Vertebrate vision relies on photon capture by the flattened membranous disks that comprise rod and cone outer segments (OSs). The mechanisms that generate and maintain the membrane curvature required for normal disk morphology are not yet defined, although the photoreceptor-specific integral membrane protein peripherin/rds (P/rds) is thought to play a role. The aim of this study was to investigate the recent proposal that an induced amphipathic helix (AH) within the cytoplasmic C-terminal domain of P/rds can contribute to the membrane curvature of OS disks.. Methods: Association of the P/rds C-terminal domain (CTER) with membranes was assayed in vitro using extruded liposomes in conjunction with purified recombinant proteins. A gradient-based floatation assay was used to characterize protein-membrane interaction and derive partition coefficients. The contribution of this domain to membrane curvature generation was assessed in cellulo, using WT and mutant variants of P/rds expressed in ...
4. Vaclavik V, Tran HV, Gaillard MC, Schorderet DF, Munier FL. Pattern dystrophy with high intrafamilial variability associated with Y141C mutation in the peripherin/RDS gene and successful treatment of subfoveal CNV related to multifocal pattern type with anti-VEGF (ranibizumab) intravitreal injections. Retina. 2012; 32(9): 1942-1949. doi: ...
Tetraspanin membrane proteins encompass a functionally diverse group of proteins involved in cell adhesion, cell recognition and membrane fusion events. These proteins are characterized by a highly conserved extra-cellular domain, known as the EC-2 domain that is involeid in formation of disulfide linked oligomers and functionally diverse C-termini. We are interested in understanding the role of two retinal specific tetraspanins, peripherin-2, the product of the rds gene, and its non-glycosylated homologue rom-1, in the development and progression of degenerative diseases. A murine model of retinitis pigmentosa (RP) in which a 10 kb insertion of exogeneous DNA results in an rds null allele as well as a rom-1 knockout mouse suggest that although peripherin-2 and rom-1 cooperate to generate healthy photoreceptors, they are not functionally equivalent and rom-1 likely plays a subsidiary role. Peripherin-2 and rom-1 form both homo and hetero-tetramers with peripherin-2 shown to oligomerize further ...
Looking for PRPH2? Find out information about PRPH2. respiratory distress syndrome of newborn Random Dot Stereogram Thin client computing solutions from Microsoft that evolved from Terminal Services. Explanation of PRPH2
In this study, we have demonstrated that the loss of Rds in cone PRs does not affect the differentiation of PRs but causes the formation of morphologically novel distended, membranous OS structures that, nonetheless, are capable of phototransduction. These structures contain S-opsin yet lack the compartmentalization of lamellae, directly implicating Rds in this process during cone OS morphogenesis. Rod PRs lacking Rds fail to form OS structures and possess rhodopsin solely in the tip of the cilium and IS membrane, causing minimal phototransduction activity and subsequent PR degeneration (Reuter and Sanyal, 1984; Nir and Papermaster, 1986). In contrast, cone PRs lacking Rds form altered OS structures lacking normal lamellar organization but, nonetheless, are capable of phototransduction, albeit with reduced sensitivity. Furthermore, the loss of Rds in cones with concomitant abortive lamellar formation prevents the extracellular matrix from forming around the cone OS, further reinforcing the ...
Membrii CNA au decis somarea publica a posturilor Antena 1 si Antena 3 pentru incalcarea articolelor 66 si 67 din Codul de reglementare a continutului audiovizual, care se refera la exprimarea obiectiva, adecvata si impartiala a opiniilor in dezbaterea problemelor de interes public. Cele doua televiziuni ale trustului Intact au fost taxate pentru ca si-au sustinut doar propriul punct de vedere litigiul cu RCS&RDS, dupa ce reteaua de cablu a decis in perioada aprilie - mai 2012, scoaterea posturilor trustului respectiv din lista sa de programe. Decizia a dus la o campanie derulata de Antena 1 si Antena 3 impotriva companiei RCS&RDS, acuzata inclusiv de interese politice. Atacurile n-au tinut cont de faptul ca Antena Group a fost cea care somase RCS & RDS sa inceteze difuzarea prin satelit a canalelor sale, daca nu achita o taxa anuala de peste 7 milioane de euro. Catalin Alexandru, reprezentantul legal al RCD&RDS, a explicat membrilor CNA ca decizia companiei de a nu mai difuza cele doua canalele ...
Membrii CNA au decis somarea publica a posturilor Antena 1 si Antena 3 pentru incalcarea articolelor 66 si 67 din Codul de reglementare a continutului audiovizual, care se refera la exprimarea obiectiva, adecvata si impartiala a opiniilor in dezbaterea problemelor de interes public. Cele doua televiziuni ale trustului Intact au fost taxate pentru ca si-au sustinut doar propriul punct de vedere litigiul cu RCS&RDS, dupa ce reteaua de cablu a decis in perioada aprilie - mai 2012, scoaterea posturilor trustului respectiv din lista sa de programe. Decizia a dus la o campanie derulata de Antena 1 si Antena 3 impotriva companiei RCS&RDS, acuzata inclusiv de interese politice. Atacurile n-au tinut cont de faptul ca Antena Group a fost cea care somase RCS & RDS sa inceteze difuzarea prin satelit a canalelor sale, daca nu achita o taxa anuala de peste 7 milioane de euro. Catalin Alexandru, reprezentantul legal al RCD&RDS, a explicat membrilor CNA ca decizia companiei de a nu mai difuza cele doua canalele ...
Unable to map to the local drive for RDS services. Are there any restrictions, if so how to fix them? Is it possible to hide the local drive? ...
JNeurosci Print ISSN: 0270-6474 Online ISSN: 1529-2401. The ideas and opinions expressed in JNeurosci do not necessarily reflect those of SfN or the JNeurosci Editorial Board. Publication of an advertisement or other product mention in JNeurosci should not be construed as an endorsement of the manufacturers claims. SfN does not assume any responsibility for any injury and/or damage to persons or property arising from or related to any use of any material contained in JNeurosci.. ...
Looking for online definition of peripherin-2 in the Medical Dictionary? peripherin-2 explanation free. What is peripherin-2? Meaning of peripherin-2 medical term. What does peripherin-2 mean?
Looking for online definition of retinal peripherin in the Medical Dictionary? retinal peripherin explanation free. What is retinal peripherin? Meaning of retinal peripherin medical term. What does retinal peripherin mean?
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Quantitative analyses of peripherin mRNPs demonstrate that ∼80% of total peripherin mRNA resides in clusters of two or more. Analyses of cells expressing the ECFP-TAA-Peripherin-R24-3′UTR construct, from which peripherin protein is not synthesized, demonstrate that ∼60% of the total mRNA remains in clusters of two or more. The reduction from ∼80 to ∼60% is mostly attributable to an ∼30% increase in mRNPs containing single mRNAs and an ∼60% decrease in the largest mRNPs containing 6-30 mRNAs, suggesting that interactions between the nascent peripherin protein chains may contribute to the formation of large mRNA clusters. There was no significant change in the number of mRNPs containing two to five mRNAs under these experimental conditions. In the absence of a 3′UTR, there was a similar decrease in clustered peripherin mRNPs with the most dramatic decrease again in the large 6-30 mRNA-containing clusters. There was also an ∼30% increase in mRNPs containing single mRNAs. The ...
Complete information for CACD gene (Genetic Locus), Central Areolar Choroidal Dystrophy, including: function, proteins, disorders, pathways, orthologs, and expression. GeneCards - The Human Gene Compendium
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Chicken polyclonal Peripherin antibody validated for IHC and tested in Human, Mouse and Rat. Referenced in 6 publications and 2 independent reviews. Immunogen…
This topic describes how to configure the hybrid access solution for an ApsaraDB RDS for PostgreSQL instance. This solution allows you to retain both the classic network endpoint and virtual private c...
VALUE sætter fokus på at styrke samarbejdet mellem alle medarbejdere som er involveret i at arbejde med læring og omsorg i dagtilbud med børn af forskellig social baggrund og værdsættelse af medarbejdere med forskellige baggrund og erfaringer. Der rettes speciel opmærksomhed på den rolle og position som professionelle med lav eller ingen uddannelse/ pædagogmedhjælpere. Spørgsmål der stilles i projektet er hvordan styrkes pædagogmedhjælperes rolle og identitet? Hvordan involveres pædagogmedhjælpere i professionel udvikling og planlægning ...
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A csontok elvesztik szil rds gukat, puh v v lnak s eldeform l dnak. Okozhatja id lt veseel gtelens g, D-vitaminhi ny, alult pl lts g, felsz v d si zavarok s egy b rendellenes m k d sek. A betegs g csontf jdalommal s gyakran izomgyenges ggel j r. A csontl gyul st D-vitamin- s kalciumadagol ssal
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Vimentin is a class III intermediate filament protein predominantly found in cells of mesenchymal origins, such as vascular endothelium and blood cells, where it functions as a major cytoskeletal component. Due to its importance and abundance in the cytoskeletal structure of mesenchymally-derived cells, vimentin is fre
Systemic steroid hormone and intracellular signaling pathways are known to act cooperatively during the development of vertebrate and invertebrate epithelia. However, the mechanism of this interaction is poorly understood. Morphogenesis of Drosophila leg imaginal disc epithelia is regulated both by the steroid hormone 20-hydroxyecdysone (ecdysone) and the RhoA GTPase signaling pathway. Recent evidence suggests that these pathways act cooperatively to control imaginal disc morphogenesis. Thus, leg imaginal disc morphogenesis is an excellent system in which to study the interaction of steroid hormone and intracellular signaling pathways. We have identified mutations in three genes, 12-5, 18-5, and 31-6, with roles in the morphogenesis of leg epithelia. Of particular interest, these mutations interact genetically with each other, mutations in the RhoA signaling pathway, and the ecdysone regulated Sb-sbd (Stubble) transmembrane serine protease. This suggests that the 12-5, 18-5, and 31-6 gene products may
If the Moons orbit was not inclined to our own, wed get two eclipses per lunation, one solar and one lunar.. 2013 has 5 eclipses, 3 lunar and 2 annular. The minimum number of eclipses that can occur in a calendar year is 4, and the maximum is 7, as will next occur in 2038.. The 3 lunar eclipses in 2013 are this weeks partial eclipse on April 25th and two faint penumbral eclipses, one on May 25th and another on October 18th. There is no total lunar eclipse in 2013. The last one occurred on December 10th 2011, and the next one wont occur until April 15th 2014, favoring the Pacific Rim region.. This eclipse will also set us up for the first solar eclipse of 2013, an annular eclipse crossing NE Australia (in fact crossing the path of last years total eclipse near Cairns) and the south Pacific on May 10th. The only solar totality that will touch the surface of the Earth in 2013 is the hybrid eclipse on November 3rd spanning Africa and the South Atlantic with a maximum totality of 1 minute & 40 ...
Vanbery Cottage is part of Dulhunty a 43 acre cattle property. Situated a scenic 12 kilometre drive South of Boonah (90 minutes drive from Brisbane Central Business District). Enjoy a beautiful country experience for couples in the heart of the amazing Scenic Rim Region of South East Queensland.. Boutique private hilltop accommodation with breathtaking valley and mountain views.. One fully self-contained Cottage features beautiful decor, fully equipped kitchen, en suite with bath.. Swim away tension in the deluxe in-ground pool with private access.. Nothing like the flicker of a fire to complete your winter escape. Relax the day away on the verandah enjoying sweeping and ever changing views and abundant wildlife.. ...
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If your ApsaraDB RDS instance has an event pending to be processed, the ApsaraDB RDS console notifies you of the event, so you can handle the event at your earliest opportunity.
Mutations in the PRPH2 gene cause a variety of other retinal disorders. Each of these conditions involves a slow degeneration of photoreceptor cells, leading to progressive vision loss. A total of more than 100 mutations in the PRPH2 gene have been identified. Many of these mutations cause autosomal dominant retinitis pigmentosa, an eye disease that first disrupts night vision and side (peripheral) vision and eventually may result in blindness. PRPH2 mutations also cause a group of retinal disorders called pattern dystrophies of the retinal pigment epithelium. These disorders typically begin in mid-adulthood and are characterized by an abnormal buildup of pigment in cells underlying the retina.. Some PRPH2 mutations can cause different eye disorders in affected members of the same family. For example, researchers have reported a family with retinitis pigmentosa, pattern dystrophy of the retinal pigment epithelium, and retinitis punctata albescens (an eye disorder similar to retinitis pigmentosa) ...
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Mutations in the photoreceptor tetraspanin gene peripherin-2/retinal degeneration slow (gene have already been Enzastaurin linked with human being illnesses including autosomal dominant retinitis pigmentosa (adRP) digenic RP design dystrophy adult vitelliform macular dystrophy central areolar choroidal dystrophy and other styles of macular degeneration (MD) (http://www. are held collectively via interactions between your second intradiscal (D2) loop of both protein (4 5 RDS and ROM-1 function collectively assembling in the internal section (cell body) Enzastaurin from the photoreceptor into tetrameric primary complexes (6). These complexes are after that trafficked towards the Operating-system where they additional assemble into higher purchase oligomeric constructions including hetero-octamers and Mouse monoclonal to NANOG RDS homo-oligomers (7). These bigger complexes are kept collectively by intermolecular disulfide bonds mediated by a particular cysteine residue (C150) (8 9 among seven D2 ...
DMC2 : Indirect Immunofluorescence Assay: Before testing, patients specimen is preabsorbed with liver powder to remove nonorgan-specific autoantibodies. After applying to a composite substrate of frozen mouse tissues (brain, kidney, and gut) and washing, fluorescein-conjugated goat-antihuman IgG is applied to detect the distribution and pattern of patient IgG binding.(Pittock SJ, Kryzer TJ, Lennon VA: Paraneoplastic antibodies coexist and predict cancer, not neurological syndrome. Ann Neurol 2004;56:715-719; Basal E, Zalewski N, Kryzer TJ, et al: Paraneoplastic neuronal intermediate filament autoimmunity. Neurology 2018 Oct 30;91[18]:e1677-e1689)   Radioimmunoassay: Duplicate aliquots of patient specimen are incubated with I(125)-labeled antigen. Immune complexes, formed by adding secondary (goat) antihuman immunoglobulin, are pelleted by centrifugation and washed. Gamma emission from the washed pellet is counted, and mean counts per minute (cpm) are compared with results yielded by high
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Diagnosis Code D36.13 information, including descriptions, synonyms, code edits, diagnostic related groups, ICD-9 conversion and references to the diseases index.
Robertson J, Doroudchi MM, Nguyen MD, Durham HD, Strong MJ, Shaw G, et al. A neurotoxic peripherin splice variant in a mouse model of ALS. J Cell Biol. 2003;160(6):939-49. ...
Robertson J, Doroudchi MM, Nguyen MD, Durham HD, Strong MJ, Shaw G, et al. A neurotoxic peripherin splice variant in a mouse model of ALS. J Cell Biol. 2003;160(6):939-49. ...
RDS provides various resources on the website, including links to potentially useful external resources and a list of members with an interest in rheumatic skin disease. This information serves only as a reference tool for patients and healthcare providers. RDS gives no warranties regarding the medical information supplied on the website. RDS is not compensated for the mention of any medical information or product on this website and does not endorse, warrant, or guarantee the effectiveness of the information or any such product. You should not initiate the use of any treatment or product mentioned on this website or on any links to external resources without first consulting a physician or healthcare provider. RDS is not responsible for any treatment decisions made by any health care provider, even those listed on the RDS website or links to external resources. All users of this website retain full responsibility for the use of the resources on this website, and RDS is not responsible for the ...
Join Bart Castle as he covers common deployment, monitoring, maintenance, and optimization tasks for Amazon RDS Databases. Gain an understanding of key configurable components, processes, and other AWS complimentary management services for supporting RDS instances.
This blog is a follow up to Migrating PostgreSQL Databases From On-Prem to the Cloud Using AWS RDS where we explore the common errors you may encounter when performing this migration.
Warmth with minimal weight: The Radical Down RDS Hooded Jacket for women scores big points with its water-resistant down for any ski tour. This down
Jakobsson, Lovisa, 2021. Gyllins trädgårds alléer och kulturella ekosystemtjänser : en underlag- och platsanalys. First cycle, G2E. Alnarp: SLU, Department of Landscape Architecture, Planning and Management (from 130101 ...
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