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Topic: Peripherin

	Novus Bio: Peripherin antibody \| Rabbit polyclonal anti-Peripherin
		Peripherin is a Class III intermediate filament subunit found in both the peripheral and central nervous systems, though it is concentrated, as its name suggests, in the neurons of peripheral ganglia and their processes.
		Peripherin is also a major component of the cytoskeleton of PC12 cells, widely used model system for studies of neuronal differentiation.
		Peripherin is not related to peripherin-RDS, a photoreceptor protein associated with retinal degeneration and blindness.
	www.novusbio.com /data_sheet/index/NB300-137&prev_module=search (320 words)

	PRPH - Peripherin
		Peripherin, an intermediate filament of sympathetic neurones, is undetectable in chromaffin cells of GR mutants.
		Peripherin, a neuronal intermediate protein, is stably expressed by neuroendocrine carcinomas of the skin, their xenograft on nude mice, and the corresponding primary cultures.
		Peripherin immunoreactivity was specifically distributed in some small ganglion neurons and in their central and peripheral extensions, particularly in fibers reaching the lower part of the outer hair cells.
	www.pdg.cnb.uam.es /UniPub/iHOP/gi/91358.html (4793 words)

	PRPH - Peripherin
		Peripherin is a neuron-specific intermediate filament protein whose expression is activated in vitro by the neuropoietic cytokines leukemia inhibitory factor (LIF) and interleukin-6.
		Peripherin is not a contributing factor to motor neuron disease in a mouse model of amyotrophic lateral sclerosis caused by mutant superoxide dismutase.
		At the perinatal stage, expression patterns of alpha-internexin and peripherin are distinguishable by spatial and temporal manner: peripherin is predominantly expressed in the olfactory nerves; whereas alpha-internexin is expressed in both olfactory nerves and olfactory bulb.
	www.pdg.cnb.uam.es /UniPub/iHOP/gg/91358.html (5345 words)

	A Fresh Perspective on ALS
		Robertson’s research focuses on peripherin, a key protein involved in the pathological aggregates found in motor neurons in ALS.
		Robertson investigated the expression of a protein splice variant called “peripherin” in ALS and discovered that abnormalities in splicing may be an important factor contributing to the disease mechanism.
		Peripherin is found in the neuronal aggregates of both ALS patients and SOD-1 transgenic mice.
	www.als.ca /_news/4106.aspx (597 words)

	The Retina
		There is no known treatment; however, since blue and ultraviolet light may make aggravate the condition, amber-colored glasses with an ultraviolet absorption coating, worn during the day, may slow down the disease process.
		Studies have shown that retinitis pigmentosa is caused by mutations in the rhodopsin gene, the peripherin gene, and possibly in other genes within the rod.
		Mutations in the peripherin gene also may be the cause of another devastating retinal disorder: “macular dystrophy.”
	www.tedmontgomery.com /the_eye/retina.html (1026 words)

	[No title] (Site not responding. Last check: )
		Peripherin, a type III intermediate filament protein, was initially described by Portier et al.
		Thompson and Ziff (1989) cloned the rat peripherin gene and determined that the peripherin protein is a type III intermediate filament protein.
		This peripherin gene, PRPH, is distinct from the photoreceptor type of peripherin (RDS; 179605), or PRPH2, which is mutant in a form of retinitis pigmentosa (608133) in the human and the mouse.
	zeta.embl-heidelberg.de:8000 /srs5bin/cgi-bin/wgetz?-e+[omim-id:170710] (1112 words)

	Monoclonal to Peripherin 8G2 from EnCor Biotechnology Inc. (Site not responding. Last check: )
		Peripherin is also expressed in certain neuroendocrine tumors and in the insulin producing cells of the pancreas.
		Peripherin is also a good diagnostic marker for ballooned axons seen in Lou Gehrig's disease (Amyotrophic lateral sclerosis) and some neuronally derived tumors.
		Peripherin is not related to peripherin/RDS, a protein of the photoreceptor outer membrane mutations of which are causative of certain forms of slow retinal degeneration.
	www.encorbio.com /monoclonal/MCA-8G2.htm (491 words)

	A neurotoxic peripherin splice variant in a mouse model of ALS -- Robertson et al. 160 (6): 939 -- The Journal of Cell ...
		and is encoded by all nine exons of the peripherin gene.
		peripherin and of transgenic mice overexpressing mutant SOD1
		was intense and correlated with the occurrence of peripherin
	www.jcb.org /cgi/content/full/160/6/939 (7104 words)

	Peripherin: AB1530
		Peripherin is a 56-58 kDa type III intermediate filament protein (Portier et al.
		During normal, late neurogenesis peripherin appears after the expression of nestin, vimentin, alpha-internexin and neurofilaments and is involved in the differentiation of neurons, namely of the peripheral nervous system, and also probably in axonal regeneration.
		Peripherin is not related to peripherin-RDS, a photoreceptor protein associated with retinal degeneration and blindness.
	www.chemicon.com /Product/ProductDatasheet.asp?ProductItem=AB1530 (382 words)

	OhioLINK ETD: Uveges, Thomas
		To determine the intragenic regions that are critical for regulating peripherin gene expression, transgenic mice were generated that expressed peripherin transgenes lacking different introns.
		Thus, accurate cell type-specific peripherin gene expression depends on elements within intron 1, but other sequences, most likely in the 5'flanking region, are required for activating the peripherin gene in response to nerve injury.
		Studies to determine the function of the peripherin IF network in the formation of neurite in vitro showed that an intact peripherin network is essential for maintenance/formation of long-branch neurite trees and for normal mitochondrial distribution.
	rave.ohiolink.edu /etdc/view?acc_num=ucin1029167857 (281 words)

	Peripherin 7C5 Monoclonal Antibody (Site not responding. Last check: )
		Peripherin is a ~57kDa intermediate filament subunit found initially in sensory neurons of the peripheral nervous systems.
		Subsequently peripherin was found in sensory and other neurons of the central nervous system and also in PC12 cells.
		Peripherin is also a good diagnostic marker for ballooned axons seen in Lou Gehrig's disease and some neuronally derived tumors.
	store.crpinc.com /datasheetsHTML/MMS-589P.htm (284 words)

	Peripherin antibody [2Q135] (ab17999) datasheet (Site not responding. Last check: )
		If Peripherin antibody [2Q135] (ab17999) does not perform as described on this datasheet, notify us within 90 days of delivery using this form, so that we can help you or offer you a replacement or a refund.
		Peripherin is a Class III intermediate filament subunit found in both the peripheral and central nervous systems, though it is concentrated, as its name suggests, in the neurons of peripheral ganglia and their processes.
		Peripherin is also a major component of the cytoskeleton of PC12 cells, widely used model system for studies of neuronal differentiation.
	www.abcam.com /?datasheet=17999 (478 words)

	Rapid transport of neural intermediate filament protein -- Helfand et al. 116 (11): 2345 -- Journal of Cell Science
		Peripherin particles and squiggles were apparent in regions between the nucleus and the cell surface between 0.5 and 2 hours (A-F; B and C are higher magnification views of the region denoted by the * in A; E and F are higher magnification views of the region indicated by the * in D).
		F-H are diagrammatic representations of the trajectories of individual peripherin squiggles in the cell bodies (F,G) and in a growth cone (H).
		Peripherin particles and squiggles are closely associated with microtubules (MT) in the growth cone of a PC12 cell in DM for 4 hours.
	jcs.biologists.org /cgi/content/full/116/11/2345 (7922 words)

	Role of Pro-inflammatory Cytokines in Peripherin-induced Neurodegeneration - The ALS Association
		Peripherin is a neuronal intermediate filament (IF) protein associated with most of the pathological (89%) IF lesions in the diseased motor neurons of patients with amyotrophic lateral sclerosis (ALS).
		This evidence suggests that peripherin may be an important factor contributing to motor neuron degeneration in ALS.
		The specific aims of this proposal are: 1) To determine the role of TNFa in mediating peripherin-induced neuronal death; 2) To investigate the inflammatory response in peripherin transgenic mice; 3) To investigate the effect of pro-inflammatory cytokines on peripherin isoform expression.
	www.alsa.org /research/grant.cfm?id=146&CFID=624126&CFTOKEN=9126538 (254 words)

	Press Releases - The Potential Role of Peripherin in Motor Neuron Degeneration (04.14.03) (Site not responding. Last check: )
		Peripherin is a protein found in neurons, normally at low levels in motor neurons.
		Peripherin is present in different forms termed Per 58, Per 56 and Per 61.
		This study demonstrates that Per 61 is toxic to motor neurons in culture and is found in association with the abnormal protein accumulations both in the SOD1 mouse model of ALS and in two cases of familial ALS.
	www.alsabayarea.org /press_releases/pr_3_14_03_natl.php (426 words)

	Peripherin antibodies from RDI Div of Fitzgerald Industries Intl/Research Diagnostics Inc
		peripherin is expressed in the developing peripheral nervous system and is highly enriched in neuronal derivatives of the neural crest.
		Peripherin is utilized over other neural markers, such as S100 and NSE in that it does not stain chromaffin cell types.
		Peripherin is also expressed in neural crest derived tumors such as neuroblastomas and ganglioneuroblastomas making it a useful marker for these neoplasms.
	www.researchd.com /neuroabs/periphab.htm (792 words)

	Intron 1 Is Required for Cell Type-Specific, But Not Injury-Responsive, Peripherin Gene Expression -- Uveges et al. 22 ... (Site not responding. Last check: )
		A region of intron 1, from 1360-1664 of the rat peripherin gene, was radiolabeled and digested with DNase I in the presence of BSA (Control), whole-cell extract from the DRG, or nuclear extract from the small intestine (SI), brain, or liver.
		Sequence from 1475-1594 of the rat peripherin gene was radiolabeled and digested with DNase I in the presence of BSA (Control), whole-cell extract from the DRG, or nuclear extract from the small intestine (SI), brain, or liver.
		Thompson MA, Lee E, Lawe D, Gizang-Ginsberg E, Ziff EB (1992) Nerve growth factor-induced derepression of peripherin gene expression is associated with alterations in proteins binding to a negative regulatory element.
	www.jneurosci.org /cgi/content/full/22/18/7959 (6030 words)

	Late Onset Death of Motor Neurons in Mice Overexpressing Wild-Type Peripherin -- Beaulieu et al. 147 (3): 531 -- The ...
		No peripherin signal was detected in motor neurons of WT mice (C), whereas motor neurons of transgenic mice exhibited a strong diffuse labeling for peripherin in their perikarya (fl arrowhead in D).
		(B) Northern blot analysis of peripherin mRNA from the brain and DRG in the presence and absence of NF-L. The blots were hybridized with the peripherin probe, stripped, rehybridized with the NF-L probe, stripped again, and rehybridized with the actin probe.
		Peripherin does not substitute for NF-L. (A–D) Electron microscopy of large myelinated axons from the ventral root of WT (A), L-/- (B), TPer (C), and TPerL-/- (D) mice.
	www.jcb.org /cgi/content/full/147/3/531 (7986 words)

	Connexion (Site not responding. Last check: )
		The identification of an exon-2 peripherin mutation in a family with heterogeneous manifestations of a butterfly pattern macular dystrophy.
		Screening for mutations in the peripherin gene of patients with autosomal dominant retinitis pigmentosa.
		Photoreceptor peripherin is the normal product of the gene responsible for retinal degeneration in the rds mouse.
	www.dsi.univ-paris5.fr /genatlas/tbiblio.php?symbol=RDS (706 words)

	[No title] (Site not responding. Last check: )
		Mouse 'retinal degeneration, slow' (rds) is a phenotype characterized by abnormal development of photoreceptor outer segments in the retina followed by a slow degeneration of rods and cones, resembling the abnormalities seen in human retinopathies.
		The peripherin that is mutant in rds of the mouse is a different protein from the peripherin (Prph) encoded on mouse chromosome 15 (Pendleton et al., 1991); see 170710.
		(1991) reported that the amino acid sequence of the bovine photoreceptor cell protein peripherin is 92.5% identical to the sequence of the mouse protein encoded by the normal rds gene.
	zeta.embl-heidelberg.de:8000 /srs5bin/cgi-bin/wgetz?-e+[omim-id:179605] (4310 words)

	Identification of a peripherin dimer: changes during axonal development and regeneration of the rat sciatic nerve. (Site not responding. Last check: )
		We showed that this 116/130 kDa protein is a disulfide dimer of peripherin, because it gave rise to a single protein band comigrating with peripherin under reducing conditions and yielded the same proteolytic pattern as peripherin upon N-chlorosuccinimide digestion.
		We investigated the changes in peripherin monomer and dimer protein levels during axonal development and regeneration.
		During postnatal development, quantitative analysis of western blots of DRG proteins showed a significant increase in peripherin monomer (+52%) and dimer (+33%) levels from the day of birth [postnatal day 0 (P0)] to P7.
	www.arclab.org /medlineupdates/abstract_8158137.html (326 words)

	The C Terminus of Peripherin/rds Participates in Rod Outer Segment Targeting and Alignment of Disk Incisures -- Tam et ...
		Colville, C.A., and Molday, R.S. Primary structure and expression of the human beta-subunit and related proteins of the rod photoreceptor cGMP-gated channel.
		Connell, G., Bascom, R., Molday, L., Reid, D., McInnes, R.R., and Molday, R.S. Photoreceptor peripherin is the normal product of the gene responsible for retinal degeneration in the rds mouse.
		Molday, R.S., Hicks, D., and Molday, L. Peripherin.
	www.molbiolcell.org /cgi/content/full/15/4/2027 (6603 words)

	Conrad Leung, Ph.D.
		Recently, we sequenced the peripherin gene of sporadic ALS patients and identified one individual with a homozygous mutation in exon 1.
		By transient transfection, we studied the assembly properties of mutant peripherin and found that it is prone to form aggregates.
		Because peripherin is expressed chiefly in the peripheral nervous system, it is a good candidate gene to be involved in the development of ALS.
	156.111.235.11 /pharm/cumc/profile.php?id=175 (404 words)

	A Frameshift Deletion in Peripherin Gene Associated with Amyotrophic Lateral Sclerosis -- Gros-Louis et al. 279 (44): ...
		Peripherin is a neuronal intermediate filament associated with
		The peripherin species were detected (green) with the monoclonal antibody (MAB1527) and the NF-L (red) with polyclonal antibody (AB1983).
		Peripherin (green) was detected with monoclonal antibody (MAB1527) and for NF-M with polyclonal antibody (AB1982).
	www.jbc.org /cgi/content/full/279/44/45951 (3403 words)

	Energy Citations Database (ECD) - Energy and Energy-Related Bibliographic Citations (Site not responding. Last check: )
		The authors determined the complete nucleotide sequence of the coding region of the human peripherin gene (PRPH), as well as 742 bp 5{prime} to the cap site and 584 bp 3{prime} to the stop codon, and compared its structure and sequence to the rat and mouse genes.
		The nucleotide sequences of the human peripherin gene exons were 90% identical to the rat gene sequences, and the predicted human peripherin protein differed from rat peripherin at only 18 of 475 amino acid residues.
		Comparison of the 5{prime} flanking regions of the human peripherin gene and rodent genes revealed extensive areas of high homology.
	www.osti.gov /energycitations/product.biblio.jsp?osti_id=250000 (300 words)

	Rat Genome Database: References
		We have cloned the rat gene encoding peripherin, a neuronal-specific intermediate filament protein that is NGF-regulated.
		Determination of the complete sequence, including 821 nucleotides of the 5'-flanking region, allows us to make conclusions about the evolutionary origin of the peripherin gene, its homology with other intermediate filament proteins, and possible mechanisms of regulation of peripherin expression in neurons.
		Together with protein sequence homologies, this conclusively demonstrates that peripherin is a type III intermediate filament protein.
	rgd.mcw.edu /tools/references/references_view.cgi?id=633741 (182 words)

	Peripherin, clone 7C5 : MAB5380
		Subsequently it was found in some sensory and other neurons of the central nervous system and also in PC12 cells.
		Peripherin belongs to the Class III family of intermediate filament subunits which includes vimentin, glial fibrillary acidic protein (GFAP) and desmin.
		By western blot, the antibody detects a band migrating at approximately 57 kDa corresponding to peripherin;
	www.chemicon.com /product/ProductDataSheet.asp?ProductItem=MAB5380 (314 words)

	Murine model of autosomal dominant retinitis pigmentosa generated by targeted deletion at codon 307 of the ...
		Peripherin is a transmembrane protein of outer segment disc
		is due to the presence of mutant peripherin protein and not
		23 Connell, G.J. and Molday, R.S. (1990) Molecular cloning, primary structure, and orientation of the vertebrate photoreceptor cell protein peripherin in the rod outer segment disk membrane.
	hmg.oxfordjournals.org /cgi/content/full/11/9/1005 (5187 words)

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