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Beaded filament structural protein 1

CP94, BFSP1, CP-115
structural protein expressed only in the lens fiber cells which has a beaded filament structure [RGD, Feb 2006] (from NCBI)
Top mentioned proteins: ACID, CAN, 5-HT1B, HAD, V1a
Papers on CP94
Sporadic and Familial Congenital Cataracts: Mutational Spectrum and new Diagnoses using Next-Generation Sequencing.
Jamieson et al., Sydney, Australia. In Hum Mutat, Jan 2016
Mutations were also found in rarely reported genes including MAF, VIM, MIP, and BFSP1.
Proteomics of human aqueous humor.
Pandey et al., Bengaluru, India. In Omics, May 2015
Sorbitol dehydrogenase (SORD), filensin (BFSP1), and phakinin (BFSP2) are some of the proteins that have not been previously reported in the aqueous humor.
Activity of quinolone CP-115,955 against bacterial and human type II topoisomerases is mediated by different interactions.
Osheroff et al., Nashville, United States. In Biochemistry, Mar 2015
CP-115,955 is a quinolone with a 4-hydroxyphenyl at C7 that displays high activity against both bacterial and human type II topoisomerases.
The effects of protoporphyrin IX-induced photodynamic therapy with and without iron chelation on human squamous carcinoma cells cultured under normoxic, hypoxic and hyperoxic conditions.
Curnow et al., Truro, United Kingdom. In Photodiagnosis Photodyn Ther, 2013
METHODS: Cells were incubated at concentrations of 5%, 20% or 40% oxygen for 24h prior to and for 3h following the administration of the PpIX precursors aminolevulinic acid (ALA), methyl aminolevulinate (MAL) or hexylaminolevulinate (HAL) with or without the iron chelator 1,2-diethyl-3-hydroxypyridin-4-one hydrochloride (CP94).
Molecular and structural analysis of genetic variations in congenital cataract.
Dada et al., New Delhi, India. In Mol Vis, 2012
Crystallin, alpha A (CRYAA), CRYAB, CRYGs, CRYBA1, CRYBA4, CRYBB1, CRYBB2, CRYBB3, beaded filament structural protein 1 (BFSP1), gap function protein, alpha 3 (GJA3), GJA8, and heat shock transcription factor 4 gene genes were amplified.
A novel beaded filament structural protein 1 (BFSP1) gene mutation associated with autosomal dominant congenital cataract in a Chinese family.
Zhang et al., China. In Mol Vis, 2012
RESULTS: A novel beaded filament structural protein 1 (BFSP1) gene missense mutation was identified.
A missense mutation in CRYGD linked with autosomal dominant congenital cataract of aculeiform type.
Singh et al., Amritsar, India. In Mol Cell Biochem, 2012
Mutation screening in twenty three candidate genes including crystallins (CRYAA, CRYAB, CRYBA1/A3, CRYBA2, CRYBA4, CRYBB1, CRYBB2, CRYBB3, CRYGA, CRYGB, CRYGC, CRYGD, and CRYGS), gap junctional channels; connexins (GJA8, GJA3), beaded filament chain proteins (BFSP1, BFSP2), major intrinsic protein (MIP), lens intrinsic membrane protein-2 (LIM2), transcriptional factor (MAF), and in genes encoding for membrane-associated proteins (TMEM114, CHMP4B, EPHA2) was performed by bi-directional sequence analysis of the amplified products.
Infantile spasms: treatment challenges.
Watemberg, Tel Aviv-Yafo, Israel. In Curr Treat Options Neurol, 2012
Finally, a very promising drug has been developed (CP-115) that may altogether replace the current therapeutic regimens in the near future.
Efficient generation of lens progenitor cells from cataract patient-specific induced pluripotent stem cells.
Lu et al., Shanghai, China. In Plos One, 2011
Next, we performed a 3-step induction procedure: HLE-iPS cells were differentiated into large numbers of lens progenitor-like cells with defined factors (Noggin, BMP and FGF2), and we determined that these cells expressed lens-specific markers (PAX6, SOX2, SIX3, CRYAB, CRYAA, BFSP1, and MIP).
An in vitro comparison of the effects of the iron-chelating agents, CP94 and dexrazoxane, on protoporphyrin IX accumulation for photodynamic therapy and/or fluorescence guided resection.
Curnow et al., Truro, United Kingdom. In Photochem Photobiol, 2011
Two iron-chelating agents, 1,2-diethyl-3-hydroxypyridin-4-one hydrochloride (CP94) and dexrazoxane, were individually combined with the porphyrin precursors aminolevulinic acid (ALA), methyl aminolevulinate (MAL) and hexyl aminolevulinate (HAL).
Functions of the intermediate filament cytoskeleton in the eye lens.
Quinlan et al., Boston, United States. In J Clin Invest, 2009
This Review focuses on the lens-specific IF proteins beaded filament structural proteins 1 and 2 (BFSP1 and BFSP2) and their role in lens physiology and disease.
Removal of Hsf4 leads to cataract development in mice through down-regulation of gamma S-crystallin and Bfsp expression.
Hu et al., Shanghai, China. In Bmc Mol Biol, 2008
disruption of the Hsf4 gene leads to cataracts via at least three pathways: down-regulation of gamma-crystallin, particularly gamma S-crystallin; decreased lens beaded filament expression; and loss of post-translational modification of alpha A-crystallin
The function of filensin and phakinin in lens transparency.
Takehana et al., Tokyo, Japan. In Mol Vis, 2007
The degradation of filensin and phakinin in normal and cataractous rat lenses, was examined.
The roles of dopamine and serotonin, and of their receptors, in regulating sleep and waking.
Jantos et al., Montevideo, Uruguay. In Prog Brain Res, 2007
Recently, DA neurons were characterised in the ventral periaqueductal grey matter (VPAG) that express Fos protein during W. Lesioning of these cells resulted in an increase of SWS and REMS, which led to the proposal that VPAG DA neurons may play a role in the promotion of W. Systemic injection of full agonists at postsynaptic 5-HT(1A) (8-OH-DPAT, flesinoxan), 5-HT(1B) (CGS 12066B, CP-94,253), 5-HT(2A/2C) (DOI, DOM) and 5-HT(3) (m-chlorophenylbiguanide) receptors increases W and reduces SWS and REMS.
Insights into the beaded filament of the eye lens.
Quinlan et al., Durham, United Kingdom. In Exp Cell Res, 2007
Filensin (BFSP1) and CP49 (BFSP2) represent two members of the IF protein superfamily that are thus far exclusively expressed in the eye lens.
Autosomal recessive juvenile onset cataract associated with mutation in BFSP1.
Hejtmancik et al., Madurai, India. In Hum Genet, 2007
This is the first report of a mutation in the BFSP1 gene associated with human inherited cataracts.
Seeing is believing! The optical properties of the eye lens are dependent upon a functional intermediate filament cytoskeleton.
Quinlan et al., Durham, United Kingdom. In Exp Cell Res, 2005
The generation of gene knockouts by the targeted deletion of Bfsp1 and Bfsp2 that encode filensin and CP49, respectively, have been made to explore the function of beaded filaments in the lens.
Effects of selected serotonin 5-HT(1) and 5-HT(2) receptor agonists on feeding behavior: possible mechanisms of action.
Schreiber et al., Wuppertal, Germany. In Neurosci Biobehav Rev, 2000
The present paper reviews studies performed with the relatively selective receptor agonists ipsapirone (5-HT(1A)), CP-94,253 (5-HT(1B)), BW 723C86 (5-HT(2B)) and ORG 37684 (5-HT(2C)), as well as the nonselective receptor agonists TFMPP (5-HT(1B/2C)), m-CPP (5-HT(2C/1B)) and DOI (5-HT(2A/2C)) in a variety of feeding paradigms in rats, both after systemic and local injection.
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