Abstract:
:Alexander disease (AxD) is a rare neurodegenerative disorder characterized by large cytoplasmic aggregates in astrocytes and myelin abnormalities and caused by dominant mutations in the gene encoding glial fibrillary acidic protein (GFAP), the main intermediate filament protein in astrocytes. We tested the effects of three mutations (R236H, R76H and L232P) associated with AxD in cells transiently expressing mutated GFAP fused to green fluorescent protein (GFP). Mutated GFAP-GFP expressed in astrocytes formed networks or aggregates similar to those found in the brains of patients with the disease. Time-lapse recordings of living astrocytes showed that aggregates of mutated GFAP-GFP may either disappear, associated with cell survival, or coalesce in a huge juxtanuclear structure associated with cell death. Immunolabeling of fixed cells suggested that this gathering of aggregates forms an aggresome-like structure. Proteasome inhibition and immunoprecipitation assays revealed mutated GFAP-GFP ubiquitination, suggesting a role of the ubiquitin-proteasome system in the disaggregation process. In astrocytes from wild-type-, GFAP-, and vimentin-deficient mice, mutated GFAP-GFP aggregated or formed a network, depending on qualitative and quantitative interactions with normal intermediate filament partners. Particularly, vimentin displayed an anti-aggregation effect on mutated GFAP. Our data indicate a dynamic and reversible aggregation of mutated GFAP, suggesting that therapeutic approaches may be possible.
journal_name
Exp Cell Resjournal_title
Experimental cell researchauthors
Mignot C,Delarasse C,Escaich S,Della Gaspera B,Noé E,Colucci-Guyon E,Babinet C,Pekny M,Vicart P,Boespflug-Tanguy O,Dautigny A,Rodriguez D,Pham-Dinh Ddoi
10.1016/j.yexcr.2007.04.035subject
Has Abstractpub_date
2007-08-01 00:00:00pages
2766-79issue
13eissn
0014-4827issn
1090-2422pii
S0014-4827(07)00212-1journal_volume
313pub_type
杂志文章abstract::Clear cell renal cell carcinoma (ccRCC) is one of the most common malignant tumors in the urinary system, and its incidence continues to increase. Regulator of calcineurin 1 (RCAN1), one of the genes on chromosome 21, is a crucial mediator of tumor inhibition. RCAN1.4 is best characterized as an endogenous inhibitor o...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/j.yexcr.2018.09.017
更新日期:2018-11-15 00:00:00
abstract::In this report, we elucidate the role of Na(+)-K+ pump in the regulation of polyamine spermidine (Spd) transport in murine leukemia (L 1210) cells in culture. Ouabain, known to bind extracellularly to the alpha-subunit of the Na(+)-K+ pump, inhibits the pump activity. The L 1210 cells were found to possess ouabain bin...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/0014-4827(92)90448-h
更新日期:1992-04-01 00:00:00
abstract::The technique of fiber DNA measurement was used to study the possibility that the lengthening of the DNA "S" phase previously reported for mouse premeiotic spermatogonia was due to a reduced number of initiation sites. The mean replicon size of neonatal mouse preleptotene cells was similar to sizes reported for adult ...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/0014-4827(83)90130-1
更新日期:1983-07-01 00:00:00
abstract::Vegetative amoebae of Dictyostelium discoideum (strain AX2) adhere to columns of hydrophobic beads (Bio-Beads SM-2) which have been coated with axenic growth medium. This binding is blocked by sodium azide and other inhibitors of aerobic respiration. Bound cells are not detached by lowering the temperature but they ca...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/0014-4827(84)90235-0
更新日期:1984-05-01 00:00:00
abstract::Based on data from developmental RNA profiles and in situ hybridization, we report a direct examination of the expression of one collagen gene (Dcg1) during drosophila melanogaster life cycle. These studies show, for the first time, that the expression of a collagen gene is both differential and tissue-specific during...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/0014-4827(86)90071-6
更新日期:1986-04-01 00:00:00
abstract::Interference of colchicine with meiotic chromosome pairing in the wild garlic, Allium ursinum, was studied using a whole-mount spreading technique for synaptonemal complexes. Colchicine was found to cause (i) pairing suppression (arrest of leptotene) and (ii) deficient pairing initiation at zygotene in connection with...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/0014-4827(88)90381-3
更新日期:1988-09-01 00:00:00
abstract::Murine embryonal carcinoma F9 cells can be induced to differentiate by 2-difluoromethylornithine (DFMO), an irreversible inhibitor of ornithine decarboxylase (ODC). The differentiated phenotype is similar to that of retinoic acid (RA)-treated F9 cells. In contrast to F9 cells the differentiated cells secrete plasminog...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/0014-4827(85)90276-9
更新日期:1985-01-01 00:00:00
abstract::The effect of gestational age on serum-mediated changes in enolase activity was tested in a fetal rat brain cell culture. After 6 days exposure to graded concentrations (10 and 20%) of nonpregnant female rat sera, enolase activity in brain cell cultures increased from 2.83 +/- 0.03 to 3.74 +/- 0.19 mumol/min/mg protei...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/0014-4827(90)90298-o
更新日期:1990-02-01 00:00:00
abstract::A portion of the DNA within intact nuclei of a spontaneously transformed Chinese hamster ovary cell line (CHO-Kl) is relatively resistant to digestion by pancreatic deoxyribonuclease, as compared to nuclei from primary cultures of Chinese hamster ovary fibroblasts. Treatment of CHO-Kl cells with derivatives of 3',5' c...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/s0014-4827(83)80007-x
更新日期:1983-04-15 00:00:00
abstract::Elastin, a major constituent of the media, may play an important role in the attachment, migration, and phenotypic properties of vascular smooth muscle cells (SMC). We examined binding proteins and binding sites on elastin in rabbit arterial SMC in primary culture. Freshly isolated cells on alpha-elastin underwent phe...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1006/excr.1995.1164
更新日期:1995-05-01 00:00:00
abstract::Oxysterol-binding protein (OSBP)-related proteins (ORPs) are conserved lipid binding proteins found in organisms ranging from yeast to mammals. Recent findings have indicated that these proteins mainly localize to contact sites of 2 different membranous organelles. ORP6, a member of the ORP subfamily III, is one of th...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/j.yexcr.2018.07.025
更新日期:2018-09-15 00:00:00
abstract::In this communication, we have studied a 72,000 mol w (p72) host protein which reacts with a mouse monoclonal antibody (PAb6) directed against antigenic determinants on the Simian virus 40 (SV40) large T antigen protein that map 5' of 0.42 map units on the viral genome. The p72 protein is an abundant basic (pI greater...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/0014-4827(88)90398-9
更新日期:1988-10-01 00:00:00
abstract::Uterine fibroids, also known as uterine leiomyomas, are a benign tumor of the human uterus and the commonest estrogen-dependent benign tumor found in women. Myocardin is an important transcriptional regulator in smooth and cardiac muscle development. The role of myocardin and its relationship with ERα in uterine fibro...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/j.yexcr.2016.11.007
更新日期:2017-01-01 00:00:00
abstract::Ubiquitylation of membrane receptors is recognised as a critical post-translational modification, governing their regulation and function. Following ubiquitylation, membrane proteins may be internalised, recycled or degraded via lysosomal or proteasomal pathways. Viruses have appropriated these cellular pathways as a ...
journal_title:Experimental cell research
pub_type: 杂志文章,评审
doi:10.1016/j.yexcr.2008.10.026
更新日期:2009-05-15 00:00:00
abstract::The Golgi apparatus plays roles in cell polarity, directional cell migration, and bipolar spindle assembly, as well as the secretary pathway. In addition, recent studies have suggested the Golgi-dependent control of mitotic entry. We studied the role of the centrosomal kinase Aurora A in maintaining the Golgi apparatu...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/j.yexcr.2018.03.024
更新日期:2018-06-01 00:00:00
abstract::Pancreatic cancer is characterized by aggressive local invasion and early metastasis formation. Active migration of the pancreatic cancer cells is essential for these processes. We have shown previously that the pancreatic cancer cells lines CFPAC1 and IMIM-PC2 show high migratory activity, and we have investigated he...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/j.yexcr.2014.04.022
更新日期:2014-08-15 00:00:00
abstract::Basic fibroblast growth factor (bFGF) binding to ocular tissues has been studied by autoradiographical and biochemical approaches directly performed on sections during mouse embryonic and postnatal development. Frozen sections of embryos (9 to 18 days), newborns, and adults (1 day to 6 months) were incubated with iodi...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/0014-4827(90)90280-n
更新日期:1990-05-01 00:00:00
abstract::We have studied the effects of the phorbol ester tumor promoter 12-O-tetradecanoyl-phorbol-13-acetate (TPA) on the contractility, locomotion, morphology, and adhesion of two mammalian fibroblastic cell lines. Using the silicone rubber substratum technique, we have found that the first observable response to the tumor ...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/0014-4827(88)90024-9
更新日期:1988-07-01 00:00:00
abstract::The duplication and segregation of the genome during cell division is crucial to maintain cell identity, development of organisms and tissue maintenance. Centromeres are at the basis of accurate chromosome segregation as they define the site of assembly of the kinetochore, a large complex of proteins that attaches to ...
journal_title:Experimental cell research
pub_type: 杂志文章,评审
doi:10.1016/j.yexcr.2020.111909
更新日期:2020-04-15 00:00:00
abstract::Human calmodulin-like protein (CLP) is a calcium-binding protein down-regulated in a cell culture model of mammary tumorigenesis as well as in a majority of breast cancers in vivo. CLP down-regulation may be a result of the poorly differentiated state of these cell lines and tumors, or CLP expression may be incompatib...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1006/excr.2001.5254
更新日期:2001-07-15 00:00:00
abstract::Nitric oxide (NO) markedly induces cardiomyocyte (CM) differentiation of embryonic stem (ES) cells. Here we examined the role of the Ca(2+) signaling in the NO-induced CM differentiation of mouse ES cells. We found that NO induced intracellular Ca(2+) increases in ES cells in a dose-dependent manner, and application o...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/j.yexcr.2016.06.016
更新日期:2016-08-01 00:00:00
abstract::It has been known for at least 20 years that growth factors induce the internalization of cognate receptor tyrosine kinases (RTKs). The internalized receptors are then sorted to lysosomes or recycled to the cell surface. More recently, data have been published to indicate other intracellular destinations for the inter...
journal_title:Experimental cell research
pub_type: 杂志文章,评审
doi:10.1016/j.yexcr.2008.09.027
更新日期:2009-05-15 00:00:00
abstract::Release of mitochondrial cytochrome c has been recently linked to the activation of the "executioner" phase of the cellular programs for death by apoptosis. This release is known to be negatively regulated by Bcl-2 and Bcl-XL proteins. We show here that treatment of human leukemia cells HL60 with 1,25-dihydroxyvitamin...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1006/excr.1997.3667
更新日期:1997-08-25 00:00:00
abstract::Synthetic tubugis are equally potent but more stable than their natural forms. Their anticancer potential was estimated on a solid melanoma in vitro and in vivo. Tubugi-1 induced the apoptosis in B16 cells accompanied with strong intracellular production of reactive species, subsequently imposing glutathione and thiol...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/j.yexcr.2019.04.028
更新日期:2019-07-15 00:00:00
abstract::The establishment of cell lines allows reproductible in vitro studies that would be far more difficult to perform using primary cells that rapidly undergo phenotypical alterations in culture. The purpose of this work was to establish an endothelial cell line appropriate for in vitro study of endothelial cell activatio...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1006/excr.1997.3831
更新日期:1998-01-10 00:00:00
abstract::Monoclonal antibodies binding to different domains of nucleolin have been used to localize nucleolin in tissue culture cells of Xenopus laevis. The monoclonal antibody b6-6E7 binds to an epitope in the N-terminal domain, which contains arrays of phosphorylation consensus sites. This monoclonal antibody binds to nucleo...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1006/excr.1997.3878
更新日期:1998-03-15 00:00:00
abstract::As retinal histogenesis proceeds there is a pronounced increase in the expression of fibroblast growth factor (FGF), reaching its maximum in the mature retina and largely in terminal differentiated retinal neurons. Recent in vivo evidence suggests that exogenous FGF functions as a differentiation and survival factor f...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1006/excr.1998.4048
更新日期:1998-05-25 00:00:00
abstract::Durations of S-phase (Ts) and total cell cycle times (Tc) were measured from the peripheral blood (PB) and bone marrow aspirates (BM) of five patients with acute nonlymphocytic leukemia (ANLL). Intravenous bromodeoxyuridine (BrdU) was used as the first label for S-phase cells and a monoclonal anti-BrdU antibody was us...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/0014-4827(88)90116-4
更新日期:1988-05-01 00:00:00
abstract::Hepatocellular carcinoma (HCC) is one of the most common cancers diagnosed worldwide. However, the mechanism underlying HCC pathogenesis remains unknown. In the present study, TRIM24 was found increased in human HCC clinical samples and positively correlated with HCC tumor grade. Furthermore, TRIM24 knockdown inhibits...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/j.yexcr.2018.04.006
更新日期:2018-06-15 00:00:00
abstract::Protein kinase B (Akt1) holds a central role for cellular growth, development, and survival, but the cellular pathways of Akt1 that prevent inflammatory demise in the vascular system remain undefined. Employing a constitutively active form of Akt1 (myristoylated Akt1) in endothelial cells (ECs), we demonstrate that Ak...
journal_title:Experimental cell research
pub_type: 杂志文章
doi:10.1016/j.yexcr.2004.01.021
更新日期:2004-06-10 00:00:00