Abstract:
:Retinitis pigmentosa (RP) is a heterogeneous group of inherited retinal degenerative diseases, characterized by the progressive death of rod and cone photoreceptors. A tremendous genetic heterogeneity is associated with the RP phenotype. Most mutations affect rods selectively and, through an unknown pathway, cause the rod cells to die by apoptosis. Cones, on the other hand, are seldom directly affected by the identified mutations, and yet, in many cases, they degenerate secondarily to rods, which accounts for loss of central vision and complete blindness. Many animal models of RP are available and have led to a better understanding of the disease and to the development of therapeutic strategies aimed at curing the specific genetic disorder (gene therapy), slowing down or even stopping the process of photoreceptor degeneration (growth factors or calcium blockers applications, vitamin supplementation), preserving the cones implicated in the central visual function (identification of endogenous cone viability factors) or even replacing the lost cells (transplantation, use of stem or precursor cells). Still, many obstacles will need to be overcome before most of these strategies can be applied to humans. In this review, we describe the different therapeutic strategies being studied worldwide and report the latest results in this field.
journal_name
Biol Celljournal_title
Biology of the cellauthors
Delyfer MN,Léveillard T,Mohand-Saïd S,Hicks D,Picaud S,Sahel JAdoi
10.1016/j.biolcel.2004.01.006subject
Has Abstractpub_date
2004-05-01 00:00:00pages
261-9issue
4eissn
0248-4900issn
1768-322Xpii
S0248490004000346journal_volume
96pub_type
杂志文章,评审abstract:BACKGROUND INFORMATION:Human gingival tissues are prone to hyperplasia under inflammatory stimuli. We have identified gingival tissue-specific mesenchymal stem cells (GMSCs) and found their functional change being correlated with drug-induced gingival hyperplasia. However, whether these cells exhibit characteristics of...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1111/boc.201200064
更新日期:2013-06-01 00:00:00
abstract::The nucleolar lesions provoked by the action of ribonuclease (RNase) on living chick embryo fibroblasts were studied by means of microcinematographic analysis and at the ultrastructural level using oxidized diaminobenzidine as a differentially contrasting stain for nucleic acids. This study has shown that the inductio...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1111/j.1768-322x.1984.tb00218.x
更新日期:1983-01-01 00:00:00
abstract::Flow cytometry has important advantages over conventional techniques. It is rapid, highly sensitive and allows multi-parametric analysis and cell sorting. Potential exists for the measurement of many cell functions by flow cytometry. The technique can be used to determine cell viability, intracellular calcium and pH, ...
journal_title:Biology of the cell
pub_type: 杂志文章,评审
doi:10.1016/0248-4900(93)90119-y
更新日期:1993-01-01 00:00:00
abstract::Tensins are focal adhesion molecules that were identified and characterised in the late 1980s to early 1990s. They play an essential role in the control of cell adhesion. Tensins can bind the tail of ß integrin via their phospho tyrosine binding domain, they exhibit various protein interaction domains including a Src ...
journal_title:Biology of the cell
pub_type: 杂志文章,评审
doi:10.1111/boc.201600053
更新日期:2017-03-01 00:00:00
abstract::Interactions of cells with the extracellular matrix are essential for the control of tissue remodelling, cell migration, and embryogenesis. At the cell-extracellular matrix contact points, specialized structures are formed and termed focal adhesions, where transmembrane adhesion receptors provide a structural link bet...
journal_title:Biology of the cell
pub_type: 杂志文章,评审
doi:10.1016/s0248-4900(00)01101-1
更新日期:2000-10-01 00:00:00
abstract:BACKGROUND:The translocase of the mitochondrial inner membrane (TIM) imports most of the nucleus-encoded proteins that are destined for the matrix, inner membrane (IM) and the intermembrane space (IMS). Trypanosoma brucei, the infectious agent for African trypanosomiasis, possesses a unique TIM complex consisting of se...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1111/boc.202000054
更新日期:2020-10-20 00:00:00
abstract::The quick-freeze, freeze-fracture, deep-etching and rotary replication techniques were used to analyze the structural organization of Giardia duodenalis. The surface of the flagella was rugous, in contrast to the cell body surface which was smooth. The ventral region was characterized in more detail, exposing the laye...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1016/0248-4900(96)84780-0
更新日期:1996-01-01 00:00:00
abstract::Interaction of sperm and egg at fertilization induces well-coordinated molecular events including specific recognition between species, adhesion and fusion, that lead to the formation of a zygote, a totipotent cell that develops into a new individual. A calcium signal, common to a great number of species, from marine ...
journal_title:Biology of the cell
pub_type: 杂志文章,评审
doi:10.1016/s0248-4900(00)01065-0
更新日期:2000-07-01 00:00:00
abstract::Much remains to be learned about how cancer cells acquire the property of migration, a prerequisite for invasiveness and metastasis. Loss of p53 functions is assumed to be a crucial step in the development of many types of cancers, leading to dysregulation of cell cycle checkpoint controls and apoptosis. However, emer...
journal_title:Biology of the cell
pub_type: 杂志文章,评审
doi:10.1042/BC20050058
更新日期:2006-03-01 00:00:00
abstract::The activity and the tissue distribution of the oxygen radical producing enzyme xanthine oxidoreductase (XOR) were measured in the digestive gland of the common marine mussel Mytilus galloprovincialis Lmk along an annual cycle. No xanthine oxidase (XOX) activity could be measured, the enzyme only displaying xanthine d...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:
更新日期:1999-11-01 00:00:00
abstract::Immunofluorescent techniques have been used in the analysis of DNA-RNA hybrids occurrence and its relationship to transcriptional events on polytene chromosomes of Drosophila subobscura. We have studied the distribution of these hybrids on uninduced/induced chromosomes. Two different indirect immunofluorescence method...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1016/0248-4900(92)90140-v
更新日期:1992-01-01 00:00:00
abstract:BACKGROUND INFORMATION:CLEM (correlative live cell and electron microscopy) seeks to bridge the data acquired with different imaging strategies, typically between light microscopy and electron microscopy. It has been successfully applied in cell cultures, although its use in multicellular systems is hampered by difficu...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1042/BC20090096
更新日期:2009-12-04 00:00:00
abstract:BACKGROUND INFORMATION:The role of the LIM-domain-containing protein Ajuba was initially described in cell adhesion and migration processes and recently in mitosis as an activator of the Aurora A kinase. RESULTS:In the present study, we show that Ajuba localizes to centrosomes and kinetochores during mitosis. This loc...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1042/BC20080060
更新日期:2009-04-01 00:00:00
abstract:BACKGROUND INFORMATION:The ribonucleases (RNases) constitute a heterogeneous group of enzymes, which exert diverse and specific biological functions. Several RNases have been shown to control gene expression and cell differentiation. RNASET2, a novel member of the Rh/T2/S family of RNases, exerts micro-environmental co...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1111/boc.201100092
更新日期:2012-01-01 00:00:00
abstract::Alpha 2-macroglobulin (alpha 2M) is a plasma inhibitor of proteinases, the steric mechanism of which is based on a considerable conformational change. The typical and distinct H-like shape of alpha 2M-chymotrypsin (alpha 2M-chy) complexes seen by electron microscopy led us to an ultrastructural study of the binding of...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1016/0248-4900(88)90092-5
更新日期:1988-01-01 00:00:00
abstract::Ultrastructural morphometry (point-counting method) was used to evaluate stereological parameters of rough endoplasmic reticulum (RER), Golgi complex, secretory granules, mitochondria and lysosomes of Rana ridibunda pituitary corticotropic (ACTH) cells in animals subjected to constant environmental conditions (CEC) in...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1111/j.1768-322x.1986.tb00472.x
更新日期:1986-01-01 00:00:00
abstract::Previous studies suggest that oligodeoxynucleotide (ODN) cellular uptake is cell cycle-dependent which may have important implications in cancer cell targeting. To further our understanding of ODN transport and activity, this study examines the relationships between the cell cycle, ODN cellular uptake, intracellular t...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:
更新日期:1997-07-01 00:00:00
abstract::The microtubule-associated proteins MAPs 1 and 2 from pig brain have been found to react with antibodies directed against human ankyrin and spectrin, respectively (Bennett and Davis, 1981; Davis and Bennett, 1982). In a complementary approach we have prepared antibodies against MAP1 alpha. MAP1 gamma and MAP2 purified...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1111/j.1768-322x.1985.tb00361.x
更新日期:1985-01-01 00:00:00
abstract::The lateral line of the zebrafish has many of the advantages that made the sensory organs of Drosophila a very productive model system: 1) it comprises a set of discrete sense organs (neuromasts) arranged in a defined, species-specific pattern, such that each organ can be individually recognized; 2) the neuromasts are...
journal_title:Biology of the cell
pub_type: 杂志文章,评审
doi:10.1016/j.biolcel.2003.10.005
更新日期:2003-12-01 00:00:00
abstract::The communication of calcium signals between cells is known to be operative between neurons where these signals integrate intimately with electrical and chemical signal communication at synapses. Recently, it has become clear that glial cells also exchange calcium signals between each other in cultures and in brain sl...
journal_title:Biology of the cell
pub_type: 杂志文章,评审
doi:10.1016/j.biolcel.2003.10.007
更新日期:2004-02-01 00:00:00
abstract:BACKGROUND INFORMATION:The interferon (IFN)-inducible protein TRIM22 (Staf50) is a member of the tripartite motif protein family and has been suggested a role in the regulation of viral replication as well as of protein ubiquitylation. In addition, we have previously shown that TRIM22 is a direct target gene for the tu...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1111/boc.201100099
更新日期:2012-08-01 00:00:00
abstract::Since almost two decades, it is known that progesterone is responsible of the release of the prophase I arrest of amphibian oocytes and leads to the activation of the universal MPF, through a puzzling transduction pathway. It involves negative regulation of the cAMP-dependent protein kinase (PKA) and synthesis of new ...
journal_title:Biology of the cell
pub_type: 杂志文章,评审
doi:10.1016/s0248-4900(01)01126-1
更新日期:2001-09-01 00:00:00
abstract::After gradually moving away from preparation methods prone to artefacts such as plastic embedding and negative staining for cell sections and single particles, the field of cryo electron microscopy (cryo-EM) is now heading off at unprecedented speed towards high-resolution analysis of biological objects of various siz...
journal_title:Biology of the cell
pub_type: 杂志文章,评审
doi:10.1111/boc.201600042
更新日期:2017-02-01 00:00:00
abstract::As a rule, chloride movement (JC1-) across amphibian skin is considered to be passive; this is implied in fact for preparations incubated in Ringer's fluid, since short-circuit current (Isc) is the quantitative expression of net, active sodium transport (JNa+). The nature of the Cl- pathway(s) was investigated by incu...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:
更新日期:1989-01-01 00:00:00
abstract::Detergent extraction of human blood platelets pre-treated with Taxol to stabilize microtubules allows isolation of marginal band (MB) cytoskeletons. We studied MB cytoskeleton structure using dark-field light microscopy and negative stain electron microscopy (EM). Dark-field illumination clearly demonstrated the "hoop...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1016/0248-4900(88)90002-0
更新日期:1988-01-01 00:00:00
abstract::Primary cultures of cells from breast carcinomas were attempted in 74 cases. Growth was observed in 46 cases. Using immunochemical demonstration of keratin proteins (KER), epithelial membrane antigen (EMA) and carcinoembryonic antigen (CEA), three morphologically distinct cell populations were characterized and descri...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1111/j.1768-322x.1987.tb00574.x
更新日期:1987-01-01 00:00:00
abstract::We took advantage of one of the main possibilities of ion microscopy, ie isotopic analysis, to study the cellular distribution of molecules labelled either with carbon 14 or with stable isotopes of low natural abundance such as nitrogen 15 and deuterium. The surface of the sample is bombarded with an ion beam (O2+, Cs...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1016/0248-4900(92)90011-o
更新日期:1992-01-01 00:00:00
abstract:BACKGROUND INFORMATION:Available data concerning the sperm morphology of teleost fishes demonstrate wide variation. In the present study, the spermatozoa of Siberian sturgeon (Acipenser baerii Brandt, 1869), a chondrostean fish, was investigated. In contrast with teleost fish, chondrostean spermatozoa have a head with ...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1042/BC20060060
更新日期:2007-02-01 00:00:00
abstract::The low pH dependent membrane fusion reaction responsible for the delivery of the Semliki Forest virus genome into the host cell for replication was visualized by electron microscopy. In order to increase the frequency at which fusion images could be detected a reversible inhibitor, ammonium chloride, was used to sync...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1111/j.1768-322x.1984.tb00297.x
更新日期:1984-01-01 00:00:00
abstract::In eukaryotes transcription and translation takes place in two different compartments separated by the nuclear envelope. Nuclear translation has been reported in several publications, however, all these published results have been criticized, particularly with respect to cytoplasmic contamination of the nuclei. We hav...
journal_title:Biology of the cell
pub_type: 杂志文章
doi:10.1016/s0248-4900(03)00059-5
更新日期:2003-07-01 00:00:00