Abstract: The inventors demonstrate that mRNA sorting to the mitochondrial surface is an efficient way to proceed to such an allotopic expression, and that this mRNA sorting can be controlled by selecting appropriate mitochondrion-targeting sequence (MTS) and appropriate 3?UTR sequences. The CDS sequence which codes for the protein to be delivered into the mitochondrion is guided by these appropriate MTS and 3?UTR sequences from the nuclear compartment to the mitochondrion-bound polysomes (where the CDS is translated), and aids in an efficient translocation of a mature functional protein into the mitochondria. Appropriate MTS and 3?UTR sequences correspond to those of nuclearly-transcribed mitochondrially-targeted mRNAs. The inventors demonstrate that, to obtain a stable therapeutically-effective importation, both an appropriate MTS and an appropriate 3?UTR should preferably be used.

Abstract: The present invention concerns a neuroglobin agonist for use in the treatment or prevention of a mitochondrial disease associated with respiratory chain complex I (RCCI) deficiency and/or respiratory chain complex III (RCCIII) deficiency.

Abstract: An expression vector containing appropriate mitochondrion-targeting sequences (MTS) and appropriate 3?UTR sequences provides efficient and stable delivery of a mRNA encoding a protein (CDS) to the mitochondrion of a mammalian cell. The MTS and 3?UTR sequences guide the CDS mRNA from the nuclear compartment of the cell to mitochondrion-bound polysomes, where the CDS is translated. This provides an efficient translocation of a mature functional protein into the mitochondria. A method of targeting mRNA expressed in the nuclear compartment of a mammalian cell to the mitochondrion is also provided. The vector and methods can be used to treat defects in mitochondrial function.

Abstract: Disclosed are methods and compositions for early diagnosis, monitoring and treatment of retinal dystrophy, age-related macular degeneration, Bardet-Biedel syndrome, Bassen-kornzweig syndrome, best disease, choroidema, gyrate atrophy, congenital amourosis, refsun syndrome, stargardt disease and Usher syndrome. In particular, the invention relates to a protein, termed “Rdcvf1,” that is differentially transcribed and expressed in subjects suffering from retinal dystrophies and the like, such as retinal dystrophy and age-related macular degeneration compared with nonsufferers, antibodies which recognize this protein, and methods for diagnosing such conditions.

Abstract: The present invention relates to an isolated nucleic acid molecule comprising i) a nucleotide sequence coding for a hyperpolarizing light-gated ion channel or pump gene from an archeon or for a light-active fragment of said gene, or the nucleotide sequence and ii) a nucleotide sequence coding for a neurotrophic factor for use in the treatment of a retinal degenerative disease.

Abstract: The present invention relates to an in vitro method for diagnosing a complete congenital stationary night blindness (cCSNB) in a subject, which method comprises determining the presence of an alteration in the GPR179 gene in a biological sample of said subject. Screening methods and therapeutic applications are further described.

Abstract: An optical device including an optical substrate including a first surface having a first zone provided with first selective interferential filtering element for selectively inhibiting transmission of incident light based on the wavelength spectrum of the incident light, the first selective interferential filtering being configured to inhibit, at a first rate of rejection, transmission of a first selected range of wavelengths of incident light, incident on the first zone within a first selected range of angles of incidence, wherein the first selected range of angles of incidence is determined based on at least one main line of sight of a user.

Abstract: An expression vector containing appropriate mitochondrion-targeting sequences (MTS) and appropriate 3?UTR sequences provides efficient and stable delivery of a mRNA encoding a protein (CDS) to the mitochondrion of a mammalian cell. The MTS and 3?UTR sequences guide the CDS mRNA from the nuclear compartment of the cell to mitochondrion-bound polysomes, where the CDS is translated. This provides an efficient translocation of a mature functional protein into the mitochondria. A method of targeting mRNA expressed in the nuclear compartment of a mammalian cell to the mitochondrion is also provided. The vector and methods can be used to treat defects in mitochondrial function.

Abstract: A composition includes an extract of seeds of Bixa orellana for the photoprotection of the retinal pigment epithelium in mammals. The use of such a composition for the treatment of macular degeneration related to age (AMD) in mammals is also described.

Abstract: Method of determining configuration of interferential filtering elements for an optical device having an optical substrate for a user, includes: providing a first set of parameters representative of at least one main line of sight of the user; determining a first selected range of angles of incidence based on the first parameters; providing a second set of parameters characterizing, for the user, a range of wavelengths to be at least partially inhibited; determining a first selected range of wavelengths of incident light to be at least partially inhibited, based on the second parameters; and configuring a first selective interferential filtering element and a first zone of a surface of the optical substrate based on the first selected range of angles of incidence and wavelengths such that the first selective interferential filtering elements inhibit, at a first rate of rejection, transmission of the first selected range of wavelengths of incident light.

Abstract: An optical device including: an optical substrate provided with selective optical filtering element configured to selectively inhibit transmission, through the optical substrate of at least one selected range of wavelengths of incident light in the visible light spectrum at an inhibition rate of at least 5%, the selective optical filtering element being further configured to transmit at least 8% of incident light of the visible spectrum outside the at least one selected range of wavelengths; wherein the at least one selected range of wavelengths has a bandwidth in a range of from 10 nm to 70 nm, preferably 10 nm to 60 nm centered on a wavelength within a range of between 430 nm and 465 nm.

Abstract: This invention relates to methods and compositions for detection and treatment of neurodegenerative diseases. In particular, the invention relates to polypeptides that can protect against neuron degeneration, nucleic acid molecules that encode such polypeptides, and antibodies that recognize said polypeptides.

Abstract: The present invention relates to methods and compositions for inhibiting or stimulating angiogenesis. The invention shows the implication of Dp71 in angiogenesis and thus provides novel therapeutic approaches, as well as novel methods for screening agents modulating angiogenesis, which target this protein. More specifically, the present invention relates to the use of Dp71 or a variant thereof (or a coding nucleic acid) for stimulating angiogenesis in a subject, particularly a human subject. The invention relates to the use of an inhibitor of Dp71 for inhibiting angiogenesis in a subject.

Abstract: Disclosed are methods and compositions for early diagnosis, monitoring and treatment of retinal dystrophy, age-related macular degeneration, Bardet-Biedel syndrome, Bassen-kornzweig syndrome, best disease, choroidema, gyrate atrophy, congenital amourosis, refsun syndrome, stargardt disease and Usher syndrome. In particular, the invention relates to a protein, termed “Rdcvf1,” that is differentially transcribed and expressed in subjects suffering from retinal dystrophies and the like, such as retinal dystrophy and age-related macular degeneration compared with nonsufferers, antibodies which recognize this protein, and methods for diagnosing such conditions.

Abstract: Disclosed are methods and compositions for early diagnosis, monitoring and treatment of retinal dystrophy, age-related macular degeneration, Bardet-Biedel syndrome, Bassen-kornzweig syndrome, best disease, choroidema, gyrate atrophy, congenital amourosis, refsun syndrome, stargardt disease and Usher syndrome. In particular, the invention relates to a protein, termed “Rdcvf1,” that is differentially transcribed and expressed in subjects suffering from retinal dystrophies and the like, such as retinal dystrophy and age-related macular degeneration compared with nonsufferers, antibodies which recognize this protein, and methods for diagnosing such conditions.

Abstract: The present invention relates to a method of identifying a subject having or at risk of having or developing a retinitis pigmentosa, comprising detecting in a sample obtained from said subject, the presence of at least one mutation in the rhodopsin (RHO) gene selected from the group consisting of c.263T>C, c.620T>A and c. 1031A>C wherein the presence of said mutation indicates an increased risk of having or being at risk of having or developing the retinitis pigmentosa.

Abstract: Disclosed are methods and compositions for early diagnosis, monitoring and treatment of retinal dystrophy, age-related macular degeneration, Bardet-Biedel syndrome, Bassen-kornzweig syndrome, best disease, choroidema, gyrate atrophy, congenital amourosis, refsun syndrome, stargardt disease and Usher syndrome. In particular, the invention relates to a protein, termed “Rdcvf1,” that is differentially transcribed and expressed in subjects suffering from retinal dystrophies and the like, such as retinal dystrophy and age-related macular degeneration compared with nonsufferers, antibodies which recognize this protein, and methods for diagnosing such conditions.

Abstract: This invention relates to methods and compositions for detection and treatment of neurodegenerative diseases. In particular, the invention relates to polypeptides that can protect against neuron degeneration, nucleic acid molecules that encode such polypeptides, and antibodies that recognize said polypeptides.

Abstract: The present invention provides an organotypic system comprising a portion of a retina containing live cells in a cultured in medium, wherein photosensitivity has been restored in at least some of said live cells. Methods of use thereof are also provided.

Abstract: The present invention relates to a method of treating or preventing a convulsive disorder in a patient in need thereof comprising administering said patient with a therapeutically effective amount of an active ingredient that induces a high level of extracellular GABA or increases GABA receptor activation once per day in the evening or at night.