Abstract: The purpose of the present invention is to provide fuel cell system capable of stable start-up and method for starting the fuel cell system. Fuel cell system includes SOFC, a turbocharger, oxidizing gas supply line, a control valve, oxidizing gas blow line, start-up air line for supplying the start-up air to the oxidizing gas supply line with a blower, and a control unit that, in state in which the control valve is closed and the blow valve is opened to supply the start-up air to the oxidizing gas supply line with the blower when the turbocharger is started, decreases the opening of the blow valve and, after the timing at which the opening of the blow valve starts to decrease, increases the opening of the control valve and then stops the supply of the starting air.

Abstract: by screening using a high-throughput evaluation system relating to the promotion of the extracellular secretion of trimers of the causative proteins COL4A3/A4/A5, which decreased in the renal tissue of patients with Alport syndrome (AS), it was found that cyclosporin A has an effect of promoting extracellular secretion of trimers of type-IV collagen. From further studies, it was found that Alisporivir and NIM258, which do not inhibit calcineurin, also have an action to promote extracellular secretion of type-IV collagen. Furthermore, the present inventors have found that these actions are based on the cyclophilin D inhibitory mechanism, and have found a radical therapeutic agent for AS by inhibition of cyclophilin D. The present invention provides a composition and an AS therapeutic/prophylactic drug, which contain a cyclophilin D inhibitor as an active ingredient, for promoting the secretion of collagen trimers in cells having a mutated type-IV collagen gene.

Abstract: A method for producing a model animal which has a desired lifetime, and in which a predetermined biological reaction can be induced, and a model animal are provided. The present invention produces a first individual in which a gene of interest is heterozygously deficient using a first ES cell from a non-human mammalian animal. Meanwhile, a fragment containing a homologous gene that has homology to the gene of interest is made, a second ES cell constituted so that a predetermined region on X chromosome of the animal can be substituted is used, and the fragment is introduced into the second ES cell to generate a substituted ES cell in which the predetermined region has been substituted with the fragment. A second individual is produced using the substituted ES cell. The first individual and the second individual are mated with each other to produce a model animal.

Abstract: A method of producing a chimera animal, characterized by transplanting, into a host animal, an aggregate of a first pluripotent stem cell that is of interest to strain establishment, a second pluripotent stem cell in which the ability to form germ cells is absent or decreased, and embryo.

Abstract: The present invention provides embryonic stem cells obtainable from an embryo of an immunodeficient mouse which is deficient in both Rag2 and Jak3 genes by culture in the presence of a GSK3 inhibitor and an MEK inhibitor, as well as a transgenic mouse, which is created with the use of these embryonic stem cells.

Abstract: A method for producing a model animal which has a desired lifetime, and in which a predetermined biological reaction can be induced, and a model animal are provided. The present invention produces a first individual in which a gene of interest is heterozygously deficient using a first ES cell from a non-human mammalian animal. Meanwhile, a fragment containing a homologous gene that has homology to the gene of interest is made, a second ES cell constituted so that a predetermined region on X chromosome of the animal can be substituted is used, and the fragment is introduced into the second ES cell to generate a substituted ES cell in which the predetermined region has been substituted with the fragment. A second individual is produced using the substituted ES cell. The first individual and the second individual are mated with each other to produce a model animal.

Abstract: A trap vector containing a loxP sequence composed of inverted repeat sequence 1, a spacer sequence and inverted repeat sequence 2 in this order, the loxP sequence being a mutant loxP wherein a part of the inverted repeat sequence 1 or 2 is mutated.

Abstract: A method of producing a chimera animal, characterized by transplanting, into a host animal, an aggregate of a first pluripotent stem cell that is of interest to strain establishment, a second pluripotent stem cell in which the ability to form germ cells is absent or decreased, and embryo.

Abstract: Disclosed is a method for introducing a mutation into a gene, which comprises the following steps: a homologous recombination step of carrying out the homologous recombination between a target gene into which the mutation is to be introduced and a target recombinant vector, thereby substituting an exon in the target gene into which the mutation is to be introduced by a target DNA sequence in the target recombinant vector; and a mutation introduction step of carrying out the specific recombination between the target DNA sequence in the resulting target recombinant gene and a mutation introduction cassette of a mutation introduction vector carrying a mutated DNA sequence containing a mutant exon by the intervening action of Cre recombinase to substitute the target DNA sequence by the mutated DNA in the mutation introduction cassette, thereby producing a mutation-introduced gene into which the mutant DNA sequence has been introduced.

Abstract: A trap vector containing a loxP sequence composed of inverted repeat sequence 1, a spacer sequence and inverted repeat sequence 2 in this order, the loxP sequence being a mutant loxP wherein a part of the inverted repeat sequence 1 or 2 is mutated.

Abstract: A trap vector containing a loxP sequence composed of inverted repeat sequence 1, a spacer sequence and inverted repeat sequence 2 in this order, the loxP sequence being a mutant loxP wherein a part of the inverted repeat sequence 1 or 2 is mutated.

Abstract: A trap vector containing a loxP sequence composed of inverted repeat sequence 1, a spacer sequence and inverted repeat sequence 2 in this order, the loxP sequence being a mutant loxP wherein a part of the inverted repeat sequence 1 or 2 is mutated.