This invention relates to a pharmaceutical composition for treatment of a bone disease comprising, as an active ingredient, a protein comprising an extracellular cysteine-rich domain, which is from the Frizzled receptor selected from the group consisting of mammalian animal-derived Frizzled 1, Frizzled 2, and Frizzled 7 and has activity of increasing bone mass, bone density, and/or bone strength, or a mutant of such domain having sequence identity of 85% or higher to the amino acid sequence of the domain and having activity of increasing bone mass, bone density, and/or bone strength, or a vector comprising a nucleic acid encoding the protein.

The present invention provides a prophylactic or therapeutic agent for a kidney disease, comprising AIM or a partial peptide thereof, or a nucleic acid comprising a base sequence encoding the same, or a screening method for a prophylactic or therapeutic agent for a kidney disease, comprising using an animal obtained by subjecting a non-human mammal deficient in AIM expression to unilateral ureteral obstruction or transient kidney ischemia/reperfusion and the like.

The present invention provides non-human animal models of neuronal injury and/or cognitive dysfunction and methods of making and using such animal models. The animal models of the invention are particularly suited to assessing neurodegeneration in selected regions of interest in the CNS, and thus especially useful for testing the therapeutic efficacy of agents targeting neurodegeneration associated with aging, neurodegenerative diseases, autoimmunity and trauma (e.g., ischemia).

The present invention provides an adeno-associated virus (AAV) vector gene therapy for use in treating a monogenic form of nephrotic syndrome, wherein the AAV vector comprises a NS-associated transgene and minimal nephrin promoter NPHS1 or podocin promoter NPHS2.

An object of the present invention is to provide a nucleic acid molecule or a viral virion containing the nucleic acid molecule, the nucleic acid molecule having integrated therein a gene encoding a fusion protein of an anti-transferrin receptor antibody and a physiologically active protein, the fusion protein having an adjusted binding activity to TfR, and being capable of reducing a side reaction such as an anemia symptom caused by binding of the anti-TfR antibody to the transferrin receptor, for example, when the gene encoding the fusion protein of the anti-TfR antibody and the physiologically active protein is used in gene therapy.

A transgenic mouse has a genome that includes the entire gene region of human transforming growth factor beta-1 (human TGF1) located downstream of a mouse Podocin promoter such that expression of the human TGF1 is controlled by the mouse Podocin promoter. The human TGF1 contains 7 exons and 6 introns, the human TGF1 is expressed in a kidney of the mouse as non-active TGF1 and becomes active TGF1 extracellularly, and the transgenic mouse spontaneously develops renal fibrosis.

An apparatus for unilateral spinal cord compression includes a fixed member and a movable member that moves longitudinally along the fixed member by using a linear actuating mechanism to compress a portion of spinal cord encompassed by the movable member and the fixed member.

The invention relates to compositions for use in animal models of neurodegenerative disease and methods therefor. More particularly, the invention relates to the use of neurotoxic sterol glycosides or neurotoxic glycolipids, or combinations thereof, in animal models of neurodegenerative disease. Neurotoxicity-modulating chromenols can also be used in these animal models in combination with the neurotoxic sterol glycosides or neurotoxic glycolipids, or combinations thereof.

The present invention provides a non-human animal IKK which shows fibrosis of tissue, since it lacks IKK gene in a myofibroblast- and/or smooth muscle cell-specific manner. Since the non-human animal shows pathology highly similar to scleroderma, it is extremely useful as an animal model of scleroderma.

Disclosed is a method of treating, reducing, or preventing pruritis in a mammal, the method comprising administering at least one natriuretic polypeptide b (Nppb) blocking agent to a mammal in an amount effective to treat or prevent pruritis in the mammal. An in vitro method of identifying a compound that inhibits Nppb activity is also disclosed.