A transgenic mouse model reproduces human hereditary systemic amyloidosis.

Amyloidoses are rare life-threatening diseases caused by protein misfolding of normally soluble proteins. The fatal outcome is predominantly due to renal failure and/or cardiac dysfunction. Because amyloid fibrils formed by all amyloidogenic proteins share structural similarity, amyloidoses may be studied in transgenic models expressing any amyloidogenic protein.

Overlapping expression patterns of the multiligand endocytic receptors cubilin and megalin in the CNS, sensory organs and developing epithelia of the rodent embryo.

Cubilin and megalin are multiligand epithelial endocytic receptors well characterized in the adult kidney and ileum where they form a complex essential for protein, lipid and vitamin uptake. Although inactivation of the megalin gene leads to holoprosencephaly and administration of anti-cubilin antibodies induces fetal resorptions or cranio-facial malformations their function in the developing embryo remains unclear. We recently showed that both proteins are strongly expressed by the maternal-fetal interfaces and the neuroepithelium of the early rodent embryo where they co-localize and form a complex important for nutrient uptake.

Pathologic features of necrotizing adenoviral prostatitis in an AIDS patient.

Adenovirus has been implicated as a cause of opportunistic infections in immunocompromised patients, frequently with multiorgan involvement and a fatal outcome. We describe a case of necrotizing adenoviral prostatitis in a 35-year-old man with terminal AIDS and generalized adenoviral infection. The histopathologic findings of intraacinar necrotizing inflammation, prominent viral nuclear inclusions in residual epithelium, and mild T-lymphocyte and macrophageal inflammatory reaction were observed in the prostate.

Expression and role of cubilin in the internalization of nutrients during the peri-implantation development of the rodent embryo.

Histiotrophic nutrition is essential during the peri-implantation development in rodents, but little is known about receptors involved in protein and lipid endocytosis derived from the endometrium and the uterine glands. Previous studies suggested that cubilin, a multiligand receptor for vitamin, iron, and protein uptake in the adult, might be important in this process, but the onset of its expression and function is not known. In this study, we analyzed the expression of cubilin in the pre- and early post-implantation rodent embryo and tested its potential function in protein and cholesterol uptake.

Fiber-type specific and position-dependent expression of a transgene in limb muscles.

We have previously shown that the proximal sequences of the human aldolase A fast-muscle-specific promoter (pM) are sufficient to target the expression of a linked CAT reporter gene to all fast, glycolytic trunk and limb muscles of transgenic mice (pM310CAT lines) in a manner mimicking the activity of the endogenous mouse promoter. When a NF1-binding site (motif M2) in this proximal regulatory region is mutated, the activity of the corresponding mM2 transgene is strongly affected but only in a some fast muscles. Here we show that the mutation of the M2 motif has only mild effects on pM activity in axial and proximal limb, while it drastically reduces this activity in both fore and hind limb distal muscles.