尿管結石を疑う腰部痛にて発症した線維筋性異形成による総腸骨動脈破裂の1例

We report a case of spontaneous rupture of the common iliac artery associated with fibromuscular dysplasia (FMD). A 21-year-old previously healthy male presented with acute onset of colic pain, suspected to be caused by a ureteral stone. Abdominal computed tomography and angiography revealed a retroperitoneal hematoma caused by rupture of the common iliac artery. In spite of an emergency operation initiated quickly, the patient died. A pathological examination demonstrated FMD of the common iliac artery. Although very rare, it is important to bear in mind that the possibility of retroperitoneal hemorrhage exists in patient with sudden lumbago.線維筋性異形成による総腸骨動脈破裂の1例を経験したので報告する。症例は21歳, 特に既往歴のない21歳の男性, 突然の腰部激痛で発症した。当初, 尿管結石による穿通発作が疑われた。腹部CTと血管造影にて総腸骨動脈の破裂による後腹膜血腫と診断された。緊急手術が行われたが, 残念ながら患者は死亡した。病理学的検索により総腸骨動脈の線維筋性異形成が原因と診断された。非常に稀な症例であるが, 突然の腰痛を主訴とする患者の中にこのような症例が含まれることを知っておくべきと考える。(著者抄録

LXXLL-Related Motifs in Dax-1 Have Target Specificity for the Orphan Nuclear Receptors Ad4BP/SF-1 and LRH-1

The orphan receptor Ad4BP/SF-1 (NR5A1) is a constitutive activator, and its activity is repressed by another orphan receptor, Dax-1 (NR0B1). In the present study, we investigated the molecular mechanisms underlying this repression by Dax-1. Yeast two-hybrid and transient-transfection assays confirmed the necessity of three LXXLL-related motifs in Dax-1 for interaction with and repression of Ad4BP/SF-1. In vitro pull-down experiments confirmed that Dax-1 interacts with Ad4BP/SF-1 and also with LRH-1 (NR5A2). The target specificity of the LXXLL-related motifs was indicated by the observations that Ad4BP/SF-1, ERα (NR3A1), LRH-1, ERR2 (NR3B2), and fly FTZ-F1 (NR5A3) interacted through their ligand binding domains with all the LXXLL-related motifs in Dax-1 whereas HNF4 (NR2A1) and RORα (NR1F1) did not. Transcriptional activities of the receptors whose DNA binding domains (DBDs) were replaced by the GAL4 DBD were repressed by Dax-1 to various levels, which correlated with the strength of interaction. Amino acid substitutions revealed that Ad4BP/SF-1 and LRH-1 preferentially interact with L(+1)XXLL-related motifs containing serine, tyrosine, serine, and threonine at positions −2, +2, +3, and +6, respectively. Taken together, our results indicate that the specificities of LXXLL-related motifs in Dax-1 based on their amino acid sequences play an important role in regulation of orphan receptors

Asymmetric expression of antivin/lefty1 in the early chick embryo

International audienceMammalian lefty and zebrafish antivin, highly related to lefty, are shown to be expressed asymmetrically and involved in the specification of the left body side of early embryos. We isolated a chick homologue of the antivin/lefty1 cDNA and studied its expression pattern during early chick development. We found that antivin/lefty1 is expressed asymmetrically on the left side of the prospective floorplate, notochord and lateral plate mesoderm of the chick embryo