Section of Pediatric Nephrology, Department of Pediatrics, Hypertension and Renal Center of Excellence, Tulane University School of Medicine, New Orleans, LA, USA.
Organogenesis. 2012 Jan-Mar;8(1):10-7. doi: 10.4161/org.19308. Epub 2012 Jan 1.
The mature renal medulla, the inner part of the kidney, consists of the medullary collecting ducts, loops of Henle, vasa recta and the interstitium. The unique spatial arrangement of these components is essential for the regulation of urine concentration and other specialized kidney functions. Thus, the proper and timely assembly of medulla constituents is a crucial morphogenetic event leading to the formation of a functioning metanephric kidney. Mechanisms that direct renal medulla formation are poorly understood. This review describes the current understanding of the key molecular and cellular mechanisms underlying morphological aspects of medulla formation. Given that hypoplasia of the renal medulla is a common manifestation of congenital obstructive nephropathy and other types of congenital anomalies of the kidney and urinary tract (CAKUT), better understanding of how disruptions in medulla formation are linked to CAKUT will enable improved diagnosis, treatment and prevention of CAKUT and their associated morbidity.
成熟的肾脏髓质,即肾脏的内部,由髓质集合管、亨利氏袢、直小血管和间质组成。这些成分的独特空间排列对于调节尿液浓缩和其他肾脏的特殊功能至关重要。因此,髓质成分的适当和及时组装是导致形成有功能的后肾的关键形态发生事件。指导肾脏髓质形成的机制还了解甚少。本综述描述了目前对肾脏髓质形成的形态学方面的关键分子和细胞机制的理解。鉴于肾脏髓质发育不良是先天性梗阻性肾病和其他类型的先天性肾和尿路异常(CAKUT)的常见表现,更好地了解髓质形成的中断如何与 CAKUT 相关联,将能够改善 CAKUT 及其相关发病率的诊断、治疗和预防。