Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029713 (immaturity)
 4,335 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The studies outlined in this review suggest that the immaturity of distal nephron segments may hinder urinary excretion of potassium early in life. Among the factors that may limit potassium secretion by principal cells in the neonatal cortical collecting duct are an unfavorable electrochemical gradient (reduced Ki, Na(+)-K(+)-ATPase activity and/or Vte), limited membrane permeability to potassium and sodium, low tubular fluid flow rate, reduced luminal sodium concentration, or increased paracellular backleak. Alternatively, enhanced potassium absorption by other relatively well-differentiated distal nephron segments may contribute in part to a reduced net potassium excretory rate in the newborn. It should be kept in mind, however, that the limited potassium secretory capacity of the immature kidney becomes clinically relevant only under conditions of potassium excess. Under normal circumstances, the tendency of the newborn to retain potassium is an appropriate and necessary condition for growth.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Maturation of renal potassium transport. 203 47

C57BL/6J mice homozygous for the cpk gene exhibit an autosomal recessive (AR) form of polycystic kidney disease (PKD), similar to human ARPKD, with massive collecting duct cysts. These cysts are lined by epithelial with an immature phenotype. Since renal expression of epidermal growth factor (EGF) is also significantly decreased in affected mice, we hypothesized that renal EGF is necessary for normal developmental maturation of the collecting duct. To determine if the lack of EGF may be a decisive factor in the initiation and/or growth of collecting duct cysts, we administered exogenous EGF (1 microgram/g body wt subcutaneously) daily for Postnatal Days 3-9 (a critical period for collecting duct maturation) to C57BL/6J-cpk mice. EGF but not sham or albumin treatment retarded the development of PKD, reduced the degree of renal failure associated with the disease, and prolonged the survival of cystic mice. Sulfated glycoprotein-2 gene expression, a marker of immaturity in collecting duct cells, was reduced in cystic kidney by EGF treatment. This finding indicates that EGF treatment was associated with an increase in the maturation of the collecting duct epithelial cells. These findings support the view that decreased EGF may play a significant role in promoting the enlargement of collecting duct cysts in a hereditary model of ARPKD and that PKD involves defective and/or arrested collecting duct cell maturation.
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PMID:Epidermal growth factor ameliorates autosomal recessive polycystic kidney disease in mice. 778 94

The newborn has a limited ability to regulate H+/HCO3- homeostasis, due in part to immaturity of the intercalated cells in the distal nephron. We traced the postnatal differentiation of the intercalated cells of the rabbit cortical collecting duct (CCD) and outer medullary collecting duct (OMCD) using MAb to the 31-kD subunit of the vacuolar H(+)-ATPase, membrane portion of erythrocyte band 3, and apical surface of B-intercalated cells (peanut agglutinin [PNA], MAb B63). In the most superficial CCD of the newborn there was no binding to these probes, although deeper in the cortex there was faint apical staining with PNA and MAb B63 and a few patterns of H(+)-ATPase and band 3 labeling of neonatal intercalated cells. The OMCD showed mostly apical H(+)-ATPase and both cytoplasmic and basolateral band 3 labeling but B-intercalated cell markers were not seen. By 3 wk of age the staining of the CCD and OMCD was more polarized, resembling those in the adult. Band 3 positive cells (as a percentage of total cells) in the CCD increased from 13 to 17% during maturation, and in the OMCD they increased from 22 to 37%. Some basolateral band 3 and apical H(+)-ATPase staining was also seen in the inner medullary collecting duct of 3-wk-old rabbits to a greater extent than in newborn or adult rabbits. Labeling of intercalated cells in the CCD and OMCD was weakest and least numerous in the newborn, greater in the 3 wk old, and greatest in the adult. Most maturing cortical intercalated cells bound both PNA and H(+)-ATPase MAb, comparable to what has been observed in the adult CCD. PNA-negative cells showing apical H(+)-ATPase labeling, consistent with the classic A-intercalated cell phenotype, comprised only 5% of identified intercalated cells in the newborn CCD compared with 12% in older animals. In or near the developing renal vesicles and ampullary structures were occasional cytoplasmically staining PNA- and B63-positive cells. Whether these cells are precursors of specific renal tubular cells cannot yet be established. Staining for principal cells (ST.9) was less intense in the neonatal cortex than in more mature cortex, but the deep cortex and outer medulla were heavily labeled at all ages. These data indicate that immature intercalated cells, in the CCD and OMCD, may undergo significant postnatal proliferation and differentiation, acquiring mature phenotypes during the first month of life. The A-intercalated cell appears more differentiated than the B cell during the 1st wk of life, suggesting that A-intercalated cells contribute more than B cells to the maintenance of acid-base homeostasis in the newborn.
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PMID:Postnatal differentiation of rabbit collecting duct intercalated cells. 882 79

A significant increase in the water permeability was found in the rat outer medullary collecting duct (OMCD) cells in presence of 10-7M of vasopressin. The latter caused a decrease in the OMCD cell volume in isoosmotic medium in adult rats. In pups, the water permeability of the OMCD cells was very high. Vasopressin seems to be unable to decrease the cell volume of the OMCD cells in pups which suggests an immaturity of the cell transduction mechanism.
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PMID:[Morphometric analysis of the effects of vasopressin in the rat kidney collecting tubules]. 1038 87

The limited renal concentration performance by the immature kidney traditionally is thought to be attributed to blunted renal response to arginine vasopressin (AVP) and medullary hypotonicity. The diminished AVP-dependent osmotic water permeability of the collecting duct is the result of decreased AVP binding and adenylate cyclase activation, and low expression of aquaporin-2 (AQP2) mRNA and low levels of AQP2 protein. Moreover, the immature kidney fails to establish deep cortico-papillary osmotic gradient because of structural immaturity, limited solute transport and increased medullary blood flow. Based on indirect clinical and experimental evidences this article puts forward a hypothesis that during perinatal period the abundant hyaluronan (HA) content in the renomedullary interstitium has a primary role in antagonizing water reabsorption and limiting concentration performance. Hydration-related alterations in renal HA appears to be mediated by antidiuretic hormone. The concept of HA-mediated renal water transport may imply that interfering selectively with renal HA metabolism may provide a new therapeutic approach to promote diuresis or antidiuresis, respectively, according to the elevation or reduction in renomedullary HA.
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PMID:Hyaluronan-related limited concentration by the immature kidney. 1614 Apr 63

The kidneys are essential organs that filter the blood, removing urinary waste while maintaining fluid and electrolyte homeostasis. Current conventional research models such as static cell cultures and animal models are insufficient to grasp the complex human in vivo situation or lack translational value. To accelerate kidney research, novel research tools are required. Recent developments have allowed the directed differentiation of induced pluripotent stem cells to generate kidney organoids. Kidney organoids resemble the human kidney in vitro and can be applied in regenerative medicine and as developmental, toxicity, and disease models. Although current studies have shown great promise, challenges remain including the immaturity, limited reproducibility, and lack of perfusable vascular and collecting duct systems. This review gives an overview of our current understanding of nephrogenesis that enabled the generation of kidney organoids. Next, the potential applications of kidney organoids are discussed followed by future perspectives. This review proposes that advancement in kidney organoid research will be facilitated through our increasing knowledge on nephrogenesis and combining promising techniques such as organ-on-a-chip models.
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PMID:3D kidney organoids for bench-to-bedside translation. 3303 8