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In situ radioligand binding with autoradiography allows localization and quantification of bound radiolabeled ligands in tissues. This is a very sensitive technique that enables the characterization of binding kinetics and ligand specificity and the quantification of the am ...

Phospholipase D (PLD), which hydrolyzes phospholipids (primarily phos-phatidylcholine) to generate phosphatidic acid, is an essential component in cellular signal transduction (1,2). Phosphatidic acid and its dephosphorylated product 1,2 diacylglycerol, are importa ...

Changes in intracellular free calcium ion concentration (i) play a major role in vascular smooth muscle cell function (1,2). Elevation of i is an important regulator of multiple downstream signaling pathways and it is a major determinant of vascular smooth muscle contraction (1–3). Agonis ...

Angiotensin type-1 receptors (AT1 receptors) mediate various physiological actions of angiotensin (Ang II) via multiple-signal transduction pathways (1). In addition to the phospholipase C pathway and dihydropyridine-sensitive voltage-dependent calcium channels, A ...

Nitric oxide (NO) is a simple gaseous monoxide that is involved in a variety of biological mechanisms in the mammalian body (1). NO is produced by a group of enzymes, nitric oxide synthases. One of the more important functions of NO is to regulate the tone of blood vessels (2). As one of the most potent vasodilato ...

The development of specific receptor inhibitors of angiotensin II and the generation of genetic knockout models of the different components of the renin-angiotensin-aldosterone (RAAS) cascade has confirmed previous and has created new evidence for elementary functions of the R ...

The proximal tubules play a critical role in providing the kidneys with their ability to regulate body fluid volume and electrolyte composition. One major function is the reabsorption of sodium and other electrolytes which is caused, in part, by angiotensin II (Ang II), a peptide that is made up of ei ...

The study of regional blood flow in the kidney has been fraught with difficulties. Quantitation of perfusion of the inner cortex and medulla of the kidney has been especially challenging to measure because there is no way to directly visualize the large or small blood vessels in these areas. Furthe ...

Isolation and perfusion of single nephron segments was first described by Burg et al. (1) in 1966. This technique has allowed us to study both transepithelial and transmembrane transport in individual nephron segments, including the proximal tubule, under carefully controlled circu ...

Understanding the endocrine, paracrine, and autocrine mechanisms involved in the regulation of renal hemodynamics has proven an elusive quest (1). What has emerged, however, is a greater appreciation for the elegance and complexity of the renal microcirculation, which provides exq ...

The blood vessels that contribute most to precapillary resistance are known as resistance arteries, consisting of arterioles and small arteries with diameters of less than 500 μm (1). These vessels regulate the vascular resistance, and thus the blood supply, through the adjustment of the ...

Determination of the number of microvessels in tissue is of great importance in the assessment of studies of vascular development, angiogenesis, and rarefaction (1). Numerous techniques, such as immunohistochemical staining, fluorescence injection, and India ink or microfil pe ...

Angiotensin II (Ang II) is an endogenous peptide that has a wide spectrum of physiological functions, including the regulation of hemodynamics and blood volume (1); growth, hypertrophy, and remodeling in the cardiovascular system (1,2); regulation of microvessel density (3,4), and modu ...

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is an arrhythmogenic disease characterized by adrenergic-induced arrhythmias in the form of bidirectional and PVT. CPVT is a distinct clinical entity associated with a high mortality rate of up to 50% by the age of 30 yr. Rec ...

Tuberous sclerosis complex (TSC) is a neurocutaneous syndrome characterized by seizures, mental retardation, and benign tumors of many organs, including the brain, kidneys, skin, retina, and heart. TSC is caused by mutations in the TSC1 and TSC2 tumor suppressor genes. The genes follow the tw ...

Friedreich ataxia (FA) is an autosomal-recessive disease primarily characterized by progressive neurological disability. A significant proportion of patients also present with a hypertrophic cardiomyopathy, which may, in some cases, cause premature death. FA is caused by ins ...

Alagille syndrome is an autosomal-dominant disorder characterized by hepatic, cardiac, ocular, skeletal, and facial abnormalities. The disease gene, Jagged1 (JAG1), was identified by molecular analyses of chromosomal alterations involving chromosome 20p. Total gene delet ...

Heterotaxy refers to the abnormal arrangement of internal organs in relation to each other. It is characterized by complex cardiac malformations that are thought to result from abnormal left-right patterning in early embryonic development. Mutations in four genes have been identif ...

Microarray technology as a method for large-scale gene expression analysis has entered into widespread use in the field of cardiovascular research.This chapter summarizes the application of arrays to study gene expression profiles of congenital heart diseases,in particular t ...

This chapter describes the use of denaturing high-performance liquid chromatography as a high-throughput method to detect genetic mutations in pediatric cardiomyopathies. An overview of the classification, incidence, and etiologies of the major cardiomyopathies is provi ...