By I.A. Hughes, A.J.L. Clark, P.-E. Mullis
Adrenal sickness in young children poses an immensely tricky diagnostic and administration challenge for the paediatrician in view of the massive variety of infrequent pathologies that could found in this fashion. This quantity brings jointly the services of the stated leaders in particular adrenal problems to supply a without problems obtainable textual content that mixes a close description of the molecular origins of those illnesses, as they're at the moment understood, with a different account of the scientific positive factors and healing innovations. starting with issues that essentially impact adrenal improvement and progressing in the direction of the defects that intrude with steroid creation, the reader profits enormous perception into the conventional body structure of the adrenal cortex fairly as published through medical problems. the key contributions of contemporary biochemistry and molecular genetics in our present knowing are seen all through, supplying a special evaluation of adrenocortical biology.
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Additional resources for Adrenal disease in childhood: clinical and molecular aspects (Endocrine Development Vol. 2)
Several candidates have emerged from these efforts. This review Cholesterol Transport Proteins 39 will attempt to summarize the studies which have been performed on these candidates. Sterol Carrier Protein 2 One of the proteins suggested to be involved in the acutely regulated intracellular transfer of cholesterol is the sterol carrier protein 2 (SCP2). SCP2 is a 13-kD protein that has been found in high abundance in liver as well as in various steroidogenic tissues. Also known as nonspecific lipid transfer protein, the role of this protein in liver and adrenal cells has previously been reviewed by Vahouny et al.
Uk Swords/Franklin/Huebner/Elias/Clark 36 Hughes IA, Clark AJL (eds): Adrenal Disease in Childhood. Clinical and Molecular Aspects. Endocr Dev. Basel, Karger, 2000, vol 2, pp 37–62 ............................ Proteins Involved in Mitochondrial Cholesterol Transport Douglas M. Stocco a, Adam J. Reinhart a, Walter L. , USA The intracellular movement of cholesterol is carefully regulated in most cells, although many of the mechanisms involved in this regulation are not well understood. The regulated movement of cholesterol occurs amongst the specialized organelles in the cell such as the plasma membrane, Golgi, lysomes, peroxisomes and endoplasmic reticulum.
Liakos P, Chambaz EM, Feige JJ, Defaye G: Expression of ACTH receptors (MC2-R and MC5-R) in the glomerulosa and fasciculata reticularis zones of bovine adrenal cortex. Endocr Res 1998;24: 427–432. Heikkila P, Arola J, Liu J, Kahri AI: ACTH regulates LDL receptor and CLA-1 mRNA in the rat adrenal cortex. Endocr Res 1998;24:591–593. Shepard TH, Landing BH, Mason DG: Familial Addison’s disease. Am J Dis Child 1959;97: 154–162. Migeon CJ, Kenny FM, Kowarski A, Snipes CA, Spaulding JS, Finkelstein JW, Blizzard RM: The syndrome of congenital adrenocortical unresponsiveness to ACTH: Report of six cases.