Avtor/Urednik		Rozman, Damjana; Waterman, Michael R
Naslov		Sterol biosynthesis
Tip		članek
Vir		In: Mason JI, editor. Genetics of steroid biosynthesis and function. Modern genetics. Vol 6. London: Routledge,
Leto izdaje		2002
Obseg		str. 37-56
Jezik		eng
Abstrakt		Sterol biosyntheis is both an elegant and complex biosynthetic process leading to essential lipids in animals, fungi and yeast, plants and perhaps even in some bacteria. While this chapter focuset on synthesis of cholesterol in animals, the biosynthetic pathway is quite conserved in these different phyla. In addition to producing cholesterol, key intermediales in this pathway are utilized in other important biological processes including synthesis of other lipids, farnesylation of proteins and generation of signalling molecules. Regulation of the levels of enzymes in this pathway is controlled by a unique transcription factor, sterol regulatory element binding protein (SREBP), which begins its life as a protein in the endoplasmic reticulum. The transactiovations domain os SREBP is proteolytically cleared from the membrane only under conditions of low sterol concentration, where is enters the nodeus and activates transcription of cholesterologenic genes. Most of these genes in humans are now characterized and their association with genetic diseases and cholesterol biosynthesis is being revealed.
Deskriptorji		STEROLS CHOLESTEROL TRANSCRIPTION, GENETIC TRANSCRIPTION FACTORS HYDROXYMETHYLGLUTARYL-COA SYNTHASE HYDROXYMETHYLGLUTARYL COA REDUCTASES MEVALONIC ACID SQUALENE CYTOCHROME P-450 LANOSTEROL SMITH-LEMLI-OPITZ SYNDROME DESMOSTEROL