LECTURE-16_Carbohydrates and Human Disease

LECTURE-16_Carbohydrates and Human Disease - Lecture #16...

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Lecture #16 Carbohydrates and human disease*. Two Examples (1) I-Cell Disease (2) Diabetes  *This material is not in Campbell and Reece.
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Lecture Goal The goal of this lecture is to demonstrate how a  detailed knowledge of biochemistry and cell biology  leads to an understanding of the pathophysiology of  human disease.
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Lecture #14-Objectives Describe the function of Glycosyltransferases Name the amino acids which are covalently linked to  oligosaccharides. Contrast N-linked glycosylation with O-linked  glycosylation with respect to cellular localization. Describe the etiology (cause) of I-Cell Disease.
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Lecture #14-Objectives Name and contrast the two types of diabetes. Describe the function of insulin. Define non-enzymatic glycation and what are the  physiological consequences of uncontrolled  glycation.  Describe the function of glucagon. Describe the Cori cycle.
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Introduction There are many diseases associated with the  cell biology and metabolism of carbohydrates. I-Cell disease  is genetic and rare. Diabetes  can be genetic but more often it  is “epigenetic” and certainly not rare.
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I - Cell Disease I - Cell Disease is interesting because: It shows how carbohydrates are important  in the regulation of cellular processes. It focuses on protein sorting and trafficking  and the role of the golgi complex and the  lysosome in biological processes.
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Glycoproteins Carbohydrates can be covalently linked to  proteins to form glycoproteins. Carbohydrates can be linked through the  amide nitrogen of asparagine (N- linkage ), or  through the oxygen of serine or threonine (O- linkage ).
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Elastase Typical Glycoprotein. Oligosaccharide
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How Are Glycoproteins Synthesized? First, the oligosaccharide is synthesized by  specific enzymes. Second, the oligosaccharide is transferred  and covalently linked to specific amino acids  of protein destined to be a glycoprotein.
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Oligosaccharide Assembly Specific  glycosyltransferases  are responsible  for oligosaccharide assembly and the formation  of glycosidic links. Prior to assembly the sugars are activated via a  sugar nucleotide.  UTP is the nucleotide triphosphate typically (but 
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This note was uploaded on 03/31/2009 for the course BIO 202 taught by Professor Dean during the Spring '08 term at SUNY Stony Brook.

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LECTURE-16_Carbohydrates and Human Disease - Lecture #16...

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