【摘 要】
：
Glycosylation is but one of numerous ubiquitous post translational modification (PTM) of proteins and lipids.PTM plays a critical role in protein folding, stability and interactions.The transfer of in
【机 构】
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Department of Metabolic Medicine, The Royal Children's Hospital Australia
【出 处】
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BIT`s 2nd Annual World Congress of Endobolism-2012(2012第二届内分
论文部分内容阅读
Glycosylation is but one of numerous ubiquitous post translational modification (PTM) of proteins and lipids.PTM plays a critical role in protein folding, stability and interactions.The transfer of initial sugar(s) to glycoproteins or glycolipids occurs in the endoplasmic reticulum (ER) or on the ER membrane.The subsequent addition of the many different sugars that make up a mature glycan is accomplished in the Golgi.Primary genetic defects in Glycosylation ie the Congenital Disorders of Glycosylation (CDG) have multi-system clinical manifestations with the central nervous system carrying the highest disease burden.Endocrine involvement has been observed in the form of hyperinsulinism,growth hormone deficiency, hypergonadotrophic hypogonadism, and skeletal involvement.Improved understanding of the molecular mechanisms associated with endocrine manifestations of diseases with primary or secondarily disturbed PTM has potential to lead towards improved treatments for end organ clinical disease.
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