生物化学资料：4-fibrous proteins.ppt

UNIT I Protein Structure and Function第一单元 蛋白质的结构和功能 Chapter 4 Fibrous Proteins 第4章 纤维状蛋白 Ⅲ. ELASTIN Having rubber-like properties, good flexibilities. Elastic fibers composed of elastin & glycoprotein microfibrils. Often found in the lungs, the walls of large arteries & elastic ligaments. Ⅲ-A. Structure of elastin An insoluble protein polymer. tropoelastin is its precursor Tropoelastin components (700AAs, primarily small, non-polar, such as Gly,Ala,Pro,&Val, only a little Hyp, no Hyl) Tropoelastin interacts with specific glycoprotein in the extracellular space. Some Lys residues are oxidatively deaminated by lysyl oxidase, forming allysine.Three of the allysine R chains plus one unaltered lysyl R chain from the same or neighboring polypeptides form a desmosine cross-link. Ⅲ-B. Role of α1-antitrypsin in elastin degradation α1-AT (AAT, α1-antitrypsin ) is an inhibitor of proteinases(such as elastase). It has the important role in the lung. Emphysema resulting from α1-AT . Ⅳ. CHARPTER SUMMARY Collagen & elastin are the typical fibrous proteins. The primary characters of collagen in the molecule component & struture. Elastin is a CT protein with rubber-like properties in tissue such as the lung. α1-AT prevents elastin degradation in the alveolar walls. A deficiency of α1-AT can cause emphysema & cirrhosis of the liver. Thanks! 胶原蛋白(collagen)存在所有多细胞动物体内，是体内含量最多的一类蛋白质，存在于几乎所有组织中，是一种细胞外蛋白质，以不溶纤维形式存在，具高度抗张能力，是决定结缔组织韧性的主要因素。 单个的Ⅰ型胶原分子分子量约285kD，宽14A，长约3000A。由三条多肽链组成。哺乳动物个体中有30种不同的多肽链构成16种不同的胶原，其中最常见的是Ⅰ,Ⅱ,Ⅲ型. the regular staggered packing规律的(明暗)交错的区带 Mesh网孔 Lumen腔 在内质网中由半乳糖基转移酶及葡萄糖基转移酶催化将糖基联于5－羟赖氨酸残基上。糖基化的作用目前尚未完全阐明，研究发现这些糖基位于胶原纤维中原胶原的接头处。推测糖基化与纤维的定向排列有关。 procollagen原胶原 Troprocollagen(完整的)胶原蛋白原 皮肤弹性过度(cutis hyperelastica)又称Ehlers-Danlos综合征，为一种具有遗传倾向的胶原异常性疾病。 Ehlers-Danlos综合征诊断要点：1.本病可分为常染色体隐性、显性或性联遗传方式，家族中有同样病人。2.症状最早发生于幼儿，常常于学走路时开始被发现。3.皮肤外伤后可以形成较大的伤口，愈合过程缓慢，愈后留下萎缩性疤痕。伤口缝合后容易反复裂开。4.皮肤弹力过度，皮肤捏起后可拉得很长。皮肤柔软，摸起来有绒样感。5.关节伸展过度，当膝关节伸展过度，患者走路困难，易摔倒。6.组织病理示真皮内弹力纤维增加