Lung Connective Tissue

Chronic Obstructive Pulmonary Disease

Volumn , Issue , 2008, Pages 467-480

  Dunsmore, Sarah E ^a   Laurent, Geoffrey J ^b  

^a BRIGHAM AND WOMEN S HOSPITAL   (United States)

^b ROYAL FREE AND UNIVERSITY COLLEGE MEDICAL SCHOOL   (United Kingdom)

Author keywords

Cartilage; Collagens; Helix propagation; Matrix proteins; Pulmonary interstitium

Indexed keywords

EID: 84889626166     PISSN: None     EISSN: None     Source Type: Book    
DOI: 10.1002/9780470755976.ch40     Document Type: Chapter

References (226)

1. Roberts CR, Rains JK, Pare PD et al. Ultrastructure and tensile properties of human tracheal cartilage. J Biomech 1998;31:81-6.
2. Matsuda M, Fung YC, Sobin SS. Collagen and elastin fibers in human pulmonary alveolar mouths and ducts. J Appl Physiol 1987;63:1185-94.
3. Sobin SS, Fung YC, Tremer HM. Collagen and elastin fibers in human pulmonary alveolar walls. J Appl Physiol 1988;64:1659-75.
4. Mercer RR, Crapo JD. Spatial distribution of collagen and elastin fibers in the lungs. J Appl Physiol 1990;69:756-65.
5. Weibel ER, Bachofen H. The fiber scaffold of lung parenchyma. In: Crystal RG, West JB, Barnes PJ, Cherniack NA, Weibel ER, eds. The Lung: Scientific Foundations. New York: Raven Press, 1991:787-94.
6. Laurent GJ. Lung collagen: more than scaffolding. Thorax 1986;41:418-28.
7. Mandl I, Darnule TV, Fierer JA, Keller S, Turino GM. Elastin degradation in human and experimental emphysema. Adv Exp Med Biol 1977;79:221-31.
8. Barnes PJ. Mediators of chronic obstructive pulmonary disease. Pharmacol Rev 2004;56:515-48.
9. Christie RV. The elastic properties of the emphysematous lung and their clinical significance. J Clin Invest 1934;13:295-321.
10. Stead WW, Fry DL, Ebert RV. The elastic properties of the lung in normal men and in patients with chronic emphysema. J Lab Clin Med 1952;40:674-81.
11. Mead J, Lindgren I, Gaensler EA. The mechanical properties of the lungs in emphysema. J Clin Invest 1955;34:1005-16.
12. Cherniack RM. The physical properties of the lung in chronic obstructive pulmonary emphysema. J Clin Invest 1956;35:394-404.
13. Rahman I. The role of oxidative stress in the pathogenesis of COPD: implications for therapy. Treat Respir Med 2005;4:175-200.
14. Rahman I. Oxidative stress in pathogenesis of chronic obstructive pulmonary disease: cellular and molecular mechanisms. Cell Biochem Biophys 2005;43:167-88.
15. Barnes PJ. Chronic obstructive pulmonary disease. N Engl J Med 2000;343:269-80.
16. Hogg JC. Role of latent viral infections in chronic obstructive pulmonary disease and asthma. Am J Respir Crit Care Med 2001;164:S71-5.
17. Anderson GP, Bozinovski S. Acquired somatic mutations in the molecular pathogenesis of COPD. Trends Pharmacol Sci 2003;24:71-6.
18. Barnes PJ. New concepts in chronic obstructive pulmonary disease. Annu Rev Med 2003;54:113-29.
19. Barnes PJ, Shapiro SD, Pauwels RA. Chronic obstructive pulmonary disease: molecular and cellular mechanisms. Eur Respir J 2003;22:672-8.
20. van der Rest M, Garrone R. Collagen family of proteins. FASEB J 1991;5:2814-23.
21. Exposito JY, Cluzel C, Garrone R, Lethias C. Evolution of collagens. Anat Rec 2002;268:302-16.
22. Yamada Y, Avvedimento VE, Mudryj M et al. The collagen gene: evidence for its evolutinary assembly by amplification of a DNA segment containing an exon of 54 bp. Cell 1980;22:887-92.
23. Chu M-L, Prockop DJ. Gene structure. In: Royce PM, Steinmann B, eds. Connective Tissue and Its Heritable Disorders, 2nd edn. New York: Wiley-Liss, 2002:223-48.
24. Pace JM, Corrado M, Missero C, Byers PH. Identification, characterization and expression analysis of a new fibrillar collagen gene, COL27A1. Matrix Biol 2003;22:3-14.
25. Koch M, Schulze J, Hansen U et al. A novel marker of tissue junctions, collagen XXII. J Biol Chem 2004;279:22 514-21.
26. Chou MY, Li HC. Genomic organization and characterization of the human type XXI collagen (COL21A1) gene. Genomics 2002;79:395-401.
27. Veit G, Kobbe B, Keene DR et al. Collagen XXVIII, a novel VWA-domain-containing protein with many imperfections in the collagenous domain. J Biol Chem 2006;DOI:10.1074/jbc.M509333200.
28. Poschl E, Pollner R, Kuhn K. The genes for the alpha 1(IV) and alpha 2(IV) chains of human basement membrane collagen type IV are arranged head-to-head and separated by a bidirectional promoter of unique structure. EMBO J 1988;7:2687-95.
29. Momota R, Sugimoto M, Oohashi T et al. Two genes, COL4A3 and COL4A4 coding for the human alpha3(IV) and alpha4(IV) collagen chains are arranged head-to-head on chromosome 2q36. FEBS Lett 1998;424:11-6.
30. Sugimoto M, Oohashi T, Ninomiya Y. The genes COL4A5 and COL4A6, coding for basement membrane collagen chains alpha 5(IV) and alpha 6(IV), are located head-tohead in close proximity on human chromosome Xq22 and COL4A6 is transcribed from two alternative promoters. Proc Natl Acad Sci U S A 1994;91:11 679-83.
31. Cutting GR, McGinniss MJ, Kasch LM, Tsipouras P, Antonarakis SE. Physical mapping by PFGE localizes the COL3A1 and COL5A2 genes to a 35-kb region on human chromosome 2. Genomics 1990;8:407-10.
32. Gerecke DR, Olson PF, Koch M et al. Complete primary structure of two splice variants of collagen XII, and assignment of alpha 1(XII) collagen (COL12A1), alpha 1(IX) collagen (COL9A1), and alpha 1(XIX) collagen (COL19A1) to human chromosome 6q12-q13. Genomics 1997;41:236-42.
33. Oh SP, Warman ML, Seldin MF et al. Cloning of cDNA and genomic DNA encoding human type XVIII collagen and localization of the alpha 1(XVIII) collagen gene to mouse chromosome 10 and human chromosome 21. Genomics 1994;19:494-9.
34. Ghosh AK. Factors involved in the regulation of type I collagen gene expression: implication in fibrosis. Exp Biol Med (Maywood) 2002;227:301-14.
35. Rossi P, Karsenty G, Roberts AB et al. A nuclear factor 1 binding site mediates the transcriptional activation of a type I collagen promoter by transforming growth factor-beta. Cell 1988;52:405-14.
36. Ramirez F, Di Liberto M. Complex and diversified regulatory programs control the expression of vertebrate collagen genes. FASEB J 1990;4:1616-23.
37. Bruggeman LA, Burbelo PD, Yamada Y, Klotman PE. A novel sequence in the type IV collagen promoter binds nuclear proteins from Engelbreth-Holm-Swarm tumor. Oncogene 1992;7:1497-502.
38. Heikkila P, Soininen R, Tryggvason K. Directional regulatory activity of cis-acting elements in the bidirectional alpha 1(IV) and alpha 2(IV) collagen gene promoter. J Biol Chem 1993;268:24 677-82.
39. Inagaki Y, Truter S, Ramirez F. Transforming growth factorbeta stimulates alpha 2(I) collagen gene expression through a cis-acting element that contains an Sp1-binding site. J Biol Chem 1994;269:14 828-34.
40. Karsenty G, Park RW. Regulation of type I collagen genes expression. Int Rev Immunol 1995;12:177-85.
41. Willimann TE, Maier R, Trueb B. A novel transcription factor and two members of the Sp 1 multigene family regulate the activity of the alpha 2 (VI) collagen promoter. Matrix Biol 1995;14:653-63.
42. Chung KY, Agarwal A, Uitto J, Mauviel A. An AP-1 binding sequence is essential for regulation of the human alpha2(I) collagen (COL1A2) promoter activity by transforming growth factor-beta. J Biol Chem 1996;271:3272-8.
43. Mori K, Hatamochi A, Ueki H, Olsen A, Jimenez SA. The transcription of human alpha 1(I) procollagen gene (COL1A1) is suppressed by tumour necrosis factor-alpha through proximal short promoter elements: evidence for suppression mechanisms mediated by two nuclear-factor binding sites. Biochem J 1996;319:811-6.
44. Higashi K, Kouba DJ, Song YJ, Uitto J, Mauviel A. A proximal element within the human alpha 2(I) collagen (COL1A2) promoter, distinct from the tumor necrosis factor-alpha response element, mediates transcriptional repression by interferon-gamma. Matrix Biol 1998;16:447-56.
45. Long F, Sonenshein GE, Linsenmayer TF. Multiple transcriptional elements in the avian type X collagen gene: identification of Sp1 family proteins as regulators for high level expression in hypertrophic chondrocytes. J Biol Chem 1998;273:6542-9.
46. Vindevoghel L, Lechleider RJ, Kon A et al. SMAD3/4-dependent transcriptional activation of the human type VII collagen gene (COL7A1) promoter by transforming growth factor beta. Proc Natl Acad Sci U S A 1998;95:14 769-74.
47. Ritzenthaler JD, Goldstein RH, Fine A et al. Transforminggrowth-factor-beta activation elements in the distal promoter regions of the rat alpha 1 type I collagen gene. Biochem J 1999;280:157-62.
48. Chen SJ, Yuan W, Lo S, Trojanowska M, Varga J. Interaction of smad3 with a proximal smad-binding element of the human alpha2(I) procollagen gene promoter required for transcriptional activation by TGF-beta. J Cell Physiol 2000;183:381-92.
49. Hernandez I, de la Torre P, Rey-Campos J et al. Collagen alpha1(I) gene contains an element responsive to tumor necrosis factor-alpha located in the 5′ untranslated region of its first exon. DNA Cell Biol 2000;19:341-52.
50. Norman JT, Lindahl GE, Shakib K et al. The Y-box binding protein YB-1 suppresses collagen alpha 1(I) gene transcription via an evolutionarily conserved regulatory element in the proximal promoter. J Biol Chem 2001;276:29 880-90.
51. Chambers D, Young DA, Howard C et al. An enhancer complex confers both high-level and cell-specific expression of the human type X collagen gene. FEBS Lett 2002;531:505-8.
52. Bridgewater LC, Walker MD, Miller GC et al. Adjacent DNA sequences modulate Sox9 transcriptional activation at paired Sox sites in three chondrocyte-specific enhancer elements. Nucleic Acids Res 2003;31:1541-53.
53. Sakata-Takatani K, Matsuo N, Sumiyoshi H, Tsuda T, Yoshioka H. Identification of a functional CBF-binding CCAAT-like motif in the core promoter of the mouse proalpha 1(V) collagen gene (Col5a1). Matrix Biol 2004;23:87-99.
54. Sakai LY, Keene DR, Morris NP, Burgeson RE. Type VII collagen is a major structural component of anchoring fibrils. J Cell Biol 1986;103:1577-86.
55. Kapoor R, Sakai LY, Funk S et al. Type VIII collagen has a restricted distribution in specialized extracellular matrices. J Cell Biol 1988;107:721-30.
56. Sage H, Iruela-Arispe ML. Type VIII collagen in murine development: association with capillary formation in vitro. Ann N Y Acad Sci 1990;580:17-31.
57. Ng LJ, Tam PP, Cheah KS. Preferential expression of alternatively spliced mRNAs encoding type II procollagen with a cysteine-rich amino-propeptide in differentiating cartilage and nonchondrogenic tissues during early mouse development. Dev Biol 1993;159:403-17.
58. Walchli C, Koch M, Chiquet M, Odermatt BF, Trueb B. Tissue-specific expression of the fibril-associated collagens XII and XIV. J Cell Sci 1994;107:669-81.
59. Miner JH, Sanes JR. Collagen IV alpha 3, alpha 4, and alpha 5 chains in rodent basal laminae: sequence, distribution, association with laminins, and developmental switches. J Cell Biol 1994;127:879-91.
60. Bohme K, Li Y, Oh PS, Olsen BR. Primary structure of the long and short splice variants of mouse collagen XII and their tissue-specific expression during embryonic development. Dev Dyn 1995;204:432-45.
61. Niederreither K, D'Souza R, Metsaranta M et al. Coordinate patterns of expression of type I and III collagens during mouse development. Matrix Biol 1995;14:705-13.
62. Lai CH, Chu ML. Tissue distribution and developmental expression of type XVI collagen in the mouse. Tissue Cell 1996;28:155-64.
63. Marvulli D, Volpin D, Bressan GM. Spatial and temporal changes of type VI collagen expression during mouse development. Dev Dyn 1996;206:447-54.
64. Perala M, Savontaus M, Metsaranta M, Vuorio E. Developmental regulation of mRNA species for types II, IX and XI collagens during mouse embryogenesis. Biochem J 1997;324:209-16.
65. Sugimoto M, Kimura T, Tsumaki N et al. Differential in situ expression of alpha2(XI) collagen mRNA isoforms in the developing mouse. Cell Tissue Res 1998;292:325-32.
66. Saarela J, Rehn M, Oikarinen A, Autio-Harmainen H, Pihlajaniemi T. The short and long forms of type XVIII collagen show clear tissue specificities in their expression and location in basement membrane zones in humans. Am J Pathol 1998;153:611-26.
67. Aho S, Uitto J. 180-kD bullous pemphigoid antigen/type XVII collagen: tissue-specific expression and molecular interactions with keratin 18. J Cell Biochem 1999;72:356-67.
68. Sumiyoshi H, Laub F, Yoshioka H, Ramirez F. Embryonic expression of type XIX collagen is transient and confined to muscle cells. Dev Dyn 2001;220:155-62.
69. Sund M, Vaisanen T, Kaukinen S et al. Distinct expression of type XIII collagen in neuronal structures and other tissues during mouse development. Matrix Biol 2001;20:215-31.
70. Muona A, Eklund L, Vaisanen T, Pihlajaniemi T. Developmentally regulated expression of type XV collagen correlates with abnormalities in Col15a1(-/-) mice. Matrix Biol 2002;21:89-102.
71. Sato K, Yomogida K, Wada T et al. Type XXVI collagen, a new member of the collagen family, is specifically expressed in the testis and ovary. J Biol Chem 2002;277:37 678-84.
72. Boot-Handford RP, Tuckwell DS, Plumb DA, Rock CF, Poulsom R. A novel and highly conserved collagen (pro(alpha)1(XXVII)) with a unique expression pattern and unusual molecular characteristics establishes a new clade within the vertebrate fibrillar collagen family. J Biol Chem 2003;278:31 067-77.
73. Koch M, Laub F, Zhou P et al. Collagen XXIV, a vertebrate fibrillar collagen with structural features of invertebrate collagens: selective expression in developing cornea and bone. J Biol Chem 2003;278:43 236-44.
74. Rossi P, de Crombrugghe B. Identification of a cell-specific transcriptional enhancer in the first intron of the mouse alpha 2 (type I) collagen gene. Proc Natl Acad Sci U S A 1987;84:5590-4.
75. Horton W, Miyashita T, Kohno K, Hassell JR, Yamada Y. Identification of a phenotype-specific enhancer in the first intron of the rat collagen II gene. Proc Natl Acad Sci U S A 1987;84:8864-8.
76. Killen PD, Burbelo PD, Martin GR, Yamada Y. Characterization of the promoter for the alpha 1 (IV) collagen gene: DNA sequences within the first intron enhance transcription. J Biol Chem 1988;263:12 310-4.
77. Burbelo PD, Martin GR, Yamada Y. Alpha 1(IV) and alpha 2(IV) collagen genes are regulated by a bidirectional promoter and a shared enhancer. Proc Natl Acad Sci U S A 1988;85:9679-82.
78. Bennett VD, Adams SL. Identification of a cartilage-specific promoter within intron 2 of the chick alpha 2(I) collagen gene. J Biol Chem 1990;265:2223-30.
79. Greenspan DS, Lee ST, Lee BS, Hoffman GG. Homology between alpha 2(V) and alpha 1(III) collagen promoters and evidence for negatively acting elements in the alpha 2(V) first intron and 5′ flanking sequences. Gene Expr 1991;1:29-39.
80. Burbelo PD, Bruggeman LA, Gabriel GC, Klotman PE, Yamada Y. Characterization of a cis-acting element required for efficient transcriptional activation of the collagen IV enhancer. J Biol Chem 1991;266:22 297-302.
81. Pogulis RJ, Freytag SO. Contribution of specific cis-acting elements to activity of the mouse pro-alpha 2(I) collagen enhancer. J Biol Chem 1993;268:2493-9.
82. Haniel A, Welge-Lussen U, Kuhn K, Poschl E. Identification and characterization of a novel transcriptional silencer in the human collagen type IV gene COL4A2. J Biol Chem 1995;270:11 209-15.
83. Krebsbach PH, Nakata K, Bernier SM et al. Identification of a minimum enhancer sequence for the type II collagen gene reveals several core sequence motifs in common with the link protein gene. J Biol Chem 1996;271:4298-303.
84. Bornstein P. Regulation of expression of the alpha 1 (I) collagen gene: a critical appraisal of the role of the first intron. Matrix Biol 1996;15:3-10.
85. Lefebvre V, Zhou G, Mukhopadhyay K et al. An 18-basepair sequence in the mouse proalpha1(II) collagen gene is sufficient for expression in cartilage and binds nuclear proteins that are selectively expressed in chondrocytes. Mol Cell Biol 1996;16:4512-23.
86. Bell DM, Leung KK, Wheatley SC et al. SOX9 directly regulates the type-II collagen gene. Nat Genet 1997;16:174-8.
87. Zhang Y, Niu Z, Cohen AJ, Nah HD, Adams SL. The chick type III collagen gene contains two promoters that are preferentially expressed in different cell types and are separated by over 20 kb of DNA containing 23 exons. Nucleic Acids Res 1997;25:2470-7.
88. Beier F, Vornehm S, Poschl E, von der Mark K, Lammi MJ. Localization of silencer and enhancer elements in the human type X collagen gene. J Cell Biochem 1997;66:210-8.
89. Hormuzdi SG, Penttinen R, Jaenisch R, Bornstein P. A genetargeting approach identifies a function for the first intron in expression of the alpha1(I) collagen gene. Mol Cell Biol 1998;18:3368-75.
90. Leung KK, Ng LJ, Ho KK, Tam PP, Cheah KS. Different cis-regulatory DNA elements mediate developmental stageand tissue-specific expression of the human COL2A1 gene in transgenic mice. J Cell Biol 1998;141:1291-300.
91. Zhang Y, Niu Z, Cohen AJ, Adams SL. The internal chondrocyte-specific promoter of the chick type III collagen gene is activated by AP1 and is repressed in fibroblasts by a complex containing an LBP1-related protein. Nucleic Acids Res 1999;27:4090-9.
92. Hirata H, Yamamura I, Yasuda K et al. Separate cis-acting DNA elements control cell type-and tissue-specific expression of collagen binding molecular chaperone HSP47. J Biol Chem 1999;274:35 703-10.
93. Liu Y, Li H, Tanaka K, Tsumaki N, Yamada Y. Identification of an enhancer sequence within the first intron required for cartilage-specific transcription of the alpha2(XI) collagen gene. J Biol Chem 2000;275:12 712-8.
94. Tanaka K, Matsumoto Y, Nakatani F, Iwamoto Y, Yamada Y. A zinc finger transcription factor, alphaA-crystallin binding protein 1, is a negative regulator of the chondrocyte-specific enhancer of the alpha1(II) collagen gene. Mol Cell Biol 2000;20:4428-35.
95. Ghayor C, Herrouin JF, Chadjichristos C et al. Regulation of human COL2A1 gene expression in chondrocytes. Identification of C-Krox-responsive elements and modulation by phenotype alteration. J Biol Chem 2000;275:27 421-38.
96. Jenkins E, Moss JB, Pace JM, Bridgewater LC. The new collagen gene COL27A1 contains SOX9-responsive enhancer elements. Matrix Biol 2005;24:177-84.
97. Antoniv TT, Tanaka S, Sudan B et al. Identification of a repressor in the first intron of the human alpha2(I) collagen gene (COL1A2). J Biol Chem 2005;280:35 417-23.
98. Rippe RA, Umezawa A, Kimball JP, Breindl M, Brenner DA. Binding of upstream stimulatory factor to an E-box in the 3′-flanking region stimulates alpha1(I) collagen gene transcription. J Biol Chem 1997;272:1753-60.
99. Parker MI, Judge K, Gevers W. Loss of type I procollagen gene expression in SV40-transformed human fibroblasts is accompanied by hypermethylation of these genes. Nucleic Acids Res 1982;10:5879-91.
100. Fernandez MP, Young MF, Sobel ME. Methylation of type II and type I collagen genes in differentiated and dedifferentiated chondrocytes. J Biol Chem 1985;260:2374-8.
101. Rhodes K, Breindl M. Developmental changes in the methylation status of regulatory elements in the murine alpha 1(I) collagen gene. Gene Expr 1992;2:59-69.
102. Takatsu M, Uyeno S, Komura J, Watanabe M, Ono T. Agedependent alterations in mRNA level and promoter methylation of collagen alpha1(I) gene in human periodontal ligament. Mech Ageing Dev 1999;110:37-48.
103. Vindevoghel L, Kon A, Lechleider RJ et al. Smad-dependent transcriptional activation of human type VII collagen gene (COL7A1) promoter by transforming growth factor-beta. J Biol Chem 1998;273:13 053-7.
104. Chen SJ, Yuan W, Mori Y et al. Stimulation of type I collagen transcription in human skin fibroblasts by TGF-beta: involvement of Smad 3. J Invest Dermatol 1999;112:49-57.
105. Kon A, Vindevoghel L, Kouba DJ et al. Cooperation between SMAD and NF-kB in growth factor regulated type VII collagen gene expression. Oncogene 1999;18:1837-44.
106. Kouba DJ, Chung KY, Nishiyama T et al. Nuclear factorkappa B mediates TNF-alpha inhibitory effect on alpha 2(I) collagen (COL1A2) gene transcription in human dermal fibroblasts. J Immunol 1999;162:4226-34.
107. Abe H, Matsubara T, Iehara N et al. Type IV collagen is transcriptionally regulated by Smad1 under advanced glycation end product (AGE) stimulation. J Biol Chem 1999;279:14 201-6.
108. Greenwel P, Tanaka S, Penkov D et al. Tumor necrosis factor alpha inhibits type I collagen synthesis through repressive CCAAT/enhancer-binding proteins. Mol Cell Biol 2000;20:912-8.
109. Iraburu MJ, Dominguez-Rosales JA, Fontana L et al. Tumor necrosis factor alpha down-regulates expression of the alpha1(I) collagen gene in rat hepatic stellate cells through a p20C/EBPbeta-and C/EBPdelta-dependent mechanism. Hepatology 2000;31:1086-93.
110. Zhang W, Ou J, Inagaki Y, Greenwel P, Ramirez F. Synergistic cooperation between Sp1 and Smad3/Smad4 mediates transforming growth factor beta1 stimulation of alpha 2(I)-collagen (COL1A2) transcription. J Biol Chem 2000;275:39 237-45.
111. Chadjichristos C, Ghayor C, Herrouin JF et al. Downregulation of human type II collagen gene expression by transforming growth factor-beta 1 (TGF-beta 1) in articular chondrocytes involves SP3/SP1 ratio. J Biol Chem 2002;277:43 903-17.
112. Naso M, Uitto J, Klement JF. Transcriptional control of the mouse Col7a1 gene in keratinocytes: basal and transforming growth factor-beta regulated expression. J Invest Dermatol 2003;121:1469-78.
113. Stefanovic B, Brenner DA. 5′ stem-loop of collagen alpha 1(I) mRNA inhibits translation in vitro but is required for triple helical collagen synthesis in vivo. J Biol Chem 2003;278:927-33.
114. Stefanovic B, Hellerbrand C, Brenner DA. Regulatory role of the conserved stem-loop structure at the 5′ end of collagen alpha1(I) mRNA. Mol Cell Biol 1999;19:4334-42.
115. Stefanovic B, Lindquist J, Brenner DA. The 5′ stem-loop regulates expression of collagen alpha1(I) mRNA in mouse fibroblasts cultured in a three-dimensional matrix. Nucleic Acids Res 2000;28:641-7.
116. Stefanovic B, Hellerbrand C, Holcik M et al. Posttranscriptional regulation of collagen alpha1(I) mRNA in hepatic stellate cells. Mol Cell Biol 1997;17:5201-9.
117. Lindquist JN, Parsons CJ, Stefanovic B, Brenner DA. Regulation of alpha1(I) collagen messenger RNA decay by interactions with alphaCP at the 3′-untranslated region. J Biol Chem 2004;279:23 822-9.
118. Berg RA, Prockop DJ. The thermal transition of a nonhydroxylated form of collagen: evidence for a role for hydroxyproline in stabilizing the triple-helix of collagen. Biochem Biophys Res Commun 1973;52:115-20.
119. Notbohm H, Mosler S, Bodo M et al. Comparative study on the thermostability of collagen I of skin and bone: influence of posttranslational hydroxylation of prolyl and lysyl residues. J Protein Chem 1992;11:635-43.
120. Burjanadze TV, Veis A. A thermodynamic analysis of the contribution of hydroxyproline to the structural stability of the collagen triple helix. Connect Tissue Res 1997;36:347-65.
121. Jenkins CL, Raines RT. Insights on the conformational stability of collagen. Nat Prod Rep 2002;19:49-59.
122. Bann JG, Peyton DH, Bachinger HP. Sweet is stable: glycosylation stabilizes collagen. FEBS Lett 2000;473:237-40.
123. Heikkinen J, Risteli M, Wang C et al. Lysyl hydroxylase 3 is a multifunctional protein possessing collagen glucosyltransferase activity. J Biol Chem 2000;275:36 158-63.
124. Rautavuoma K, Takaluoma K, Passoja K et al. Characterization of three fragments that constitute the monomers of the human lysyl hydroxylase isoenzymes 1-3. The 30-kDa N-terminal fragment is not required for lysyl hydroxylase activity. J Biol Chem 2002;277:23 084-91.
125. Rautavuoma K, Takaluoma K, Sormunen R et al. Premature aggregation of type IV collagen and early lethality in lysyl hydroxylase 3 null mice. Proc Natl Acad Sci U S A 2004;101:14 120-5.
126. Clark CC, Kefalides NA. Carbohydrate moieties of procollagen: incorporation of isotopically labeled mannose and glucosamine into propeptides of procollagen secreted by matrix-free chick embryo tendon cells. Proc Natl Acad Sci U S A 1976;73:34-8.
127. Olsen BR, Guzman NA, Engel J, Condit C, Aase S. Purification and characterization of a peptide from the carboxyterminal region of chick tendon procollagen type I. Biochemistry 1977;16:3030-6.
128. Clark CC. The distribution and initial characterization of oligosaccharide units on the COOH-terminal propeptide extensions of the pro-alpha 1 and pro-alpha 2 chains of type I procollagen. J Biol Chem 1979;254:10 798-802.
129. Nayak BR, Spiro RG. Localization and structure of the asparagine-linked oligosaccharides of type IV collagen from glomerular basement membrane and lens capsule. J Biol Chem 1991;266:13 978-87.
130. Fujiwara S, Shinkai H, Timpl R. Structure of N-linked oligosaccharide chains in the triple-helical domains of human type VI and mouse type IV collagen. Matrix 1991;11:307-12.
131. Lamande SR, Bateman JF. The type I collagen pro alpha 1(I) COOH-terminal propeptide N-linked oligosaccharide: functional analysis by site-directed mutagenesis. J Biol Chem 1995;270:17 858-65.
132. McLaughlin SH, Bulleid NJ. Molecular recognition in procollagen chain assembly. Matrix Biol 1998;16:369-77.
133. Soder S, Poschl E. The NC1 domain of human collagen IV is necessary to initiate triple helix formation. Biochem Biophys Res Commun 2004;325:276-80.
134. Koivu J, Myllyla R. Interchain disulfide bond formation in types I and II procollagen: evidence for a protein disulfide isomerase catalyzing bond formation. J Biol Chem 1987;262:6159-64.
135. Gerard S, Puett D, Mitchell WM. Kinetics of collagen fold formation in human type I procollagen and the effect of disulfide bonds. Biochemistry 1981;20:1857-65.
136. Koivu J, Myllyla R, Helaakoski T et al. A single polypeptide acts both as the beta subunit of prolyl 4-hydroxylase and as a protein disulfide-isomerase. J Biol Chem 1987;262:6447-9.
137. Pihlajaniemi T, Helaakoski T, Tasanen K et al. Molecular cloning of the beta-subunit of human prolyl 4-hydroxylase. This subunit and protein disulphide isomerase are products of the same gene. EMBO J 1987;6:643-9.
138. Wilson R, Lees JF, Bulleid NJ. Protein disulfide isomerase acts as a molecular chaperone during the assembly of procollagen. J Biol Chem 1998;273:9637-43.
139. Kellokumpu S, Suokas M, Risteli L, Myllyla R. Protein disulfide isomerase and newly synthesized procollagen chains form higher-order structures in the lumen of the endoplasmic reticulum. J Biol Chem 1997;272:2770-7.
140. Bottomley MJ, Batten MR, Lumb RA, Bulleid NJ. Quality control in the endoplasmic reticulum: PDI mediates the ER retention of unassembled procollagen C-propeptides. Curr Biol 2001;11:1114-8.
141. Bachinger HP, Bruckner P, Timpl R, Prockop DJ, Engel J. Folding mechanism of the triple helix in type-III collagen and type-III pN-collagen: role of disulfide bridges and peptide bond isomerization. Eur J Biochem 1980;106:619-32.
142. Dolz R, Engel J, Kuhn K. Folding of collagen IV. Eur J Biochem 1988;178:357-66.
143. Snellman A, Tu H, Vaisanen T et al. A short sequence in the N-terminal region is required for the trimerization of type XIII collagen and is conserved in other collagenous transmembrane proteins. EMBO J 2000;19:5051-9.
144. Areida SK, Reinhardt DP, Muller PK et al. Properties of the collagen type XVII ectodomain: evidence for n-to cterminal triple helix folding. J Biol Chem 2001;276:1594-601.
145. Bachinger HP, Bruckner P, Timpl R, Engel J. The role of cis-trans isomerization of peptide bonds in the coil leads to and comes from triple helix conversion of collagen. Eur J Biochem 1978;90:605-13.
146. Bruckner P, Eikenberry EF. Formation of the triple helix of type I procollagen in cellulo: temperature-dependent kinetics support a model based on cis in equilibrium trans isomerization of peptide bonds. Eur J Biochem 1984;140:391-5.
147. Davis JM, Boswell BA, Bachinger HP. Thermal stability and folding of type IV procollagen and effect of peptidyl-prolyl cis-trans-isomerase on the folding of the triple helix. J Biol Chem 1989;264:8956-62.
148. Canty EG, Kadler KE. Procollagen trafficking, processing and fibrillogenesis. J Cell Sci 2005;118:1341-53.
149. Nagata K. Expression and function of heat shock protein 47: a collagen-specific molecular chaperone in the endoplasmic reticulum. Matrix Biol 1998;16:379-86.
150. Nagata K. HSP47 as a collagen-specific molecular chaperone: function and expression in normal mouse development. Semin Cell Dev Biol 2003;14:275-82.
151. Koide T, Asada S, Nagata K. Substrate recognition of collagenspecific molecular chaperone HSP47: structural requirements and binding regulation. J Biol Chem 1999;274:34 523-6.
152. Koide T, Aso A, Yorihuzi T, Nagata K. Conformational requirements of collagenous peptides for recognition by the chaperone protein HSP47. J Biol Chem 2000;275:27 957-63.
153. Koide T, Takahara Y, Asada S, Nagata K. Xaa-Arg-Gly triplets in the collagen triple helix are dominant binding sites for the molecular chaperone HSP47. J Biol Chem 2002;277:6178-82.
154. Tasab M, Jenkinson L, Bulleid NJ. Sequence-specific recognition of collagen triple helices by the collagen-specific molecular chaperone HSP47. J Biol Chem 2002;277:35 007-12.
155. Tasab M, Batten MR, Bulleid NJ. Hsp47: a molecular chaperone that interacts with and stabilizes correctly folded procollagen. EMBO J 2000;19:2204-11.
156. Macdonald JR, Bachinger HP. HSP47 binds cooperatively to triple helical type I collagen but has little effect on the thermal stability or rate of refolding. J Biol Chem 2001;276:25 399-403.
157. Nagai N, Hosokawa M, Itohara S et al. Embryonic lethality of molecular chaperone hsp47 knockout mice is associated with defects in collagen biosynthesis. J Cell Biol 2000;150:1499-506.
158. Bonfanti L, Mironov AA Jr, Martinez-Menarguez JA et al. Procollagen traverses the Golgi stack without leaving the lumen of cisternae: evidence for cisternal maturation. Cell 1998;95:993-1003.
159. Kadler KE, Holmes DF, Trotter JA, Chapman JA. Collagen fibril formation. Biochem J 1996;316:1-11.
160. Kadler KE, Holmes DF, Graham H, Starborg T. Tip-mediated fusion involving unipolar collagen fibrils accounts for rapid fibril elongation, the occurrence of fibrillar branched networks in skin and the paucity of collagen fibril ends in vertebrates. Matrix Biol 2000;19:359-65.
161. Birk DE. Type V collagen: heterotypic type I/V collagen interactions in the regulation of fibril assembly. Micron 2001;32:223-37.
162. Hulmes DJ. Building collagen molecules, fibrils, and suprafibrillar structures. J Struct Biol 2002;137:2-10.
163. Kadler K. Matrix loading: assembly of extracellular matrix collagen fibrils during embryogenesis. Birth Defects Res Part C Embryo Today 2004;72:1-11.
164. Miyahara M, Njieha FK, Prockop DJ. Formation of collagen fibrils in vitro by cleavage of procollagen with procollagen proteinases. J Biol Chem 1982;257:8442-8.
165. Miyahara M, Hayashi K, Berger J et al. Formation of collagen fibrils by enzymic cleavage of precursors of type I collagen in vitro. J Biol Chem 1984;259:9891-8.
166. Kadler KE, Hojima Y, Prockop DJ. Assembly of collagen fibrils de novo by cleavage of the type I pC-collagen with procollagen C-proteinase: assay of critical concentration demonstrates that collagen self-assembly is a classical example of an entropy-driven process. J Biol Chem 1987;262:15 696-701.
167. Shaw LM, Olsen BR. FACIT collagens: diverse molecular bridges in extracellular matrices. Trends Biochem Sci 1991;16:191-4.
168. Knupp C, Squire JM. Molecular packing in networkforming collagens. Adv Protein Chem 2005;70:375-403.
169. Burgeson RE. Type VII collagen, anchoring fibrils, and epidermolysis bullosa. J Invest Dermatol 1993;101:252-5.
170. Baldock C, Sherratt MJ, Shuttleworth CA, Kielty CM. The supramolecular organization of collagen VI microfibrils. J Mol Biol 2003;330:297-307.
171. Franzke CW, Bruckner P, Bruckner-Tuderman L. Collagenous transmembrane proteins: recent insights into biology and pathology. J Biol Chem 2005;280:4005-8.
172. Pierce JA, Hocott JB. Studies on the collagen and elastin content of the human lung. J Clin Invest 1960;39:8-14.
173. West JB, Mathieu-Costello O. Structure, strength, failure, and remodeling of the pulmonary blood-gas barrier. Annu Rev Physiol 1999;61:543-72.
174. Kielty CM, Sherratt MJ, Shuttleworth CA. Elastic fibres. J Cell Sci 2002;115:2817-28.
175. Shapiro SD, Endicott SK, Province MA, Pierce JA, Campbell EJ. Marked longevity of human lung parenchymal elastic fibers deduced from prevalence of D-aspartate and nuclear weapons-related radiocarbon. J Clin Invest 1991;87:1828-34.
176. Mithieux SM, Weiss AS. Elastin. Adv Protein Chem 2005;70:437-61.
177. Li B, Daggett V. Molecular basis for the extensibility of elastin. J Muscle Res Cell Motil 2002;23:561-73.
178. Sage H. The evolution of elastin: correlation of functional properties with protein structure and phylogenetic distribution. Comp Biochem Physiol B 1983;74:373-80.
179. Vrhovski B, Weiss AS. Biochemistry of tropoelastin. Eur J Biochem 1998;258:1-18.
180. Fazio MJ, Mattei MG, Passage E et al. Human elastin gene: new evidence for localization to the long arm of chromosome 7. Am J Hum Genet 1991;48:696-703.
181. Foster K, Ferrell R, King-Underwood L et al. Description of a dinucleotide repeat polymorphism in the human elastin gene and its use to confirm assignment of the gene to chromosome 7. Ann Hum Genet 1993;57:87-96.
182. Bashir MM, Indik Z, Yeh H et al. Characterization of the complete human elastin gene: delineation of unusual features in the 5′-flanking region. J Biol Chem 1989;264:8887-91.
183. Tassabehji M, Metcalfe K, Donnai D et al. Elastin: genomic structure and point mutations in patients with supravalvular aortic stenosis. Hum Mol Genet 1997;6:1029-36.
184. Yeh H, Anderson N, Ornstein-Goldstein N et al. Structure of the bovine elastin gene and S1 nuclease analysis of alternative splicing of elastin mRNA in the bovine nuchal ligament. Biochemistry 1989;28:2365-70.
185. Rosenbloom J, Bashir M, Yeh H et al. Regulation of elastin gene expression. Ann N Y Acad Sci 1991;624:116-36.
186. Kahari VM, Fazio MJ, Chen YQ et al. Deletion analyses of 5′-flanking region of the human elastin gene: delineation of functional promoter and regulatory cis-elements. J Biol Chem 1990;265:9485-90.
187. Swee MH, Parks WC, Pierce RA. Developmental regulation of elastin production: expression of tropoelastin pre-mRNA persists after down-regulation of steady-state mRNA levels. J Biol Chem 1995;270:14 899-906.
188. Parks WC. Posttranscriptional regulation of lung elastin production. Am J Respir Cell Mol Biol 1997;17:1-2.
189. Rosenbloom J, Cywinski A. Inhibition of proline hydroxylation does not inhibit secretion of tropoelastin by chick aorta cells. FEBS Lett 1976;65:246-50.
190. Davis EC, Broekelmann TJ, Ozawa Y, Mecham RP. Identification of tropoelastin as a ligand for the 65-kD FK506-binding protein, FKBP65, in the secretory pathway. J Cell Biol 1998;140:295-303.
191. Hinek A, Rabinovitch M. 67-kD elastin-binding protein is a protective 'companion' of extracellular insoluble elastin and intracellular tropoelastin. J Cell Biol 1994;126:563-74.
192. Kozel BA, Rongish BJ, Czirok A et al. Elastic fiber formation: a dynamic view of extracellular matrix assembly using timer reporters. J Cell Physiol 2006;DOI:10.1002/jcp.20546.
193. Kozel BA, Wachi H, Davis EC, Mecham RP. Domains in tropoelastin that mediate elastin deposition in vitro and in vivo. J Biol Chem 2003;278:18 491-8.
194. Bedell-Hogan D, Trackman P, Abrams W, Rosenbloom J, Kagan H. Oxidation, cross-linking, and insolubilization of recombinant tropoelastin by purified lysyl oxidase. J Biol Chem 1993;268:10 345-50.
195. Thomassin L, Werneck CC, Broekelmann TJ et al. The Proregions of lysyl oxidase and lysyl oxidase-like 1 are required for deposition onto elastic fibers. J Biol Chem 2005;280:42 848-55.
196. Rock MJ, Cain SA, Freeman LJ et al. Molecular basis of elastic fiber formation: critical interactions and a tropoelastinfibrillin-1 cross-link. J Biol Chem 2004;279:23 748-58.
197. Yanagisawa H, Davis EC, Starcher BC et al. Fibulin-5 is an elastin-binding protein essential for elastic fibre development in vivo. Nature 2002;415:168-71.
198. Nakamura T, Lozano PR, Ikeda Y et al. Fibulin-5/DANCE is essential for elastogenesis in vivo. Nature 2002;415:171-5.
199. Kelleher CM, Silverman EK, Broekelmann T et al. A functional mutation in the terminal exon of elastin in severe, early-onset chronic obstructive pulmonary disease. Am J Respir Cell Mol Biol 2005;33:355-62.
200. Iozzo RV. Matrix proteoglycans: from molecular design to cellular function. Annu Rev Biochem 1998;67:609-52.
201. Bensadoun ES, Burke AK, Hogg JC, Roberts CR. Proteoglycan deposition in pulmonary fibrosis. Am J Respir Crit Care Med 1996;154:1819-28.
202. Belknap JK, Weiser-Evans MC, Grieshaber SS, Majack RA, Stenmark KR. Relationship between perlecan and tropoelastin gene expression and cell replication in the developing rat pulmonary vasculature. Am J Respir Cell Mol Biol 1999;20:24-34.
203. Groffen AJ, Buskens CA, van Kuppevelt TH et al. Primary structure and high expression of human agrin in basement membranes of adult lung and kidney. Eur J Biochem 1998;254:123-8.
204. Verbeek MM, Otte-Holler I, van den Born J et al. Agrin is a major heparan sulfate proteoglycan accumulating in Alzheimer's disease brain. Am J Pathol 1999;155:2115-25.
205. van Straaten JF, Coers W, Noordhoek JA et al. Proteoglycan changes in the extracellular matrix of lung tissue from patients with pulmonary emphysema. Mod Pathol 1999;12:697-705.
206. Chakravarti S, Magnuson T, Lass JH et al. Lumican regulates collagen fibril assembly: skin fragility and corneal opacity in the absence of lumican. J Cell Biol 1998;141:1277-86.
207. Svensson L, Aszodi A, Reinholt FP et al. Fibromodulin-null mice have abnormal collagen fibrils, tissue organization, and altered lumican deposition in tendon. J Biol Chem 1999;274:9636-48.
208. Pare OD, Roberts CR, Bai TR, Wiggs BJ. The functional consequences of airway remodeling in asthma. Monaldi Arch Chest Dis 1997;52:589-96.
209. Laurell C-B, Eriksson S. The electrophoretic a1-globulin pattern of serum in a1-antitrypsin deficiency. Scand J Clin Lab Invest 1963;15:132-40.
210. Eriksson S. Pulmonary emphysema and alpha1-antitrypsin deficiency. Acta Med Scand 1964;175:197-205.
211. Ohbayashi H. Neutrophil elastase inhibitors as treatment for COPD. Expert Opin Investig Drugs 2002;11:965-80.
212. Shapiro SD. Proteinases in chronic obstructive pulmonary disease. Biochem Soc Trans 2001;30:98-102.
213. Hautamaki RD, Kobayashi DK, Senior RM, Shapiro SD. Requirement for macrophage elastase for cigarette smokeinduced emphysema in mice. Science 1997;277:2002-4.
214. Zheng T, Zhu Z, Wang Z et al. Inducible targeting of IL-13 to the adult lung causes matrix metalloproteinase-and cathepsindependent emphysema. J Clin Invest 2000;106:1081-93.
215. Mahadeva R, Shapiro SD. Chronic obstructive pulmonary disease 3: Experimental animal models of pulmonary emphysema. Thorax 2002;57:908-14.
216. Leco KJ, Waterhouse P, Sanchez OH et al. Spontaneous air space enlargement in the lungs of mice lacking tissue inhibitor of metalloproteinases-3 (TIMP-3). J Clin Invest 2001;108:817-29.
217. Wert SE, Yoshida M, LeVine AM et al. Increased metalloproteinase activity, oxidant production, and emphysema in surfactant protein D gene-inactivated mice. Proc Natl Acad Sci U S A 2000;97:5972-7.
218. Morris DG, Huang X, Kaminski N et al. Loss of integrin alpha(v)beta6-mediated TGF-beta activation causes Mmp12-dependent emphysema. Nature 2003;422:169-73.
219. Kasahara Y, Tuder RM, Taraseviciene-Stewart L et al. Inhibition of VEGF receptors causes lung cell apoptosis and emphysema. J Clin Invest 2000;106:1311-9.
220. Aoshiba K, Yokohori N, Nagai A. Alveolar wall apoptosis causes lung destruction and emphysematous changes. Am J Respir Cell Mol Biol 2003;28:555-62.
221. Massaro GD, Massaro D. Retinoic acid treatment abrogates elastase-induced pulmonary emphysema in rats. Nat Med 1997;3:675-7.
222. Ali NN, Edgar AJ, Samadikuchaksaraei A et al. Derivation of type II alveolar epithelial cells from murine embryonic stem cells. Tissue Eng 2002;8:541-50.
223. Krause DS, Theise ND, Collector MI et al. Multi-organ, multi-lineage engraftment by a single bone marrow-derived stem cell. Cell 2001;105:369-77.
224. Kotton DN, Ma BY, Cardoso WV et al. Bone marrow-derived cells as progenitors of lung alveolar epithelium. Development 2001;128:5181-8.
225. Dunsmore SE, Chambers RC, Laurent GJ. Matrix proteins. In: Gibson GJ, Geddes DM, Costabel U, Sterk PJ, Corrin B (eds). Respiratory Medicine, 3rd edn. New York: Elsevier, 2003:83.
226. Dunsmore SE. Collagens. In: Laurent G., Shapiro S., eds. Encyclopedia of Respiratory Medicine. New York: Elsevier, 2006:173.