Both skin and gingival fibroblasts in the Papillon-Lefévre patient synthesized Type I and Type III collagen in a ratio very similar to fibroblasts of control cells. The only abnormality appeared to be elevated levels of collagen and protein synthesis by gingival cells derived from the Papillon-Lefévre patient.
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References
1.
Gorlin, R.J. ; Sedano, H.; and Anderson, V.E.: The Syndrome of Palmar-Plantar Hyperkeratosis and Premature Periodontal Destruction of the Teeth, J Pediatr65:895-908, 1964.
2.
Benya, P.D.; Padilla, S.R.; and Nimni, M.E.: The Progeny of Rabbit Articular Chondrocytes Synthesize Collagen Type I and III and Type I Trimer, But Not Type II, Biochemistry16:865-872, 1977.
3.
Cheung, H.S. ; Harvey, W.; Benya, P.D.; and Nimni, M.E.: New Collagen Markers of "Derepression" Synthesized by Rabbit Articular Chondrocytes in Culture, Biochem Biophys Res Commun68:1371-1378, 1976 .
4.
Cheung, H.S. ; Cottrell, W.H.; Stephenson, K.; and Nimni, M.E.: In vitro Collagen Biosynthesis in Healing and Normal Rabbit Articular Cartilage , J Bone Joint Surg60A: 1076-1081, 1978 .
5.
Miller, E.J. : Structural Studies on Cartilage Collagen Employing Limited Cleavage and Solubilization with Pepsin, Biochemistry11:4903-4909, 1972.
6.
Ballard, J.B. and Butler, W.T.: Proteins of the Periodontium. Biochemical Studies on Collagen and Non-collagenous Proteins of Human Gingiva , J Oral Pathol3:176-184, 1974.
7.
Schneir, M.; Ogata, S.; and Fine, A.: Confirmation That Neither Phenotype nor Hydroxylation of Collagen is Altered in Overgrown Gingiva from Diphenylhydantoin-treated Patients, J Dent Res57:506-510, 1978.
8.
Narayanan, A.S. and Page, R.C.: Biochemical Characterization of Collagen Synthesized by Fibroblasts Derived from Normal and Diseased Human Gingiva , J Biol Chem251: 5464-5471, 1976 .
Shoshan, S. ; Finkelstein, S.; and Rosenzweig , K.A.: Disc Electrophoretic Pattern of Gingival Collagen Isolated from a Patient with Palmoplantar Hyperkeratosis , J Periodont Res5:255-258, 1970.
11.
LeRoy, E.C.: Increased Collagen Synthesis by Scleroderma Skin Fibroblasts in vitro, J Clin Invest54:880-889, 1974.
12.
Perlish, J.S. ; Bashey, R.I.; Stephen , R.E.; and Fleischmajer , R.: Connective Tissue Synthesis by Sclerodermal Fibroblast. I. In vitro Collagen Synthesis by Normal and Scleroderma Dermal Fibroblasts, Arthritis Rheum19: 891-899, 1976.
13.
Buckingham, R.B.; Princi, R.K.; Rodnan , G.P.; and Taylor, F.: Increased Collagen Accumulation in Dermal Fibroblast Cultures from Patients with Progressive Systemic Sclerosis, J Lab Clin Med92:5-21, 1978.
14.
Counts, D.F. ; Knighten, P.; and Hegreberg, G.: Biochemical Changes in the Skin of Mink with Ehlers-Danlos Syndrome: Increased Collagen Biosynthesis in the Dermis of Affected Mink, J Invest Dermatol69:521-5 26, 1977.
15.
Berg, R.A.; Schwartz, M.L.; and Crystal, R.G.: Regulation of the Production of Secretory Proteins: Intracellular Degradation of Newly Synthesized "Defective" Collagen, Proc Natl Acad Sci USA77:4746-4750, 1980 .