Characterization of a Type VI Collagen-related Mr-140 000 Protein from Cutis-laxa Fibroblasts in Culture

Overview

Journal Biochem J

Specialty Biochemistry

Date 1985 Apr 15

PMID 4004777

Citations 12

Authors

S W Crawford

J A Featherstone

K Holbrook

S L Yong

P Bornstein

H SAGE

Affiliations

Soon will be listed here.

Abstract

The precise biochemical defects in connective-tissue metabolism that are responsible for the laxity of skin seen in the syndrome of cutis laxa are largely unknown. We have studied fibroblasts cultured from skin explants of a 2-year-old male with the syndrome. Electron-microscopic examination of this skin revealed decreased amounts of amorphous elastin and an increase in elastin-associated microfibrils. Although the cultured fibroblasts were similar to control skin fibroblasts in morphology, growth rate and total protein synthesis, there was a 4-6-fold increase in accumulation of a collagenous protein of Mr 140 000 in both the culture medium and in the cell layer. This protein was structurally distinct from collagen types I, III, IV, V and VIII. It was found to be related to a cell-surface-associated glycoprotein, GP140, by both antigenic cross-reactivity and peptide mapping. Our data support observations that GP140 is a precursor of at least one form of pepsin-extracted type VI collagen.

Citing Articles

An immunohistochemical study examining the role of collagen type VI in the rodent periodontal ligament.

Sloan P, Carter D, Kielty C, Shuttleworth C Histochem J. 1993; 25(7):523-30.

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Type VI collagen and glycoprotein MFPI are distinct components of the extracellular matrix.

Ayad S, Chambers C, Berry L, Shuttleworth C, Grant M Biochem J. 1986; 236(1):299-302.

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Collagen studies in congenital cutis laxa.

Taieb A, Aumailley M, Courouge-Dorcier D, Rabaud M, Bioulac-Sage P, Maleville J Arch Dermatol Res. 1987; 279(5):308-14.

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Ultrastructure of type VI collagen in human skin and cartilage suggests an anchoring function for this filamentous network.

Keene D, Engvall E, Glanville R J Cell Biol. 1988; 107(5):1995-2006.

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Collagen gene expression by cultured human skin fibroblasts. Abundant steady-state levels of type VI procollagen messenger RNAs.

Olsen D, Peltonen J, Jaakkola S, Chu M, Uitto J J Clin Invest. 1989; 83(3):791-5.

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