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Biochemistry of Collagen in Diseases
- JOUNI UITTO, M.D., Ph.D.;
- LOUANN W. MURRAY, Ph.D.;
- BRUCE BLUMBERG, Ph.D.; and
- AVA SHAMBAN, M.D.
Abstract
Collagen, the major extracellular matrix component, comprises a family of closely related, yet genetically distinct proteins that provide physiologic tensile properties to the tissues. The recent progress made in understanding the normal biochemistry and biology of collagens has permitted us to identify different levels at which errors might be introduced into the structure and metabolism of this protein. Several acquired and heritable diseases are now known to involve distinct abnormalities in collagen. This conference reviews some of the latest discoveries in the biochemistry of collagen and highlights some disease entities in which definitive information on molecular alterations in collagen is available.
- achondrogenesis II
- collagen
- collagen diseases
- connective tissue diseases
- cutis laxa
- dermal fibrosis
- dwarfing syndromes
- Ehlers-Danlos syndrome
- familial cutaneous collagenoma
- focal dermal hypoplasia
- gene expression, regulation
- homocystinuria
- keloid
- kinky hair (Menkes') syndrome
- Kniest dysplasia
- Marfan syndrome
- osteogenesis imperfecta
- systemic sclerosis
- spondyloepiphyseal dysplasia
Article and Author Information
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▸An edited summary of an Interdepartmental Conference arranged by the Department of Medicine of the UCLA School of Medicine, Los Angeles, California. William M. Pardridge, M.D., Professor of Medicine, is Director of Conferences.
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▸Authors who wish to cite a section of the conference and specifically indicate its author can use this example for the form of reference:
MURRAY LW. Genetic diversity of collagen structure, pp 740-744. In: UITTO J, moderator. Biochemistry of collagen in diseases. Ann Intern Med. 1986;105:740-756.
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▸Requests for reprints should be addressed to Jouni Uitto, M.D., Ph.D.; Division of Dermatology, Harbor-UCLA Medical Center, 1000 West Carson Street; Torrance, CA 90509.
- © 1986 American College of Physicians
