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Titolo:
Collagens and collagen-related diseases
Autore:
Myllyharju, J; Kivirikko, KI;
Indirizzi:
Univ Oulu, Dept Med Biochem, Oulu 90014, Finland Univ Oulu Oulu Finland 90014 Oulu, Dept Med Biochem, Oulu 90014, Finland Univ Oulu, Bioctr, Collagen Res Unit, Oulu, Finland Univ Oulu Oulu Finland v Oulu, Bioctr, Collagen Res Unit, Oulu, Finland
Titolo Testata:
ANNALS OF MEDICINE
fascicolo: 1, volume: 33, anno: 2001,
pagine: 7 - 21
SICI:
0785-3890(200102)33:1<7:CACD>2.0.ZU;2-1
Fonte:
ISI
Lingua:
ENG
Soggetto:
EHLERS-DANLOS-SYNDROME; MULTIPLE EPIPHYSEAL DYSPLASIA; PROTEIN DISULFIDE-ISOMERASE; SYNDROME TYPE-VI; SYNDROME TYPE-I; ANGIOGENESIS INHIBITOR ENDOSTATIN; HUMAN PROLYL 4-HYDROXYLASE; LYSYL HYDROXYLASE ISOFORM; EXON-INTRON ORGANIZATION; PROCOLLAGEN C-PROTEINASE;
Keywords:
collagen; fibrosis; heritable diseases; intervertebral disc disease; mutant mice; osteoarthrosis;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
144
Recensione:
Indirizzi per estratti:
Indirizzo: Kivirikko, KI Univ Oulu, Dept Med Biochem, POB 5000, Oulu 90014, Finland Univ Oulu POB 5000 Oulu Finland 90014 , Oulu 90014, Finland
Citazione:
J. Myllyharju e K.I. Kivirikko, "Collagens and collagen-related diseases", ANN MED, 33(1), 2001, pp. 7-21

Abstract

The collagen superfamily of proteins plays a dominant role in maintaining the integrity of various tissues and also has a number of other important functions. The superfamily now includes more than 20 collagen types with altogether at least 38 distinct polypeptide chains, and more than 15 additional proteins that have collagen-like domains. Most collagens form polymeric assemblies, such as fibrils, networks and filaments, and the superfamily canbe divided into several families based on these assemblies and other features. All collagens also contain noncollagenous domains, and many of these have important functions that are distinct from those of the collagen domains, Major interest has been focused on endostatin, a fragment released from type XVIII collagen. which potently inhibits angiogenesis and tumour growth. Collagen synthesis requires eight specific post-translational enzymes, some of which are attractive targets for the development of drugs to inhibit collagen accumulation in fibrotic diseases. The critical roles of collagenshave been clearly illustrated by the wide spectrum of diseases caused by the more than 1000 mutations that have thus far been identified in 22 genes for 12 out of the more than 20 collagen types. These diseases include osteogenesis imperfecta, many chondrodysplasias, several subtypes of the Ehlers-Danlos syndrome, Alport syndrome, Bethlem myopathy, certain subtypes of epidermolysis bullosa, Knobloch syndrome and also some cases of osteoporosis, arterial aneurysms. osteoarthrosis, and intervertebral disc disease, The characterization of mutations in additional collagen genes will probably add further diseases to this list. Mice with genetically engineered collagen mutations have proved valuable for defining the functions of various collagens and for studying many aspects of the related diseases.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 14/07/20 alle ore 06:57:14