Literature DB >> 1054840

Abnormal collagen metabolism in cultured cells in osteogenesis imperfecta.

R P Penttinen, J R Lichtenstein, G R Martin, V A McKusick.   

Abstract

Cells obtained from normal human skin synthesize predominantly type I collogen in culture. Cells obtained from the skin of an infant with a severe form of osteogenesis imperfecta were found to synthesize as much type III as type I collagen. Decreased synthesis of type I collagen could explain the tissue fragility observed in this case.

Entities:  

Mesh:

Substances:

Year:  1975        PMID: 1054840      PMCID: PMC432358          DOI: 10.1073/pnas.72.2.586

Source DB:  PubMed          Journal:  Proc Natl Acad Sci U S A        ISSN: 0027-8424            Impact factor:   11.205


  19 in total

1.  (Alpha1(3))3 human skin collagen. Release by pepsin digestion and preponderance in fetal life.

Authors:  E H Epstein
Journal:  J Biol Chem       Date:  1974-05-25       Impact factor: 5.157

2.  Human aorta collagens: evidence for three distinct species.

Authors:  R L Trelstad
Journal:  Biochem Biophys Res Commun       Date:  1974-04-08       Impact factor: 3.575

3.  Structural studies on cartilage collagen employing limited cleavage and solubilization with pepsin.

Authors:  E J Miller
Journal:  Biochemistry       Date:  1972-12-19       Impact factor: 3.162

Review 4.  A review of biochemical studies on the genetically distinct collagens of the skeletal system.

Authors:  E J Miller
Journal:  Clin Orthop Relat Res       Date:  1973-05       Impact factor: 4.176

5.  Preparation of type III procollagen and collagen from rat skin.

Authors:  P H Byers; K H McKenney; J R Lichtenstein; G R Martin
Journal:  Biochemistry       Date:  1974-12-03       Impact factor: 3.162

6.  Defect in conversion of procollagen to collagen in a form of Ehlers-Danlos syndrome.

Authors:  J R Lichtenstein; G R Martin; L D Kohn; P H Byers; V A McKusick
Journal:  Science       Date:  1973-10-19       Impact factor: 47.728

7.  Collagen polymorphism: characterization of molecules with the chain composition (alpha 1 (3)03 in human tissues.

Authors:  E Chung; E J Miller
Journal:  Science       Date:  1974-03       Impact factor: 47.728

8.  Acquired and heritable defects in collagen synthesis and fibrogenesis.

Authors:  V A McKusick; G E Martin; J R Lichtenstein; R P Penttinen; D W Rowe; M D Sussman; P H Byers
Journal:  Trans Am Clin Climatol Assoc       Date:  1974

9.  A heritable disorder of connective tissue. Hydroxylysine-deficient collagen disease.

Authors:  S R Pinnell; S M Krane; J E Kenzora; M J Glimcher
Journal:  N Engl J Med       Date:  1972-05-11       Impact factor: 91.245

10.  The nature of the collagen synthesized by cultured human fibroblasts.

Authors:  D L Layman; E B McGoodwin; G R Martin
Journal:  Proc Natl Acad Sci U S A       Date:  1971-02       Impact factor: 11.205

View more
  43 in total

Review 1.  Molecular abnormalities of collagen.

Authors:  F M Pope; A C Nicholls
Journal:  J Clin Pathol Suppl (R Coll Pathol)       Date:  1978

2.  Biochemical characteristics and biological significance of the genetically-distinct collagens.

Authors:  E J Miller
Journal:  Mol Cell Biochem       Date:  1976-12-10       Impact factor: 3.396

3.  Bone disease in the elderly.

Authors:  R Smith
Journal:  Proc R Soc Med       Date:  1976-12

4.  Increased expression of the gene for the pro alpha 1(IV) chain of basement-membrane procollagen in cultured skin fibroblasts from two variants of osteogenesis imperfecta.

Authors:  T Pihlajaniemi; J McKeon; S Gay; R Gay; W J de Wet; J C Myers; D J Prockop
Journal:  Biochem J       Date:  1989-01-15       Impact factor: 3.857

5.  [Pathophysiology and metabolism in osteogenesis imperfecta (author's transl)].

Authors:  K J Münzenberg
Journal:  Arch Orthop Unfallchir       Date:  1977-12-09

6.  Pseudoxanthoma elasticum and acrosclerosis.

Authors:  J D Wilkinson
Journal:  Proc R Soc Med       Date:  1977-08

7.  Genetic heterogeneity in osteogenesis imperfecta.

Authors:  D O Sillence; A Senn; D M Danks
Journal:  J Med Genet       Date:  1979-04       Impact factor: 6.318

8.  Osteogenesis imperfecta type IIA: evidence for dominant inheritance.

Authors:  I D Young; E M Thompson; C M Hall; M E Pembrey
Journal:  J Med Genet       Date:  1987-07       Impact factor: 6.318

9.  Nuclease S1 mapping of a homozygous mutation in the carboxyl-propeptide-coding region of the pro alpha 2(I) collagen gene in a patient with osteogenesis imperfecta.

Authors:  L A Dickson; T Pihlajaniemi; S Deak; F M Pope; A Nicholls; D J Prockop; J C Myers
Journal:  Proc Natl Acad Sci U S A       Date:  1984-07       Impact factor: 11.205

10.  Immunohistological study on collagen in cartilage-bone metamorphosis and degenerative osteoarthrosis.

Authors:  S Gay; P K Müller; C Lemmen; K Remberger; K Matzen; K Kühn
Journal:  Klin Wochenschr       Date:  1976-10-15
View more

北京卡尤迪生物科技股份有限公司 © 2022-2023.