Literature DB >> 2899976

Achondroplasia is not caused by mutation in the gene for type II collagen.

C A Francomano1, R E Pyeritz.   

Abstract

Achondroplasia is the most common human skeletal dysplasia. It is inherited as an autosomal dominant trait but the underlying biochemical cause is unknown. Genomic DNA from 49 affected individuals and two multiplex families with achondroplasia was studied using probes spanning COL2A1, the structural gene for type II collagen. Two lines of evidence speak against mutation in COL2A1 as the cause of achondroplasia: (1) no gross rearrangements are seen on Southern blot analysis of DNA from probands, and (2) linkage studies in multiplex families demonstrate discordant inheritance of achondroplasia and COL2A1 alleles.

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Year:  1988        PMID: 2899976     DOI: 10.1002/ajmg.1320290433

Source DB:  PubMed          Journal:  Am J Med Genet        ISSN: 0148-7299


  4 in total

1.  Lethal skeletal dysplasia owing to double heterozygosity for achondroplasia and spondyloepiphyseal dysplasia congenita.

Authors:  I D Young; N R Ruggins; J M Somers; J M Zuccollo; N Rutter
Journal:  J Med Genet       Date:  1992-11       Impact factor: 6.318

Review 2.  Molecular heterogeneity in chondrodysplasias.

Authors:  P H Byers
Journal:  Am J Hum Genet       Date:  1989-07       Impact factor: 11.025

3.  Analysis of the chondroitin sulfate proteoglycan core protein (CSPGCP) gene in achondroplasia and pseudoachondroplasia.

Authors:  J E Finkelstein; K Doege; Y Yamada; R E Pyeritz; J M Graham; J B Moeschler; R M Pauli; J T Hecht; C A Francomano
Journal:  Am J Hum Genet       Date:  1991-01       Impact factor: 11.025

4.  SSCP and segregation analysis of the human type X collagen gene (COL10A1) in heritable forms of chondrodysplasia.

Authors:  W A Sweetman; B Rash; B Sykes; P Beighton; J T Hecht; B Zabel; J T Thomas; R Boot-Handford; M E Grant; G A Wallis
Journal:  Am J Hum Genet       Date:  1992-10       Impact factor: 11.025

  4 in total

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