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Journal of Undergraduate Research

Keywords

ultrastructural, cartilage matrix fibrils, Pulmonary Hypoplasia

College

Life Sciences

Department

Physiology and Developmental Biology

Abstract

Pulmonary Hypoplasia is a lethal neonatal disorder associated with the reduction of thoracic volume due to skeletal dysplasia.’ The homozygous recessive chondrodysplasia (cho/ cho) in mice serves as our model enabling us to demonstrate how the cartilage matrix is tied together on a molecular level. Atypically wide type II collagen fibrils have been observed in the cartilage of cho/ cho fetuses. This suggests that the mutation has altered the process by which collagen is assembled into fibrils in the extracellular matrix. Recently the cho mutation was mapped to chromosome 3 which is linked to a! (XI) collagen. Based upon this knowledge an immunogold technique was applied in order to define the pathogenic mechanisms for the cartilage defect in (cho/cho) mice. The immunogold technique enables us to correlate fibril diameter with the extent of distributed al(XQ antibodies in the cartilage matrix fibrils in the heterozygote (carriers for the cho mutation). My project is to conduct and experiment with an immunogold technique described by Dr. Douglas Keene and associates.’

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