Analysis of Phenotypic Variability in Neurofibromatosis Type I (NF1)

Abstract

This project was designed to characterize the sources of phenotypic variability in neurofibromatosis 1 (NF1) by a combination of clinical, statistical, epidemiological, and molecular genetic methods. We have analysed associations found in clinical and genetic data from the NNFF International Database and two other databases with logistic regression and generalized estimating equations. We then extended these methods to use multivariate probit models on familial data. These are novel statistical techniques that have not been used in this way before and yielded interesting results about the sources of phenotypic variability. We were able to determine that the presence of some features of NF1 are more influenced by variability in the NF1 allele, others by the normal NF1 allele and still others by unlinked modifying genes. We have set up, and tested the screening protocol to identify the constitutional mutations of NF1 and have obtained a number of blood samples. We have results for one of our phenotypic subgroups and are completing the analysis at the present time.

Open PDF

Document Details

Document Type
Technical Report
Publication Date
Oct 01, 2001
Accession Number
ADA403609

Entities

People

  • Jan M. Friedman

Organizations

  • University of British Columbia

Tags

DTIC Thesaurus Topics

  • Arteries
  • Brain
  • Cardiovascular Diseases
  • Cardiovascular Physiological Phenomena
  • Cardiovascular System
  • Congenital Heart Defects
  • Genetics
  • Health Services
  • Medical Personnel

Fields of Study

  • Biology

Readers

  • Molecular and genetic basis of cancer.
  • Regression Analysis.

Technology Areas

  • Biotechnology