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Pediatrics
Center for Human Molecular Genetics

Maurice Godfrey, Ph.D.

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ACADEMIC DEGREES:
Graduate School: Columbia University, New York, N.Y.
Post-doctoral training: Shriners Hospital and Oregon Health Sciences University, Portland

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Contact Name: Maurice Godfrey, Ph.D.
Phone Number: 402-559-6689
e-mail address: mgodfrey@unmc.edu

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Principal Investigator, American Heart Association, Nebraska Affiliate Fibrillin-2 in cardiovascular development: A mouse model." 7/1/2000-6/30/2002

Principal Investigator, American Heart Association, Nebraska Affiliate "Aortic aneurysms and latent transforming growth factor beta proteins." 7/1/98- 6/30/2000

Principal Investigator, March of Dimes Birth Defects Foundation "Molecular basis of Marfan-like syndromes." 6/98-5/2000

Principal Investigator, American Heart Association, Established Investigator Award "Fibrillin and the Marfan syndrome: A connective tissue paradigm for cardiovascular disease" 7/1/95-6/30/2000

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Godfrey, M., Cisler, J., Christiano, A.M., Uitto, J., Geerts, M.L., De Bie, S., and De Paepe, A. Fibrillin immunofluorescence in pseudoxanthoma elasticum. J. Am. Acad. Derm. 32:589-594, 1995.

Godfrey, M., Raghunath, M. Cisler, J., Bevins, C.L., DePaepe, A., Di Rocco, M., Gregoritch, J., Imaizumi, K., Kaplan, P., Kuroki, Y., Silberbach, M., Superti-Furga, A., Van Thienen, M., Vetter, U., and Steinmann, B. Abnormal morphology of fibrillin microfibrils in fibroblast cultures from patients with neonatal Marfan syndrome. Am. J. Pathol. 146: 1414-1421, 1995.

Schaefer, G.B. and Godfrey, M. Quantitation of fibrillin immunofluorescence in fibroblast cultures in the Marfan syndrome. Clinical Genetics 47: 144-149, 1995.

Wang, M., Price, C.E., Han, J., Cisler, J., Imaizumi, K., Van Thienen, M.N., De Paepe, A., and Godfrey, M. Recurrent mis-splicing of fibrillin exon 32 in two patients with neonatal Marfan syndrome. Human Molecular Genetics 4:607-613, 1995.

Wang, M., Mata, J.E., Price, C.E., Iversen, P.L., and Godfrey, M. Prenatal and presymptomatic diagnosis of the Marfan syndrome using fluorescence PCR and an automated sequencer. Prenatal Diagnosis 15:499-507, 1995.

Kilpatrick, M.W., Phylactou, L.A., Godfrey, M., Wu, G.Y., Wu, C.H., and Tsipouras, P. Delivery of a hammerhead ribozyme specifically down-regulates the production of fibrillin-1 by cultured dermal fibroblasts. Human Molecular Genetics 5:1939-1944, 1996.

Karttunen, L., Lonnqvist, L., Godfrey, M., Peltonen, L., and Syvanen, A-C. The effect of defined Marfan mutations upon the abundance of steady-state mRNA derived from the wild-type and mutant fibrillin-1 alleles and from the fibrillin-2 gene. Genome Research 6: 392-403, 1996.

Wang, M., Kishnani, P., Decker-Phillips, M., Kahler, S.G., Chen, Y-T., and Godfrey, M. Double mutant allele in the FBN1 gene in a case of neonatal Marfan syndrome. J. Med. Genet. 33: 760-763, 1996.

Wirtz, M.K., Samples, J.R., Kramer, P.L., Rust, K., Yount, J., Acott, T.S., Koler, R.D., Cisler, J., Jahed, A., Gorlin, R.J., Godfrey, M. Weill-Marchesani syndrome- possible linkage of the autosomal dominant form to 15q21.1. Am. J. Med. Genet. 65: 68-75, 1996.

Wang, M., Clericuzio, C.L., and Godfrey, M. Familial occurrence of typical and severe lethal congenital contractural arachnodactyly caused by mis-splicing of exon 34 of fibrillin-2 (FBN2). Am. J. Hum. Genet. 59:1027-1034, 1996.

Wang, M., Wang, J-Y., Imaizumi, K., Burton, B.K., Jones, M.C., Lamberti, J.J., and Godfrey, M. Three novel fibrillin mutations in exons 25 and 27: classical versus neonatal Marfan syndrome. Human Mutation 9: 359-362, 1997.

Collod-Béroud, G., Béroud, C., Adès, L., Black, C., Boxer, M., Brock, D.J., Godfrey, M., Hayward, C., Karttunen, L., Milewicz, D., Peltonen, L., Richards, R.I., Wang, M., Junien, C., Boileau, C. Marfan Database (second edition): Software and database for the analysis of mutations in the human FBN1 gene. Nucleic Acids Research 25:147-150, 1997.

Wang, M., Mathews, K.R., Imaizumi, K., Beiraghi, S., Blumberg, B., Scheuner, M., Graham, Jr, J.M., and Godfrey, M. P1148A in fibrillin-1: it is not a mutation anymore. Nature Genetics 15:12, 1997.

Schievink, W.I., Wijdicks, E.F.M., Michels, V.V., Vockley, G., and Godfrey, M Heritable connective tissue disorders in patients with spontaneous cervical artery dissections. A prospective clinical study. Neurology 50:1166-9, 1998.

Belleh S., Spooner, L., Allanson, J., and Godfrey, M. Prenatal diagnosis in congenital contractural arachnodactyly. Genetic Testing 1:293-296, 1998.

Booms, P., Cisler, J., Mathews, K.R., Godfrey, M., Tiecke, F., Kaufmann, U.C., Vetter, U., Hagemeier, C., and Robinson, P.N. Novel exon skipping mutation in the fibrillin-1 gene: Two ‘hot spots’ for the neonatal Marfan syndrome. Clinical Genetics 55: 110-7, 1999.

Zhou, G., Price, C.E., Rosenquist, T.H., Gadson, P.F. and Godfrey, M. Partial cloning and sequencing of chick fibrillin-1 cDNA. InVitro Cellular and Developmental Biology -Animal 36:19-25, 2000

Robinson, P.N. and Godfrey, M. The molecular genetics of the Marfan syndrome and related microfibrilopathies. J. Med. Genet. 37:9-25, 2000.

Belleh, S., Zhou, G., Wang, M., Der Kaloustian, V.M., Pagon, R.A., and Godfrey, M. Two novel fibrillin-2 mutations in congenital contractural arachnodactyly. Am. J. Med Genet. 92: 7-12, 2000.

Godfrey M. (Updated November 2000) Congenital Contractural Arachnodactyly. In: GeneClinics: Medical Genetics Knowledge Base [database online]. Copyright, University of Washington, Seattle. Available at http://www.geneclinics.org/profiles/cca (in press)

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  • PREVIOUS GRADUATE STUDENTS/POST-DOCTORAL FELLOWS (present location):

Christopher Price, M.S., M.D. (Resident, University of Nebraska Medical Center)
Guimei Zhou, M.S. M.D. (Instructor, University of Nebraska Medical Center)
Kurt Mathews, M.S., M.D. (Resident, University of Nebraska Medical Center)
Jason Cisler, M.D. (Resident, Northwestern University)
Mei Wang, M.D. (Faculty, University of Nebraska Medical Center)
Ji-Yi Wang, M.D. (Resident, State University of New York, Stonybrook)
Silvia Plaza, M.D.

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  • CURRENT RESEARCH PROJECTS:
  • OF MICE, MEN, AND CHICKENS:
    FIBRILLINS IN DEVELOPMENT AND DISEASE

    The outline below reflects the interests of our laboratory in the elucidation and characterization of some heritable disorders of connective tissue. These studies include direct analysis of patient material for diagnostic purposes, the design of mouse models using transgenic or knockout technologies, and the identification of chick fibrillin homologs for studies of the ontogeny of the cardiovascular system and limb development.

I. Human Studies

A. Fibrillin immunochemical analyses in the Marfan syndrome and related disorders.

a. Neonatal Marfan syndrome
b. Pseudoxanthoma elasticum
c. Weill-Marchesani syndrome
d. Unspecified Connective Tissue Disease

    B. Genetic linkage of Marfan syndrome and congenital contractural arachnodactyly (CCA).
    C. Prenatal and presymptomatic diagnoses in the Marfan syndrome (MFS) and CCA.
    D. Mutation detection in disorders caused by defects in FBN1 and FBN2.
    E. Mutation analyses of other microfibrils in MFS-, CCA- like disorders (LTBP-2, MAGP etc).
    F. Genotype/phenotype correlation of MFS, CCA, and related disorders.
    G. Fibrillin modulation analyses using antisense oligonucleotides.

    II. Mouse models

    A. Vector constructs and development of FBN2 knockout mice.
    B. Developmental studies in knockout animal models.

    III. Chicken models

    A. Identification of the chicken homologs to fibrillin-1 (partially complete) and fibrillin-2.
    B. In situ analyses of the fibrillins in chick development.
    C. Antisense targeting of the fibrillin in development.

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