Sanjay Bidichandani, MBBS, PhD - OUHSC Biochemistry & Molecular Biology

Gillian Air / Sanjay Bidichandani / Robert Broyles
Paul DeAngelis / Jay Hanas / Martin Levine / Guangpu Li

Jialing Lin / Hiroyuki Matsumoto / Blaine Mooers / Ann Louise Olson

Karla Rodgers / Robert Steinberg / Leon Unger / Paul Weigel / Christopher West

Sanjay I. Bidichandani, M.B.B.S., Ph.D.
Associate Professor
Biochemistry & Molecular Biology, Pediatrics
M.B.B.S., University of Poona, India, 1990
Ph.D., University of Glasgow, U.K., 1994

Phone: (405) 271-1360 Fax: (405) 271-3910 E-mail: sanjay-bidichandani@ouhsc.edu	Mailing Address: 975 N.E. 10th St., BRC 458A Oklahoma City, OK 73104
Bidichandani Lab Staff

Molecular genetics and pathophysiology of Friedreich's ataxia; Molecular biology of the GAA triplet repeat expansion; Mutational mechanisms in inherited human disease.

Over the past decade it has become apparent that abnormal expansions of trinucleotide repeats is an important mutational mechanism that causes a wide variety of inherited neurological diseases. Friedreich ataxia, the most prevalent of the inherited ataxic disorders, is caused by an abnormally large expansion of a "GAA" trinucleotide repeat sequence within the gene encoding frataxin. Research in my lab is aimed at (a) dissecting the molecular pathogenesis of Friedreich ataxia, and (b) characterizing the molecular biology of the expanded GAA trinucleotide sequence. The overall goal is to gather pertinent information that would allow design of rational therapeutic strategies for this otherwise fatal disease.

Selected Publications: [Search Pubmed]

Pollard L.M., Bourn R., Bidichandani S.I. Repair of double-strand breaks within the repeat tract enhances instability of the (GAA•TTC)_n sequence. Nucleic Acids Res., 36:489-500 (2008).

Pollard L.M., Chutake Y., Rindler P.M., and Bidichandani S.I. Deficiency of RecA-dependent RecFOR and RecBCD pathways causes increased instability of the (GAA•TTC)n sequence when GAA is the lagging strand template. Nucleic Acids Res., 35:6884-6894 (2007).

De Biase I., Rasmussen A., Monticelli A., Al-Mahdawi S., Pook M., Cocozza S., Bidichandani S.I. (2007) Somatic instability of the expanded GAA triplet-repeat sequence in Friedreich ataxia progresses throughout life. Genomics, 90:1-5.

Rasmussen A., De Biase I., Fragoso-Benitez M, Ashizawa T., Alonso M. E., Bidichandani S.I. (2007) Anticipation and intergenerational repeat instability in SCA17. Ann. Neurol., 61:107-110.

De Biase I., Rasmussen A., Endres D., Al-Mahdawi S., Monticelli A., Cocozza S., Pook M., Bidichandani S.I. (2007) Progressive GAA expansions in dorsal root ganglia of Friedreich ataxia patients. Ann. Neurol., 61:55-60.

Clark R.M., De Biase I., Malykhina A., Al-Mahdawi S., Pook M., Bidichandani S.I. (2007) The GAA triplet-repeat is unstable in the context of the human FXN locus and displays age-dependent expansions in cerebellum and DRG in a transgenic mouse model. Hum Genet.,

120:633-40.

Rindler P., Clark R.M., Pollard L.M., De Biase I., Bidichandani S.I. (2006) Replication in mammalian cells recapitulates the locus-specific differences seen in GAA triplet-repeat instability. Nucleic Acids Res., 34:6352-6361.

Rasmussen A., Gómez M., Alonso E., Bidichandani S.I. (2006) Clinical heterogeneity of recessive ataxia in the Mexican population. J. Neurol. Neurosurg. Psych., 77:1370-1372.

Clark R.M., Bhaskar S.S., Miyahara M., Dalgliesh G.L., Bidichandani S.I. (2006) Expansion of GAA trinucleotide repeats in mammals. Genomics, 87:57-67.

De Biase I., Rasmussen A., Bidichandani S.I. (2006) Evolution and Instability of the GAA Triplet-Repeat Sequence in Friedreich ataxia Genetic Instabilities and Hereditary Neurological Diseases, Ed. Robert D. Wells and Tetsuo Ashizawa. Elsevier-Academic Press, San Diego, 305-319.

Sharma R., De Biase I., Gómez M., Delatycki M.B., Ashizawa T., and Bidichandani S.I. (2004) Friedreich ataxia in carriers of unstable borderline GAA triplet-repeat alleles. Ann. Neurol. 56:898-901.

Pollard L.M., Sharma R., Gómez M., Shah S., Delatycki M., Pianese L., Monticelli A., Keats B., and Bidichandani S.I. (2004) Replication-mediated instability of the GAA triplet-repeat mutation in Friedreich ataxia. Nucl. Acids Res., 32:5962-5971.

Gómez M., Nath S.K., Clark R.M., Bhatti S., Sharma R., Alonzo E., Rasmussen A. and Bidichandani S.I. (2004) Genetic admixture of European FRDA genes is the cause of Friedreich ataxia in the Mexican population. Genomics 84:779-784 [featured on the cover]

Potaman V.N., Oussatcheva E.A., Lyubchenko Y.L., Shlyakhtenko L.S., Bidichandani S.I., Ashizawa T., Sinden R.R. (2004) Length-dependent structure formation in Friedreich’s ataxia (GAA)n•(TTC)n repeats at neutral pH. Nucl. Acids Res. 32:122-1231

Clark R.M., Dalgliesh G.L., Endres D., Gomez G., Taylor J., Bidichandani S.I. (2004) Expansion of GAA triplet-repeats in the human genome: Unique origin of the FRDA mutation at the center of an Alu. Genomics, 83: 373-183 [featured on the cover]

Gomes-Pereira M., Bidichandani S.I., Monckton D.G. (2004) Analysis of unstable triplet repeats using small-pool polymerase chain reaction. Methods Mol Biol. 277:61-76.

Sharma R., Bhatti S., Gomez M., Clark R.M., Murray C. Ashizawa T., Bidichandani S.I. (2002) The GAA triplet-repeat sequence in Friedreich ataxia shows a high level of somatic instability in vivo with a significant predilection for large contractions. Hum. Mol. Genet., 11:2175-2187.

Bidichandani SI and Ashizawa T, 2002: Friedreich Ataxia. In: GeneReviews: Genetic Disease Online Reviews at GeneTests-GeneClinics [database online]. Copyright, University of Washington, Seattle. Available at http://www.geneclinics.org

Bidichandani S.I., Garcia C., Patel P.I., Dimachkie M. 2000: Very late onset Friedreich ataxia despite large triplet repeat expansions. Arch. Neurol. 57:246-251.

Bidichandani S.I., Purandare S.M., Taylor E.E., Machkhas H., Harati Y., Gibbs R.A., Ashizawa T., Patel P.I. 1999: Somatic sequence variation in Friedreich ataxia includes complete contraction of the expanded GAA trinucleotide repeat, significant length variation in serially passaged lymphoblasts, and enhanced mutagenesis in the flanking sequence. Hum. Mol. Genet. 8:2425-2436.

Bidichandani S.I., Ashizawa T., Patel P.I. 1998: The GAA triplet repeat expansion in Friedreich ataxia interferes with transcription and may be associated with an unusual DNA structure. Am. J. Hum. Genet. 62:111-121.

Bidichandani S.I., Ashizawa T., Patel, P.I. (1997) Atypical Friedreich ataxia caused by compound heterozygosity for a novel missense mutation and the GAA triplet repeat expansion. Am. J. Hum. Genet. 60:1251-1256.

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