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Peter J. McGuire, M.S., M.D.

Investigator, Medical Genomics and Metabolic Genetics Branch
Head, Metabolism, Infection and Immunity Section

Selected Publications

Offit, K., Gilewski, T., McGuire, P., Schluger, A., Hampel, H., Brown, K., Swenson, J., Neuhausen, S., Skolnick, M., Norton, L., and Goldgar, D. Germline BRCA1 185delAG mutations in Jewish women affected by breast cancer. Lancet, 347, 1643-1644. 1996. [PubMed]

Neuhausen, S., Gilewski, T., Norton, L., Tran, T., McGuire, P., Swensen, J., Hampel, H., Borgen, P., Brown, K., Skolnick, M., et al.  Recurrent BRCA2 6174delT mutations in Ashkenazi Jewish women affected by breast cancer. Nature Genetics, 13, 126-128. 1996. [PubMed]
Gurrieri, C., McGuire, P., Zan, H., Yan, X. J., Cerutti, A., Albesiano, E., Allen, S., Vinciguerra, V., Rai, K. R., Ferrarini, M., et al. Chronic lymphocytic leukemia B cells can undergo somatic hypermutation and intraclonal Ig VHDJH gene diversification. J Exp Med, 196, 629-639. 2002. [PubMed]
McGuire, P., Lim-Melia, E., Diaz, G.A., Raymond, K., Larkin, A., Shneider, B.L.,Wasserstein, M.P., Sansaricq, C. Combined liver-kidney transplant for the management of methylmalonic aciduria: a case report and review of the literature. Mol Genet Metab, 93: 22-29. 2008. [PubMed]
McGuire, P., Parikh, A., Diaz, G.A. Profiling of oxidative stress in patients with inborn errors of metabolism. Mol Genet Metab, 98:173-180. 2009. [PubMed]
Weisfeld-Adams, J.D.,  Morrissey, M.A., Kirmse, B.M., Salveson, B.R. Wasserstein, M.P.,   McGuire, P.J., Sunny, S., Cohen-Pfeffer, J.L., Yu, C., Caggana, M. Newborn screening and early biochemical follow-up in combined methylmalonic aciduria and homocystinuria, cblC type, and utility of methionine as a secondary screening analyte. Mol Genet Metab, 99:116-123. 2010. [PubMed]
McGuire, P.J., Cunningham-Rundles, C., Ochs, H., Diaz, G.A. Oligoclonality, impaired class switch and B cell memory responses in WHIM syndrome. Clin Immunol, 135:412-421. 2010. [PubMed]
McGuire, P.J., Lee, H.S., members of the Urea Cycle Disorders Consortium, Summar, M. Infectious precipitants of acute hyperammonemia are associated with indicators of increased morbidity in patients with urea cycle disorders. J Pediatr, 163:1705-1710. 2013. [PubMed]
Chandler, R. J., Tarasenko, T.N., Cusmano-Ozog, K., Sun, Q., Reid Sutton, V., Venditti, C.P., McGuire, P.J. Liver-directed adeno-associated virus serotype 8 gene transfer rescues a lethal murine model of citrullinemia type 1. Gene Ther, 20:1188-1191. 2013. [PubMed]
McGuire, P.J., Tarasenko, T.N., Wang, T., Levy, E., Zerfas, P.M., Moran, T.M., Lee, H.S., Bequette, B.J., Diaz, G.A. Acute metabolic decompensation due to influenza in a mouse model of ornithine transcarbamylase deficiency. Dis Model Mech, 7:205-213. 2014. [PubMed]
Tan M., Peng C., Anderson K.A., Chhoy P., Xie Z., Dai L., Park J.S., Chen Y., Huang H., Zhang Y., Ro J., Wagner G.R., Green M.F., Madsen A.S., Schmiesing J., Peterson B.S., Xu G., Ilkayeva O.R., Muehlbauer M.J., Braulke T., Mühlhausen C., Backos D.S., Olsen C.A., McGuire P.J., Pletcher S.D., Lombard D.B., Hirschey M.D., Zhao Y. Lysine Glutarylation Is a Novel Protein Modification Regulated by SIRT5. Cell Metab, 19:605-617. 2014. [PubMed]

Batshaw, M.L., Tuchman, M., Summar, M., Seminara, J.; Members of the Urea Cycle Disorders Consortium (McGuire, P.J.).  A longitudinal study of urea cycle disorders. Mol Genet Metab, 113:127-130. 2014. [PubMed]

Tarasenko, T.N., Rosas, O.R., Singh, L.N., Kristaponis, K., Vernon, H.J, McGuire, P.J. A new mouse model of ornithine transcarbamylase deficiency (spf-j) displays cerebral amino acid perturbations at baseline and upon systemic immune activation. PLOS One, 10:e0116594. 2015. [PubMed]

Tarasenko, T.N., Gomez-Rodriguez, J., McGuire, P.J. Impaired T-cell function in argininosuccinate synthetase deficiency. J Leuk Biol, 97:273-278. 2015. [PubMed]

Gartner, V. McGuire, P.J., Lee, P.R.  Child neurology: Medium-chain acyl-coenzyme A dehydrogenase (MCAD) deficiency. Neurology, 85:e37-e40. 2015. [PubMed]

Tarasenko, T.N., Singh, L.N., Chatterji-Len, M., Zerfas, P.M., Cusmano-Ozog, K., McGuire, P.J. Kupffer cells modulate hepatic fatty acid oxidation during infection with PR8 influenza. Biochim Biophys Acta 1852:2391-2401. 2015. [PubMed]

Macleod, E., Hall, K.D., McGuire, P.J. Computational modeling to predict nitrogen balance during acute metabolic decompensation in patients with urea cycle disorders. J Inherit Metab Dis, 39:17-24. 2016. [PubMed]

Harrington, E.A., Sloan, J.L., Manoli, I., Chandler, R.J., Schneider, M., McGuire, P.J., Calcedo, R., Wilson, J.M., Venditti, C.P. Neutralizing antibodies against adeno-associated viral capsids in patients with mut methylmalonic acidemia. Hum Gene Ther, 27:345-353. 2016. [PubMed]

Larsen, S.E., Bilenkin, A., Tarasenko, T.N., Arjunaraja, S., Stinson, J.R., McGuire, P.J., Snow, A.L. Sensitivity to restimulation-induced cell death is linked to glycolytic metabolism in human T cells. J Immunol, 198:147-155. 2017. PMCID: PMC5310646. [PubMed]

Tarasenko, T.N., Pacheco, S.E., Koenig, M.K., Gomez-Rodriguez, J., Kapnick, S.M., Diaz, F., Zerfas, P.M., Barca, E., Sudderth, J., DeBerardinis, R.J., Covian-Garcia, R., Balaban, R.S., DiMauro, S., McGuire, P.J. Cytochrome c oxidase (COX) activity is a metabolic checkpoint that regulates cell fate decisions during T-cell activation and differentiation. Cell Metab 25, 1254 - 1268. 2017. [PubMed]

Tarasenko, T.N., McGuire, P.J. The liver is a metabolic and immunologic organ: a reconsideration of metabolic decompensation due to infection in inborn errors of metabolism. Mol Genet Metab, 121: 283-88. PMCID: PMC5553615. 2017. [PubMed]

Kapnick, S.M., Pacheco, S.E., McGuire, P.J. The emerging role of immune dysfunction in mitochondrial diseases as a paradigm for understanding immunometabolism. Metabolism, [epub ahead of print]. 2017. [PubMed

Last Updated: February 26, 2018