HHP Directory

Dr. Kimberlee Matalon

Associate Professor

Office Number: 104E GAR
Phone: 713-743-4117
Fax: 713-743-9860

Email: Kmatalon@uh.edu

Mailing Address:
3855 Holman St., Rm 104 Garrison
Houston, TX 77204-6015


Research Interests

Metabolic diseases, metabolism, and nutrition

Recent Publications and Research Activity

Michals, K., Matalon, R., and Wong, P.W.K.: Dietary Treatment of Tyrosinemia, Type 1. J.Am.Diet.Assoc., 73:507-514, 1978.

Ziyai, F., Wong, P.W.K., Justice, P., and Michals, K.: Protein Induced Hypoglycemia in a Phenylketonuria Patient. J. Pediatr. 92:681-682, 1978.

Melnick, C., Michals, K., and Matalon, R.: Language Acquisition in Children with Phenylketonuria and Normal Intelligence. J. Pediatr. Vol. 98(2):262-272, 1981.

Matalon, R., Michals, K., Matthews, D., and Bier, D.: The Use of Deuterated Phenylalanine for the in Vivo Assay of Phenylalanine Hydroxylase Activity in Children. J. Inher. Metab. Dis. 5:17-19, 1982. Presented.

Matalon, R., Michals, K., Naidu, S., and Hughes, J.: Treatment of Nonketotic Hyperglycinemia with Diazepam, Choline and Folic Acid. J. Inher. Metab. Dis. 5:3-5, 1982. Presented.

Matalon, R., Michals, K., Lee, C.L. and Nixon, J. C.: Screening for Biopterin Defects in Newborns with Phenylketonuria (PKU) and Other Hyperphenylalaninemias. Ann. Cl. Lab. Sci. 12:411-414, 1982. Presented.

Fisher, E., Weiss, E. B., Michals, K., Dubrow, I. W., Hastrieter, A. R. and Matalon, R.: Spontaneous Chylothorax in Noonan's Syndrome. Eur. J. Pediatr. 138:282-284, 1982. Matalon, R., Naidu, S., Hughes, J., and Michals, K.: Nonketotic Hyperglycinemia: Treatment with Diazepam-A Competitor for Glycine Receptors. Pediatrics. 71:581-584, 1983.

Michals, K., Pringle, K., Pang, E.M. and Matalon, R.: Glucose-6-phosphatase as a Marker for Tumors of Liver and Kidney Origin. Biochem. Med. 30, 127-130, 1983.

Matalon, R., Stumpf, D., Michals, K., Hart, R., Parks, G., and Goodman, S.: Lipoamide Dehydrogenase (LAD) Deficiency with Primary Lactic Acidosis: Favorable Response to Treatment with Oral Lipoic Acid. J. Pediatr. 104(1): 65-69, 1984.

Matalon, R., and Michals, K.: Gluconeogenic Enzymes in the Human Placenta. J. Inher. Metab. Dis., 7:179-181, 1984.

Schuett, V.E., Brown, E.S., and Michals, K.: Reinstitution of Diet Therapy in PKU Patients from Twenty-two US Clinics. AJPH 75(1):39-42, 1985.

Michals, K., Dominik, M., Schuett, V., Brown, E., and Matalon, R.: Return to Diet Therapy in Patients with Phenylketonuria. J. Pediatr. 106:933-936, 1985.

Michals, K. and Matalon, R.: Phenylalanine Metabolites, Attention Span and Hyperactivity, Am. J. Clin. Nutr. 42:361-365, 1985.

Murphy, J.V., Thome, L.M., Michals, K., and Matalon, R.: Folic Acid Responsive Rages, Seizures and Homocytinuria. J. Inher. Metab. Dis. 8:109-110, 1985.

Michals, K., Lopus, M., Gashkoff, P., Matalon, R.: Phenylalanine Metabolites in Treated Phenylketonuric Children. J. Inher. Metab. Dis. 9:212-214, 1986.

Matalon, R., Michals, K., and Gleason, L.: Maternal PKU: Strategies for Dietary Treatment and Monitoring Compliance. Annals N.Y. Acad. Sci. Vol. 477:223-230, 1986.

Michals, K.: Diet Therapy for Phenylketonuria. New Challenges. Top. Clin. Nutr. 2(3):40-43, 1987.

Michals, K., Lopus, M., Gashkoff, P. and Matalon, R.: Phenylalanine Metabolites as Indicators of Dietary Compliance in Children with PKU. Biochem. Med. 39:18-23, 1988.

Michals, K., Azen, C., Koch, R., Acosta, P. and Matalon, R.: Blood Phenylalanine Levels and Intelligence of Ten Year Old Children with Phenylketonuria in the National Collaborative Study. J. Am. Diet. Assoc. 88:1226-1229, 1988.

Matalon, R., Michals, K., Sebesta, D., Deanching, M., Gashkoff, P., and Casanova, J.: Aspartoacylase Deficiency and N-Acetylaspartic Aciduria in Patients with Canavan Disease. Am. J. Med. Gen., 29:463-471, 1988.

Matalon, R., Deanching, M., Marback, R., and Michals, K.: Carrier Detection for Sanfilippo A Syndrome. J. Inher. Metab. Dis. 11:158-160, 1988.

Matalon, R., Kaul, R., Casanova, J., Michals, K., Johnson, A., Rapin, I., Gashkoff, P. and Deanching, M. SSIEM Award. Aspartoacylase Deficiency: The Enzyme Defect in Canavan Disease. J. Inher. Metab Dis. 12:2, 329-331,1989.

Matalon, R., Michals, K., Blau, N., and Rouse, B. Hyperphenylalaninemia due to Inherited Deficiencies of Tetrahydrobiopterin. Advances in Pediatrics. 36:67-82, 1989.

Wolff, J.A., Barshop, B., Nyhan, W.L., Leslie, J., Seegmiller, J.E., Gruber, H., Garst, M., Winter, S., Michals, K. and Matalon, R. Effects of Ascorbic Acid in Alkaptonuria: Alterations in Benzoquinone Acetic Acid and an Ontogenic Effect in Infancy. Ped. Res. 26:2, 140-144,1989.

Matalon, R., Michals, K., Kaul, R. and Mafee, M. Spongy Degeneration of the Brain, Canavan Disease. Int. Pediatrics, 121-124, 1990.

Fisch, O.R., Matalon, R., Weisberg, S., and Michals, K.: Children of Fathers with Phenylketonuria: An International Survey. J. Pediatr. 118, 739-741, 1991.

Kaul R., Michals K., Casanova J. and Matalon R.: The role of n-acetylaspartic acid in brain metabolism and the pathogenesis in Canavan disease. Int. Pediatrics, 6: 40-43, 1991.

Matalon R., Michals K., Kaul R.: Defects in the Oxidation of Fatty Acids Int. Pediatrics, 6: 126-128, 1991.

Matalon R., and Michals K.: Phenylketonuria: Screening, treatment and maternal PKU. Clin. Biochem. 24:337-341, 1991.

Matalon R., Michals K., Azen C., Friedman E.G., Koch R., Wenz E., Levy H., Rohr F., Rouse B., Castiglioni L., Hanley W., Austin V., De la Cruz, F.: Maternal PKU collaborative study (MPKUCS): The effect of nutrient intake on pregnancy outcome. J. Inher. Metab. Dis. 14: 371-374, 1991.

Matalon R., Michals K., Gashkoff P., Kaul R.: Prenatal Diagnosis of Canavan Disease. J. Inher. Metab. Dis. 15: 392-394, 1992.

Gleason LA., Michals K., Matalon R., Langenberg P., and Kamath S.: A Treatment Program for Adolescents with Phenylketonuria. Clin. Ped., 31: 331-335, 1992.

Matalon R., Kaul R., and Michals K.: Biochemical and Molecular Studies of Canavan Disease. J. Japanese Soc. Inher. Metab. Dis. 9: 13-19, 1993.

Matalon R., Michals K., Kaul R., Whitman V., Rodriguez-Novo J., Goodman S. and Thorburn D.: Malonic Aciduria and Cardiomyopathy. J. Inher. Metab. Disease 16: 471-573, 1993.

Matalon R., Kaul R., and Michals, K., Canavan Disease: Biochemical and Molecular Studies. J. Inher. Metab. Disease 16: 744-752, 1993.

Matalon R., Michals K., Azen C., Friedman E., Koch R., Rouse B, Hanley W.B. and de la Cruz, F.: "Maternal PKU Collaborative Study: Pregnancy Outcome and Post-Natal Head Growth" J. Inher. Metab. Dis. 17: 353-355, 1994.

Kaul R., Matalon R., Allen R., Fisch R.O., Michals K., Petrosky, A. and Sullivan D.: "Frequency of 12 Mutations in 114 Children with Phenylketonuria in the Midwest Region of the USA" J. Inher. Metab. Dis. 17: 356-358, 1994.

Kaul, R., Gao, G.P., Aloya, M., Balamurugan, K., Petrosky, A., Michals, K., and Matalon, R.: Canavan Disease: Mutations Among Jewish and Non-Jewish Patients. Am. J. Hum. Genet 55: 34-41, 1994.

Levy, H., Goss, B., Sullivan, D., Michals-Matalon, K., Dobbs, J.M., Goldberg, P., and Guttler, F. "Maternal Mild Hyperphenylalaninemia: Results of treated and untreated pregnancies in two sisters." J. Pediatr. 125: 467-469, 1994.

Kaul, R., Gao, G.P., Michals, K., Whelan, D.T., Levin, S., and Matalon, R.: A novel (cys 152>arg) missense mutation in Arab patients with Canavan Disease. Human Mutation: 5: 269-271, 1995.

Matalon, R., Kaul, R., Gao, G.P., Michals, K., Gray, R.G.F., Bennett-Briton, S., Norman, A., Smith, M., and Jakobs, C.: Prenatal Diagnosis For Canavan Disease: The Use of DNA Markers: J. Inher. Metab. Dis. 18: 215-217, 1995.

Matalon, R., Michals, K. and Kaul R. Canavan Disease: From spongy degeneration to molecular analysis. J. Pediatr. 127: 511-517, 1995.

Michals, K. Acosta, P.B., Austin, V., Castiglioni, L., Rohr, F., Wenz, E., and Azen,C.: "Nutrition and Reproductive Outcome in Maternal Phenylketonuria" Eur. J. Pediatr. 155:S165-168, 1996.

Fisch, R.O., Matalon, R., Weisberg, S., and Michals, K. Phenylketonuria: Current Dietary Treatment Practices in The United States and Canada. J. Am. Coll. Nutr. 16, 147-151, 1997.

Matalon, R. and Michals-Matalon, K. Molecular Biology of Canavan Disease. Eur. J Pediatr. Neuro. 2: 69-76 1998.

Michals-Matalon, K., Acosta, P.B., Matalon, R.: Congenital Heart Disease in Maternal Phenylketonuria: Effects of Blood Phenylalanine and Nutrient Intake. Ment. Retar. Dev. Dis. Res. Rev. 5:122-124, 1998.

Matalon, R., Michals-Matalon, K. Recent Advances in Canavan Disease. Advances in Pediatrics. 46:493-506, 1999.

Michals-Matalon, K., Matalon, R. Biochemistry and Molecular Biology of Canavan disease. Neurochem Res 24:507-513, 1999.

Matalon R, Michals-Matalon K. Prenatal diagnosis in Canavan disease. Prenat Diagn 19:669-670, 1999.

Koch R, Friedman E, Azen C, Hanley, W, Levy H, Matalon R, Rouse B, Trefz F, Waisbren S, Michals-Matalon, K., Acosta, P., Guttler, F., Ullrich, K., Platt, L., de la Cruz, F. The International Collaborative Study of Maternal Phenylketonuria. Status Report 1998. Men. Retard. Dev. Dis. Res. 5:117-121, 1999.

Matalon, R., Michals-Matalon, K. Spongy degeneration of the brain, Canavan disease: Biochemical and molecular findings. Frontiers in Bioscience 5:307-311, 2000.

Matalon, R., Rady, P.L., Platt, K.A., Skinner, H.B., Quast, M.J., Campbell, G.A., Matalon, K., Ceci, J.D., Tyring, S.K., Nehls, M., Surendran, S., Wei, J., Ezell, E.L., Szucs, S. Knock-out mouse for Canavan disease: A Model for Gene Transfer to the Central Nervous System. J. Gene Med. Vol. 2, pp: 165-175, 2000.

Matalon R, Michals-Matalon K. Spongy degeneration of the brain, Canavan disease: Biochemical and molecular findings. Ped Path Molecular Med 18:471-481, 2000.

Koch R, Friedman E, Azen C, Hanley, W, Levy H, Matalon R, Rouse B, Trefz F, Waisbren S, Michals-Matalon, K., Acosta, P., Guttler, F., Ullrich, K., Platt, L., de la Cruz, F. The International Collaborative Study of Maternal Phenylketonuria. Status Report 1998. Eur J Pediatr 159:161-162, 2000.

Acosta P, Michals-Matalon K, Castiglioni L, Rohr F, Wenz E, Austin V, Funk-Wentsel P, Azen C. Intake of major nutrients by women in the Maternal Phenylketonuria (MPKU) Study and effects on plasma phenylalanine concentration. Am J. Clin Nutr 73:792-796, 2001.

Matalon K, New developments in phenylketonuria. Topics Clin Nutr 16:41-50;2001.

Howell, RR, Matalon R, Michals-Matalon K, et al National Institute of Health Consensus Development Conference Statement: Phenylketonuria: Screening and Management. National Institutes of Health Concensus Development Panel. Pediatrics. 108:4, 2001.

Michals-Matalon K, Platt LD, Acosta P, Azen C, Walla CA. Nutrient Intake and Congenital Heart Disease in Maternal Phenylketonuria. Am J Obstet Gynecol 187:441-444; 2002.

Matalon R, Matalon KM. Canavan disease prenatal diagnosis and genetic counseling. Obstet Gynecol Clin Am 29:297-304; 2002.

Rady, PL, Surendran, S, Vu AT, Hawkins J, Michals-Matalon K, Trying S, Merren J, Kumar AK, Matalon R. Founder Mutation R245H of Sanfilippo Syndrome Type A in the Cayman Islands. Genet Test 6:211-15; 2002.

Surendran, S. Michals-Matalon, K., Krywawych S, Qazi QH, Tuchman R, Rady PL, Tyring SK, Matalon R. DOOR syndrome: Deficiency of E1 component of the 2-oxoglutarate dehydrogenase complex. Am J Med Genet 113:371-4; 2002.

Matalon, R., Surendran, S., Michals Matalon, K., Quast M, Jinga W, Ezell E, Szucs S. Future role of large neutral amino acids in the transport of phenylalanine into the brain. Pediatrics. In press

Michals Matalon, K, Acosta P, Azen C. The role of nutrition in pregnancy with PKU and birth defects. Pediatrics. In press


B.S. in Foods and Nutrition at Northern Illinois University, DeKalb, IL, 1974

Training in Feeding the Handicapped Child at University of Washington, Seattle, WA., 1976

Ph.D. at University of Illinois at Chicago Chicago, IL, 1982