Reuben Matalon, MD, PhD

  • 7166 Citations
  • 48 h-Index
1966 …2017

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Abnormal expression of genes associated with development and inflammation in the heart of mouse maternal phenylketonuria offspring

Matalon, R., Surendran, S., McDonald, J. D., Okorodudu, A. O., Tyring, S. K., Michals-Matalon, K. & Harris, P., Jan 1 2005, In : International Journal of Immunopathology and Pharmacology. 18, 3, p. 557-565 9 p.

Research output: Contribution to journalArticle

11 Scopus citations

Absence-like and tonic seizures in aspartoacylase/attractin double-mutant mice

Gohma, H., Kuramoto, T., Matalon, R., Surendran, S., Tyring, S., Kitada, K., Sasa, M. & Serikawa, T., Apr 26 2007, In : Experimental Animals. 56, 2, p. 161-165 5 p.

Research output: Contribution to journalArticle

3 Scopus citations

A CHEMICAL METHOD FOR THE ANTENATAL DIAGNOSIS OF MUCOPOLYSACCHARIDOSES

Matalon, R., Dorfman, A. & Nadler, H. L., Apr 8 1972, In : The Lancet. 299, 7754, p. 798-799 2 p.

Research output: Contribution to journalArticle

14 Scopus citations

A chemical method for the antenatal diagnosis of mucopolysaccharidoses.

Matalon, R., Dorfman, A., Nadler, H. L. & Jacobson, C. B., Jan 10 1970, In : Lancet. 1, 7637, p. 83-84 2 p.

Research output: Contribution to journalArticle

Acid mucopolysaccharides in cultured fibroblasts of cystic fibrosis of the pancreas

Matalon, R. & Dorfman, A., Dec 30 1968, In : Biochemical and Biophysical Research Communications. 33, 6, p. 954-958 5 p.

Research output: Contribution to journalArticle

45 Scopus citations

Acid mucopolysaccharides in cultured human fibroblasts.

Matalon, R. & Dorfman, A., Oct 18 1969, In : Lancet. 2, 7625, p. 838-841 4 p.

Research output: Contribution to journalArticle

54 Scopus citations

A complex chromosome rearrangement, der(6)ins(6)(p21.1q25.3q27)inv(6)(p25.3q27), in a child with cleidocranial dysplasia

Northup, J. K., Matalon, R., Lockhart, L. H., Hawkins, J. C. & Velagaleti, G. V. N., Jul 1 2011, In : European Journal of Medical Genetics. 54, 4, p. e394-e398

Research output: Contribution to journalArticle

3 Scopus citations

Acute otitis media and other complications of viral respiratory infection

Chonmaitree, T., Trujillo, R., Jennings, K., Alvarez-Fernandez, P., Patel, J., Loeffelholz, M. J., Nokso-Koivisto, J., Matalon, R., Pyles, R., Miller, A. L. & McCormick, D. P., Apr 1 2016, In : Pediatrics. 137, 4

Research output: Contribution to journalArticle

33 Scopus citations

Acute otitis media severity: Association with cytokine gene polymorphisms and other risk factors

McCormick, D. P., Grady, J. J., Diego, A., Matalon, R., Revai, K., Patel, J. A., Han, Y. & Chonmaitree, T., May 1 2011, In : International Journal of Pediatric Otorhinolaryngology. 75, 5, p. 708-712 5 p.

Research output: Contribution to journalArticle

18 Scopus citations

Allelic mutations in the mucopolysaccharidoses

Leisti, J., Rimoin, D. L., Kaback, M., Shapiro, L. J. & Matalon, R., Dec 1 1976, In : Birth Defects: Original Article Series. 12, 6, p. 81-91 11 p.

Research output: Contribution to journalArticle

4 Scopus citations

Altered expression of myocilin in the brain of a mouse model for phenylketonuria (PKU)

Surendran, S., Matalon, D., Tyring, S. K., Rady, P. L., Velagaleti, G. R. V. & Matalon, R., Jul 15 2005, In : Neuroscience Letters. 382, 3, p. 323-326 4 p.

Research output: Contribution to journalArticle

4 Scopus citations

Analysis of the pentafluorobenzoyl derivative of phenylethylamine utilizing negative ion chemical ionization and gas chromatography/mass spectrometry

Gashkoff, P., Matalon, R., Papa, V. & Sebasta, D., Oct 1988, In : Biological Mass Spectrometry. 16, 1-12, p. 327-329 3 p.

Research output: Contribution to journalArticle

5 Scopus citations

A new mucolipidosis with psychomotor retardation, corneal clouding, and retinal degeneration

Newell, F. W., Matalon, R. & Meyer, S., Jan 1 1976, In : Transactions of the American Ophthalmological Society. Vol.73, p. 172-186 15 p.

Research output: Contribution to journalArticle

3 Scopus citations

A new mucolipidosis with psychomotor retardation, corneal clouding, and retinal degeneration

Newell, F. W., Matalon, R. & Meyer, S., Sep 1975, In : American Journal of Ophthalmology. 80, 3 PART 1, p. 440-449 10 p.

Research output: Contribution to journalArticle

28 Scopus citations

A novel mutation in the switch 3 region of G(s)α in a patient with albright hereditary osteodystrophy impairs GDP binding and receptor activation

Warner, D. R., Weng, G., Yu, S., Matalon, R. & Weinstein, L. S., Sep 11 1998, In : Journal of Biological Chemistry. 273, 37, p. 23976-23983 8 p.

Research output: Contribution to journalArticle

55 Scopus citations

A preliminary report of the collaborative study of maternal phenylketonuria in the United States and Canada

Koch, R., Hanley, W., Levy, H., Matalon, R., Rouse, B., Cruz, F. D., Azen, C. & Friedman, E. G., Jul 1 1990, In : Journal of Inherited Metabolic Disease. 13, 4, p. 641-650 10 p.

Research output: Contribution to journalArticle

35 Scopus citations

A single intravenous rAAV injection as late as P20 achieves efficacious and sustained CNS gene therapy in Canavan mice

Ahmed, S. S., Li, H., Cao, C., Sikoglu, E. M., Denninger, A. R., Su, Q., Eaton, S., Liso Navarro, A. A., Xie, J., Szucs, S., Zhang, H., Moore, C., Kirschner, D. A., Seyfried, T. N., Flotte, T. R., Matalon, R. & Gao, G., Dec 2013, In : Molecular Therapy. 21, 12, p. 2136-2147 12 p.

Research output: Contribution to journalArticle

45 Scopus citations

Aspartoacyclase deficiency and N-acetylaspartic aciduria in patients with Canavan disease

Matalon, R., Michals, K., Sebesta, D., Deanching, M., Gashkoff, P. & Casanova, J., Jan 1 1988, In : American Journal of Medical Genetics. 29, 2, p. 463-471 9 p.

Research output: Contribution to journalArticle

297 Scopus citations

Aspartoacylase Deficiency: The Enzyme Defect in Canavan Disease

Matalon, R., Kaul, R., Casanova, J., Michals, K., Johnson, A., Rapin, I., Gashkoff, P. & Deanching, M., Apr 1989, In : Journal of Inherited Metabolic Disease. 12, 2, p. 329-331 3 p.

Research output: Contribution to journalArticle

75 Scopus citations

Aspartoacylase deficiency affects early postnatal development of oligodendrocytes and myelination

Mattan, N. S., Ghiani, C. A., Lloyd, M., Matalon, R., Bok, D., Casaccia, P. & de Vellis, J., Nov 1 2010, In : Neurobiology of Disease. 40, 2, p. 432-443 12 p.

Research output: Contribution to journalArticle

23 Scopus citations

Aspartoacylase deficiency does not affect N-acetylaspartylglutamate level or glutamate carboxypeptidase II activity in the knockout mouse brain

Surendran, S., Ezell, E. L., Quast, M. J., Wei, J., Tyring, S. K., Michals-Matalon, K. & Matalon, R., Aug 6 2004, In : Brain Research. 1016, 2, p. 268-271 4 p.

Research output: Contribution to journalArticle

12 Scopus citations

Aspartoacylase gene knockout in the mouse: Impact on reproduction

Surendran, S., Szucs, S., Tyring, S. K. & Matalon, R., Jan 1 2005, In : Reproductive Toxicology. 20, 2, p. 281-283 3 p.

Research output: Contribution to journalArticle

7 Scopus citations

Aspartoacylase gene knockout results in severe vacuolation in the white matter and gray matter of the spinal cord in the mouse

Surendran, S., Campbell, G. A., Tyring, S. K. & Matalon, R., Mar 2005, In : Neurobiology of Disease. 18, 2, p. 385-389 5 p.

Research output: Contribution to journalArticle

21 Scopus citations

Aspartoacylase gene transfer to the mammalian central nervous system with therapeutic implications for Canavan disease

Leone, P., Janson, C. G., Bilianuk, L., Wang, Z., Sorgi, F., Huang, L., Matalon, R., Kaul, R., Zeng, Z., Freese, A., McPhee, S. W., Mee, E. & During, M. J., Jul 15 2000, In : Annals of Neurology. 48, 1, p. 27-38 12 p.

Research output: Contribution to journalArticle

121 Scopus citations

Association between cytokine gene polymorphisms and risk for upper respiratory tract infection and acute otitis media

Revai, K., Patel, J. A., Grady, J. J., Nair, S., Matalon, R. & Chonmaitree, T., Jul 15 2009, In : Clinical Infectious Diseases. 49, 2, p. 257-261 5 p.

Research output: Contribution to journalArticle

34 Scopus citations

Association of proinflammatory cytokine gene polymorphisms with susceptibility to otitis media

Patel, J. A., Nair, S., Revai, K., Grady, J., Saeed, K., Matalon, R., Block, S. & Chonmaitree, T., Dec 2006, In : Pediatrics. 118, 6, p. 2273-2279 7 p.

Research output: Contribution to journalArticle

59 Scopus citations

A therapeutic trial of amniotic epithelial cell implantation in patients with lysosomal storage diseases

Yeager, A. M., Singer, H. S., Buck, J. R., Matalon, R., Brennan, S., O'Toole, S. O. & Moser, H. W., Dec 1 1985, In : American Journal of Medical Genetics. 22, 2, p. 347-355 9 p.

Research output: Contribution to journalArticle

44 Scopus citations

A Treatment Program for Adolescents With Phenylketonuria

Gleason, L. A., Michals, K., Matalon, R., Langenberg, P. & Kamath, S., Jun 1992, In : Clinical pediatrics. 31, 6, p. 331-335 5 p.

Research output: Contribution to journalArticle

24 Scopus citations

Bimodal occurrence of aspartoacylase in myelin and cytosol of brain

Wang, J., Matalon, R., Bhatia, G., Wu, G., Li, H., Liu, T., Lu, Z. H. & Ledeen, R. W., Apr 1 2007, In : Journal of neurochemistry. 101, 2, p. 448-457 10 p.

Research output: Contribution to journalArticle

12 Scopus citations

Biochemistry and molecular biology of Canavan disease

Matalon, R. & Michals-Matalon, K., Jan 1 1999, In : Neurochemical Research. 24, 4, p. 507-513 7 p.

Research output: Contribution to journalArticle

47 Scopus citations

Biopterin responsive phenylalanine hydroxylase deficiency

Matalon, R., Koch, R., Michals-Matalon, K., Moseley, K., Surendran, S., Tyring, S., Erlandsen, H., Gamez, A., Stevens, R. C., Romstad, A., Møller, L. B. & Guttler, F., Jan 1 2004, In : Genetics in Medicine. 6, 1, p. 27-32 6 p.

Research output: Contribution to journalArticle

52 Scopus citations

Biopterin synthesis defects: Problems in diagnosis

Hoganson, G., Berlow, S., Kaufman, S., Milstien, S., Schuett, V., Matalon, R., Naylor, E. & Seifert, W., Dec 1 1984, In : Pediatrics. 74, 6, p. 1004-1011 8 p.

Research output: Contribution to journalArticle

20 Scopus citations

Blood phenylalanine levels and intelligence of 10-year-old children with PKU in the National Collaborative Study

Michals, K., Azen, C., Acosta, P., Koch, R. & Matalon, R., Jan 1 1988, In : Journal of the American Dietetic Association. 88, 10, p. 1226-1229 4 p.

Research output: Contribution to journalArticle

30 Scopus citations

Canavan disease

Verdura, C., Perez-Pascual, S. R. & Matalon, R., Jan 1 1994, In : International Pediatrics. 9, 3, p. 221-223 3 p.

Research output: Contribution to journalArticle

Canavan disease: Biochemical and molecular studies

Matalon, R., Kaul, R. & Michals, K., Jul 1 1993, In : Journal of Inherited Metabolic Disease. 16, 4, p. 744-752 9 p.

Research output: Contribution to journalArticle

44 Scopus citations

Canavan disease: Mutations among Jewish and non-Jewish patients

Kaul, R., Gao, G. P., Aloya, M., Balamurugan, K., Petrosky, A., Michals, K. & Matalon, R., Jul 19 1994, In : American Journal of Human Genetics. 55, 1, p. 34-41 8 p.

Research output: Contribution to journalArticle

106 Scopus citations

Canavan disease: Diagnosis and molecular analysis

Matalon, R., Jun 1 1997, In : Genetic Testing. 1, 1, p. 21-25 5 p.

Research output: Contribution to journalArticle

23 Scopus citations

Canavan disease

Matalon, R., Jan 1 1996, In : Molecular and Chemical Neuropathology. 27, 1, p. 54-57 4 p.

Research output: Contribution to journalArticle

1 Scopus citations

Canavan disease: Molecular basis of aspartoacylase deficiency

Kaul, R., Gao, G. P., Balamurugan, K. & Matalon, R., May 1 1994, In : Journal of Inherited Metabolic Disease. 17, 3, p. 295-297 3 p.

Research output: Contribution to journalArticle

10 Scopus citations

Canavan disease: From spongy degeneration to molecular analysis

Matalon, R., Michals, K. & Kaul, R., Oct 1995, In : The Journal of Pediatrics. 127, 4, p. 511-517 7 p.

Research output: Contribution to journalArticle

98 Scopus citations

Canavan Disease: Genomic Organization and Localization of Human ASPA to 17p13-ter and Conservation of the ASPA Gene during Evolution

Kaul, R., Balamurugan, K., Gao, G. P. & Matalon, R., May 15 1994, In : Genomics. 21, 2, p. 364-370 7 p.

Research output: Contribution to journalArticle

52 Scopus citations

Carrier detection for sanfilippo A syndrome

Matalon, R., Deanching, M., Marback, R. & Michals, K., Jun 1 1988, In : Journal of Inherited Metabolic Disease. 11, 2, p. 158-160 3 p.

Research output: Contribution to journalArticle

9 Scopus citations

Characterization of mouse and human GTP cyclohydrolase I genes. Mutations in patients with GTP cyclohydrolase I deficiency

Ichinose, H., Ohye, T., Matsuda, Y., Hori, T. A., Blau, N., Burlina, A., Rouse, B., Matalon, R., Fujita, K. & Nagatsu, T., Jan 1 1995, In : Journal of Biological Chemistry. 270, 17, p. 10062-10071 10 p.

Research output: Contribution to journalArticle

Open Access
133 Scopus citations

Children of fathers with phenylketonuria: An international survey

Fisch, R. O., Matalon, R., Weisberg, S. & Michals, K., May 1991, In : The Journal of Pediatrics. 118, 5, p. 739-741 3 p.

Research output: Contribution to journalArticle

5 Scopus citations

Cloning of the human aspartoacylase cDNA and a common missense mutation in Canavan disease

Kaul, R., Gao, G. P., Balamurugan, K. & Matalon, R., Oct 1993, In : Nature Genetics. 5, 2, p. 118-123 6 p.

Research output: Contribution to journalArticle

174 Scopus citations
7 Scopus citations

Comprehensive analysis of genetic polymorphisms in the interleukin-10 promoter: Implications for immune regulation in specific ethnic populations

Rady, P. L., Matalon, R., Grady, J., Smith, E. M., Hudnall, S. D., Kellner, L. H., Nitowsky, H., Tyring, S. K. & Hughes, T. K., Jun 1 2004, In : Genetic Testing. 8, 2, p. 194-203 10 p.

Research output: Contribution to journalArticle

25 Scopus citations

Congenital heart disease in maternal phenylketonuria: Report from the Maternal PKU Collaborative Study

Levy, H. L., Guldberg, P., Güttler, F., Hanley, W. B., Matalon, R., Rouse, B. M., Trefz, F., Azen, C., Allred, E. N., De La Cruz, F. & Koch, R., Jan 1 2001, In : Pediatric Research. 49, 5, p. 636-642 7 p.

Research output: Contribution to journalArticle

Open Access
68 Scopus citations

Congenital heart disease in maternal phenylketonuria: Effects of blood phenylalanine and nutrient intake

Matalon, K. M., Acosta, P., Azen, C. & Matalon, R., Jun 8 1999, In : Mental Retardation and Developmental Disabilities Research Reviews. 5, 2, p. 122-124 3 p.

Research output: Contribution to journalArticle

17 Scopus citations

Correction of kinetic and stability defects by tetrahydrobiopterin in phenylketonuria patients with certain phenylalanine hydroxylase mutations

Erlandsen, H., Pey, A. L., Gámez, A., Pérez, B., Desviat, L. R., Aguado, C., Koch, R., Surendran, S., Tyring, S., Matalon, R., Scriver, C. R., Ugarte, M., Martínez, A. & Stevens, R. C., Nov 30 2004, In : Proceedings of the National Academy of Sciences of the United States of America. 101, 48, p. 16903-16908 6 p.

Research output: Contribution to journalArticle

138 Scopus citations