S Surendran

Summary

Affiliation: University of Texas Medical Branch
Country: USA

Publications

  1. ncbi request reprint Aspartoacylase gene knockout results in severe vacuolation in the white matter and gray matter of the spinal cord in the mouse
    Sankar Surendran
    Department of Pediatrics, The University of Texas Medical Branch, Childrens Hospital, 301 University Boulevard, Galveston, TX 77555 0359, USA
    Neurobiol Dis 18:385-9. 2005
  2. ncbi request reprint Expression of calpastatin, minopontin, NIPSNAP1, rabaptin-5 and neuronatin in the phenylketonuria (PKU) mouse brain: possible role on cognitive defect seen in PKU
    Sankar Surendran
    Department of Internal Medicine, The University of Texas Medical Branch, Galveston, TX 77555 0632, USA
    Neurochem Int 46:595-9. 2005
  3. ncbi request reprint Aspartoacylase gene knockout in the mouse: impact on reproduction
    Sankar Surendran
    Department of Internal Medicine, The University of Texas Medical Branch, Galveston, TX 77555, USA
    Reprod Toxicol 20:281-3. 2005
  4. ncbi request reprint Altered expression of myocilin in the brain of a mouse model for phenylketonuria (PKU)
    Sankar Surendran
    Department of Internal Medicine, The University of Texas Medical Branch, Galveston, TX 77555 0632, USA
    Neurosci Lett 382:323-6. 2005
  5. ncbi request reprint Altered expression of neuronal nitric oxide synthase in the duodenum longitudinal muscle-myenteric plexus of obesity induced diabetes mouse: implications on enteric neurodegeneration
    Sankar Surendran
    Department of Internal Medicine, The University of Texas Medical Branch, Galveston, TX 77555, USA
    Biochem Biophys Res Commun 338:919-22. 2005
  6. ncbi request reprint Upregulation of aspartoacylase activity in the duodenum of obesity induced diabetes mouse: implications on diabetic neuropathy
    Sankar Surendran
    Department of Internal Medicine, The University of Texas Medical Branch, Galveston, TX, USA
    Biochem Biophys Res Commun 345:973-5. 2006
  7. ncbi request reprint Possible role of prostaglandin E2 in human amniotic epithelial cell death: an in vitro study
    S Surendran
    Department of Pediatrics, Childrens Hospital, University of Texas Medical Branch, Galveston 77555 0359, USA
    Inflamm Res 50:483-5. 2001
  8. ncbi request reprint Possible role of fas antigen (CD 95) in human amniotic epithelial cell death: an in vitro study
    S Surendran
    National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, 187 8502, Japan
    Cell Biol Int 25:485-8. 2001
  9. ncbi request reprint Expression of glutamate transporter, GABRA6, serine proteinase inhibitor 2 and low levels of glutamate and GABA in the brain of knock-out mouse for Canavan disease
    Sankar Surendran
    Departments of Pediatrics, Children s Hospital, The University of Texas Medical Branch, Galveston, TX 77555 0359, USA
    Brain Res Bull 61:427-35. 2003
  10. ncbi request reprint Abnormal expression of genes associated with development and inflammation in the heart of mouse maternal phenylketonuria offspring
    R Matalon
    Department of Pediatrics, The University of Texas Medical Branch UTMB, Galveston, Texas 77555 0632, USA
    Int J Immunopathol Pharmacol 18:557-65. 2005

Collaborators

Detail Information

Publications35

  1. ncbi request reprint Aspartoacylase gene knockout results in severe vacuolation in the white matter and gray matter of the spinal cord in the mouse
    Sankar Surendran
    Department of Pediatrics, The University of Texas Medical Branch, Childrens Hospital, 301 University Boulevard, Galveston, TX 77555 0359, USA
    Neurobiol Dis 18:385-9. 2005
    ....
  2. ncbi request reprint Expression of calpastatin, minopontin, NIPSNAP1, rabaptin-5 and neuronatin in the phenylketonuria (PKU) mouse brain: possible role on cognitive defect seen in PKU
    Sankar Surendran
    Department of Internal Medicine, The University of Texas Medical Branch, Galveston, TX 77555 0632, USA
    Neurochem Int 46:595-9. 2005
    ..These changes in the PKU mouse brain are likely to contribute cognitive impairment seen in the PKU mouse, if documented also in patients with PKU...
  3. ncbi request reprint Aspartoacylase gene knockout in the mouse: impact on reproduction
    Sankar Surendran
    Department of Internal Medicine, The University of Texas Medical Branch, Galveston, TX 77555, USA
    Reprod Toxicol 20:281-3. 2005
    ..KO/KO mothers mated with KO/+ males showed fetal death. KO/KO mothers produced fewer offspring compared to KO/+ mothers. These data suggest that ASPA is necessary for normal reproduction and postnatal survival...
  4. ncbi request reprint Altered expression of myocilin in the brain of a mouse model for phenylketonuria (PKU)
    Sankar Surendran
    Department of Internal Medicine, The University of Texas Medical Branch, Galveston, TX 77555 0632, USA
    Neurosci Lett 382:323-6. 2005
    ..These data suggest that PAH defect alters other genes expression likely to contribute neurophysiological abnormalities seen in the mouse, if documented also in patients with PKU...
  5. ncbi request reprint Altered expression of neuronal nitric oxide synthase in the duodenum longitudinal muscle-myenteric plexus of obesity induced diabetes mouse: implications on enteric neurodegeneration
    Sankar Surendran
    Department of Internal Medicine, The University of Texas Medical Branch, Galveston, TX 77555, USA
    Biochem Biophys Res Commun 338:919-22. 2005
    ..In addition, reduced expression of nNOS is likely to contribute to apoptosis seen in the enteric neurons of male type 2 diabetic mice...
  6. ncbi request reprint Upregulation of aspartoacylase activity in the duodenum of obesity induced diabetes mouse: implications on diabetic neuropathy
    Sankar Surendran
    Department of Internal Medicine, The University of Texas Medical Branch, Galveston, TX, USA
    Biochem Biophys Res Commun 345:973-5. 2006
    ....
  7. ncbi request reprint Possible role of prostaglandin E2 in human amniotic epithelial cell death: an in vitro study
    S Surendran
    Department of Pediatrics, Childrens Hospital, University of Texas Medical Branch, Galveston 77555 0359, USA
    Inflamm Res 50:483-5. 2001
    ..In this study, the role of prostaglandin E2 (PGE2) in HAE cells in vitro was examined...
  8. ncbi request reprint Possible role of fas antigen (CD 95) in human amniotic epithelial cell death: an in vitro study
    S Surendran
    National Institute of Neuroscience, National Center of Neurology and Psychiatry, Kodaira, Tokyo, 187 8502, Japan
    Cell Biol Int 25:485-8. 2001
    ..Apoptotic cell death induced by this antibody was significantly increased, although far fewer cells underwent apoptosis, as determined by the TUNEL method. This study suggests that Fas antigen is an important mediator in HAE cell death...
  9. ncbi request reprint Expression of glutamate transporter, GABRA6, serine proteinase inhibitor 2 and low levels of glutamate and GABA in the brain of knock-out mouse for Canavan disease
    Sankar Surendran
    Departments of Pediatrics, Children s Hospital, The University of Texas Medical Branch, Galveston, TX 77555 0359, USA
    Brain Res Bull 61:427-35. 2003
    ..This first report showing abnormal expression of EAAT4, GABRA6, Spi2 combined with lower levels of glutamate and GABA are likely to be associated with the pathophysiology of CD...
  10. ncbi request reprint Abnormal expression of genes associated with development and inflammation in the heart of mouse maternal phenylketonuria offspring
    R Matalon
    Department of Pediatrics, The University of Texas Medical Branch UTMB, Galveston, Texas 77555 0632, USA
    Int J Immunopathol Pharmacol 18:557-65. 2005
    ..Our results suggest that altered gene expression affects protein production. These changes are likely involved in the cardiovascular defects seen in the mouse...
  11. ncbi request reprint Canavan disease: a monogenic trait with complex genomic interaction
    Sankar Surendran
    Department of Pediatrics, Children s Hospital, The University of Texas Medical Branch, Galveston, TX 77555 0359, USA
    Mol Genet Metab 80:74-80. 2003
    ..Aspartoacylase gene transfer trial in the mouse brain using adenoassociated virus (AAV) as a vector are encouraging showing improved myelination and decrease in spongy degeneration in the area of the injection and also beyond that site...
  12. ncbi request reprint High level of orexin A observed in the phenylketonuria mouse brain is due to the abnormal expression of prepro-orexin
    Sankar Surendran
    Department of Pediatrics, University of Texas Medical Branch, Galveston, TX, USA
    Biochem Biophys Res Commun 317:522-6. 2004
    ..The metabolic environment in the brain of PKU mouse affects normal expression of other genes possibly to result in pathophysiology seen in the PKU mouse, if documented also in patients with PKU...
  13. ncbi request reprint Mental retardation and hypotonia seen in the knock out mouse for Canavan disease is not due to succinate semialdehyde dehydrogenase deficiency
    S Surendran
    Department of Pediatrics, Children s Hospital, The University of Texas Medical Branch, Galveston, TX 77555 0359, USA
    Neurosci Lett 358:29-32. 2004
    ..The normal SSADH activity in the CD mouse brain suggests that mental retardation and hypotonia seen in the CD mouse is not due to SSADH activity and if documented also in patients with CD...
  14. ncbi request reprint Metabolic changes in the knockout mouse for Canavan's disease: implications for patients with Canavan's disease
    Sankar Surendran
    Department of Pediatrics, The University of Texas Medical Branch, Galveston, TX 77555 0359, USA
    J Child Neurol 18:611-5. 2003
    ..These changes might be similar to what is seen in patients with Canavan's disease and could have therapeutic implications...
  15. ncbi request reprint Malonyl CoA decarboxylase deficiency: C to T transition in intron 2 of the MCD gene
    S Surendran
    Department of Pediatric Cytogenetics, Children's Hospital, University of Texas Medical Branch, Galveston, Texas 77555-0359, USA
    J Neurosci Res 65:591-4. 2001
    ..The data indicate that homozygous mutation in intron 2 disrupt normal splicing of the gene, leading to lower expression of the MCD protein and MCD deficiency...
  16. ncbi request reprint Knock-out mouse for Canavan disease: a model for gene transfer to the central nervous system
    R Matalon
    Department of Pediatrics, Children s Hospital, UTMB Galveston, TX 77555 0359, USA
    J Gene Med 2:165-75. 2000
    ..The lack of an animal model has been a limiting factor in developing vectors for the treatment of CD. This paper reports the successful creation of a knock-out mouse for Canavan disease that can be used for gene transfer...
  17. ncbi request reprint Hyaluronidase increases the biodistribution of acid alpha-1,4 glucosidase in the muscle of Pompe disease mice: an approach to enhance the efficacy of enzyme replacement therapy
    Reuben Matalon
    Department of Pediatrics, The University of Texas Medical Branch, Galveston, TX 77555 0359, USA
    Biochem Biophys Res Commun 350:783-7. 2006
    ..These studies suggest that hyase enhances penetration of enzyme into the tissues including muscle during ERT and therefore hyase pretreatment may be important in treating Pompe disease...
  18. ncbi request reprint Canavan disease: studies on the knockout mouse
    Reuben Matalon
    Department of Pediatrics, University of Texas Medical Branch, Galveston, TX 77555, USA
    Adv Exp Med Biol 576:77-93; discussion 361-3. 2006
    ..Adenoassociated virus mediated gene transfer and stem cell therapy in the knockout mouse are the latest attempts to alter pathophysiology in the CD mouse...
  19. ncbi request reprint Mouse neural progenitor cells differentiate into oligodendrocytes in the brain of a knockout mouse model of Canavan disease
    Sankar Surendran
    Pediatrics Rm 3 350, Department of Pediatrics, Childrens Hospital, The University of Texas Medical Branch, Galveston, TX 77555 0359, USA
    Brain Res Dev Brain Res 153:19-27. 2004
    ..This is the first report to describe the survival, distribution and differentiation of NPCs within the brain of CD mouse and a first step toward the potential clinical use of cell therapy to treat CD...
  20. ncbi request reprint Adeno-associated virus-mediated aspartoacylase gene transfer to the brain of knockout mouse for canavan disease
    Reuben Matalon
    Department of Pediatrics, Galveston, Texas 77555 0359, USA
    Mol Ther 7:580-7. 2003
    ..MRS revealed that in vivo NAA levels were also reduced. These data indicate that rAAV-mediated ASPA delivery may be an interesting avenue for the treatment of CD...
  21. ncbi request reprint Future role of large neutral amino acids in transport of phenylalanine into the brain
    Reuben Matalon
    Department of Pediatrics, University of Texas Medical Branch, Galveston, Texas, USA
    Pediatrics 112:1570-4. 2003
    ..Mutant PKU mice produced by exposure to N-ethyl-N'-nitrosourea (ENU) were used to examine the effect of large neutral amino acid (LNAA) supplementation on brain and blood phenylalanine (Phe)...
  22. ncbi request reprint Aspartoacylase deficiency does not affect N-acetylaspartylglutamate level or glutamate carboxypeptidase II activity in the knockout mouse brain
    Sankar Surendran
    Department of Pediatrics, Childrens Hospital, The University of Texas Medical Branch, Rm 3 350, Galveston, TX 77555 0359, USA
    Brain Res 1016:268-71. 2004
    ..These data suggest that ASPA deficiency does not affect the NAAG or GCP II level in the knockout mouse brain, if documented also in patients with CD...
  23. ncbi request reprint Upregulation of N-acetylaspartic acid alters inflammation, transcription and contractile associated protein levels in the stomach and smooth muscle contractility
    Sankar Surendran
    Department of Pediatrics, University of Texas Health Science Center, Houston, TX 77030, USA
    Mol Biol Rep 36:201-6. 2009
    ..These studies suggest that NAA alters proteins levels and smooth muscle contractility and these changes likely to contribute to gastrointestinal disorder seen in these diseases...
  24. ncbi request reprint Molecular basis of Canavan's disease: from human to mouse
    Sankar Surendran
    Department of Pediatrics, The University of Texas Medical Branch, Galveston, TX 77555 0359, USA
    J Child Neurol 18:604-10. 2003
    ..These developments have afforded new tools for research in the attempts to understand the pathophysiology of Canavan's disease, design new therapies, and explore methods for gene transfer to the central nervous system...
  25. ncbi request reprint High levels of orexin A in the brain of the mouse model for phenylketonuria: possible role of orexin A in hyperactivity seen in children with PKU
    Sankar Surendran
    Department of Pediatrics, University of Texas Medical Branch, Galveston, Texas 77555 0359, USA
    Neurochem Res 28:1891-4. 2003
    ..This is the first report of increased level of orexin in the PKU mouse brain. Hyperactivity is commonly observed in children with PKU; thus these findings suggest that orexin A is a contributing factor for the hyperactivity...
  26. ncbi request reprint Mild elevation of N-acetylaspartic acid and macrocephaly: diagnostic problem
    Sankar Surendran
    Department of Pediatrics, University of Texas Medical Branch, Galveston, Texas 77555 0359, USA
    J Child Neurol 18:809-12. 2003
    ..Expression of the mutation on exon 6 showed normal aspartoacylase activity. These data suggest that expression of the mutation may help to understand the enzyme defect in a patient with slightly increased N-acetylaspartic acid excretion...
  27. ncbi request reprint Biopterin responsive phenylalanine hydroxylase deficiency
    Reuben Matalon
    Department of Pediatrics and Microbiology, University of Texas Medical Branch, Galveston, Texas 77555, USA
    Genet Med 6:27-32. 2004
    ..A pilot study was undertaken to examine the response to 6-R-l-erythro-5,6,7,8-tetrahydrobiopterin (BH4) in patients with atypical and classical PKU...
  28. ncbi request reprint Absence-like and tonic seizures in aspartoacylase/attractin double-mutant mice
    Hiroshi Gohma
    Institute of Laboratory Animals, Graduate School of Medicine, Kyoto University, Kyoto, Japan
    Exp Anim 56:161-5. 2007
    ....
  29. ncbi request reprint Upregulation of aspartoacylase seen in diabetes is due to advanced glycation end-products
    Sankar Surendran
    Med Hypotheses 68:926. 2007
  30. pmc Defective N-acetylaspartate catabolism reduces brain acetate levels and myelin lipid synthesis in Canavan's disease
    Chikkathur N Madhavarao
    Department of Anatomy, Physiology, and Genetics, Uniformed Services University of the Health Sciences, 4301 Jones Bridge Road, Bethesda, MD 20814, USA
    Proc Natl Acad Sci U S A 102:5221-6. 2005
    ....
  31. ncbi request reprint Does ASPA gene mutation in Canavan disease alter oligodendrocyte development? A tissue culture study of ASPA KO mice brain
    Shalini Kumar
    Mental Retardation Research Center, Department of Neurobiology and Psychiatry, Brain Research Institute, David Geffen School of Medicine, University of California, Los Angeles, USA
    Adv Exp Med Biol 576:175-82; discussion 361-3. 2006
  32. ncbi request reprint Fas (CD95) alters neuronal nitric oxide synthase expression to contribute in diabetic gastroparesis
    Sankar Surendran
    Med Hypotheses 68:1427. 2007
  33. ncbi request reprint Trends in enzyme therapy for phenylketonuria
    Woomi Kim
    Department of Molecular and Experimental Medicine, The Scripps Research Institute, 10550 North Torrey Pines Road, La Jolla, CA 92037, USA
    Mol Ther 10:220-4. 2004
    ..Developing stable and functional forms of both enzymes has proven difficult, but recent success in producing polyethylene glycol-modified forms of active and stable PAH shows promise...
  34. ncbi request reprint Mutations in the regulatory domain of phenylalanine hydroxylase and response to tetrahydrobiopterin
    Lin Wang
    Department of Molecular Biology, The Scripps Research Institute, La Jolla, California, USA
    Genet Test 11:174-8. 2007
    ..The level of blood Phe decreased after BH4 treatment in all of the patients. These studies suggest that mutations in the regulatory domain also responded to BH4 even if the patient had classical PKU...
  35. pmc Correction of kinetic and stability defects by tetrahydrobiopterin in phenylketonuria patients with certain phenylalanine hydroxylase mutations
    Heidi Erlandsen
    Department of Molecular Biology, The Scripps Research Institute, 10550 North Torrey Pines Road, La Jolla, CA 92037, USA
    Proc Natl Acad Sci U S A 101:16903-8. 2004
    ..Of fundamental importance is the observation that BH4 appears to increase Phe catabolism if at least one of the two heterozygous mutations has any residual activity remaining...