G T Berry

Summary

Affiliation: The Children's Hospital of Philadelphia
Country: USA

Publications

  1. ncbi request reprint Neonatal hypoglycaemia in severe succinyl-CoA: 3-oxoacid CoA-transferase deficiency
    G T Berry
    Department of Pediatrics, University of Pennsylvania School of Medicine, The Children s Hospital of Philadelphia, 19104, USA
    J Inherit Metab Dis 24:587-95. 2001
  2. ncbi request reprint Evidence for alternate galactose oxidation in a patient with deletion of the galactose-1-phosphate uridyltransferase gene
    G T Berry
    Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Mol Genet Metab 72:316-21. 2001
  3. ncbi request reprint Galactose metabolism by the mouse with galactose-1-phosphate uridyltransferase deficiency
    C Ning
    Division of Biochemical Development and Molecular Diseases, The Children s Hospital of Philadelphia, Pennsylvania 19104, USA
    Pediatr Res 48:211-7. 2000
  4. ncbi request reprint The structural organization of the human Na+/myo-inositol cotransporter (SLC5A3) gene and characterization of the promoter
    J J Mallee
    Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, USA
    Genomics 46:459-65. 1997
  5. ncbi request reprint Elevation of erythrocyte redox potential linked to galactonate biosynthesis: elimination by Tolrestat
    G T Berry
    Department of Pediatrics, University of Pennsylvania School of Medicine, The Children s Hospital of Philadelphia, 19104, USA
    Metabolism 47:1423-8. 1998
  6. ncbi request reprint Murine chromosome 16 telomeric region, homologous with human chromosome 21q22, contains the osmoregulatory Na(+)/myo-inositol cotransporter (SLC5A3) gene
    K E McVeigh
    Department of Pediatrics, The Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4399, USA
    Cytogenet Cell Genet 88:153-8. 2000
  7. ncbi request reprint Galactose metabolism in mice with galactose-1-phosphate uridyltransferase deficiency: sucklings and 7-week-old animals fed a high-galactose diet
    C Ning
    Department of Pediatrics, University of Pennsylvania, Philadelphia, Pennsylvania 19104 4318, USA
    Mol Genet Metab 72:306-15. 2001
  8. ncbi request reprint Multiple comparison of primary structure of the osmoregulatory Na+/myo-inositol cotransporter from bovine, human, and canine species
    J J Mallee
    Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    Mamm Genome 7:252. 1996
  9. ncbi request reprint In vivo evidence of brain galactitol accumulation in an infant with galactosemia and encephalopathy
    G T Berry
    Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    J Pediatr 138:260-2. 2001
  10. ncbi request reprint Proton magnetic resonance spectroscopy of brain metabolites in galactosemia
    Z J Wang
    Department of Radiology, The Children s Hospital of Philadelphia, PA 19104, USA
    Ann Neurol 50:266-9. 2001

Collaborators

Detail Information

Publications11

  1. ncbi request reprint Neonatal hypoglycaemia in severe succinyl-CoA: 3-oxoacid CoA-transferase deficiency
    G T Berry
    Department of Pediatrics, University of Pennsylvania School of Medicine, The Children s Hospital of Philadelphia, 19104, USA
    J Inherit Metab Dis 24:587-95. 2001
    ..The presence of hypoglycaemia does not exclude the diagnosis of SCOT deficiency in infancy...
  2. ncbi request reprint Evidence for alternate galactose oxidation in a patient with deletion of the galactose-1-phosphate uridyltransferase gene
    G T Berry
    Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Mol Genet Metab 72:316-21. 2001
    ..Further knowledge of this alternate galactose oxidative route and its regulation may aid in formulating new strategies for the treatment of galactosemia...
  3. ncbi request reprint Galactose metabolism by the mouse with galactose-1-phosphate uridyltransferase deficiency
    C Ning
    Division of Biochemical Development and Molecular Diseases, The Children s Hospital of Philadelphia, Pennsylvania 19104, USA
    Pediatr Res 48:211-7. 2000
    ....
  4. ncbi request reprint The structural organization of the human Na+/myo-inositol cotransporter (SLC5A3) gene and characterization of the promoter
    J J Mallee
    Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, USA
    Genomics 46:459-65. 1997
    ..It is localized to chromosome 21q22.1. An overexpression of the SLC5A3 gene deserves consideration as a factor in the pathophysiology of Down syndrome...
  5. ncbi request reprint Elevation of erythrocyte redox potential linked to galactonate biosynthesis: elimination by Tolrestat
    G T Berry
    Department of Pediatrics, University of Pennsylvania School of Medicine, The Children s Hospital of Philadelphia, 19104, USA
    Metabolism 47:1423-8. 1998
    ..A functioning galactonate pathway is a route of galactose disposal in patients with GALT deficiency, but by altering the cellular redox potential, it may also contribute to galactose toxicity...
  6. ncbi request reprint Murine chromosome 16 telomeric region, homologous with human chromosome 21q22, contains the osmoregulatory Na(+)/myo-inositol cotransporter (SLC5A3) gene
    K E McVeigh
    Department of Pediatrics, The Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 4399, USA
    Cytogenet Cell Genet 88:153-8. 2000
    ..An increased Slc5a3 copy number may explain the increased levels of myo-inositol in the brains of trisomy 16 mice and the increased rate of transport of myo-inositol into cultured neurons derived from trisomy 16 mice...
  7. ncbi request reprint Galactose metabolism in mice with galactose-1-phosphate uridyltransferase deficiency: sucklings and 7-week-old animals fed a high-galactose diet
    C Ning
    Department of Pediatrics, University of Pennsylvania, Philadelphia, Pennsylvania 19104 4318, USA
    Mol Genet Metab 72:306-15. 2001
    ..Absence of GALT appears necessary but insufficient to produce human galactosemic phenotype...
  8. ncbi request reprint Multiple comparison of primary structure of the osmoregulatory Na+/myo-inositol cotransporter from bovine, human, and canine species
    J J Mallee
    Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    Mamm Genome 7:252. 1996
  9. ncbi request reprint In vivo evidence of brain galactitol accumulation in an infant with galactosemia and encephalopathy
    G T Berry
    Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    J Pediatr 138:260-2. 2001
    ..Using in vivo proton magnetic resonance spectroscopy, we detected approximately 8 mmol galactitol per kilogram of brain tissue, an amount potentially relevant to the pathogenesis of brain edema...
  10. ncbi request reprint Proton magnetic resonance spectroscopy of brain metabolites in galactosemia
    Z J Wang
    Department of Radiology, The Children s Hospital of Philadelphia, PA 19104, USA
    Ann Neurol 50:266-9. 2001
    ..The results demonstrate that a markedly elevated brain galactitol level may be present only in newborn infants with galactosemia who exhibit massive urinary galactitol excretion...
  11. ncbi request reprint The 21q22.1 STS marker, VN02 (EST00541 cDNA), is part of the 3' sequence of the human Na+/myo-inositol cotransporter (SLC5A3) gene
    G T Berry
    Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    Cytogenet Cell Genet 73:77-8. 1996
    ..1 marker VN02, an expressed sequence tag (EST00541). Through DNA sequence analysis, it was determined that this STS marker if part of the 3' untranslated region of the SLC5A3 gene...