Chester B. Whitley is an American human geneticist and physician whose research accomplishments include significant positive developments in inherited metabolic diseases. Whitley has developed a method of detecting mucopolysaccharide storage diseases[1][2][3][4][5] and made significant advances in the treatment of genetic diseases including the development of enzyme replacement therapies and/or gene therapies for Hurler syndrome, Scheie syndrome, and Hunter syndrome.[6][7] Under his direction, his research laboratory at the University of Minnesota was the first clinical laboratory in North America to offer 'mutation analysis' by DNA sequencing methodology. [year this happened needed] [citation needed]

Additionally, Whitley has made significant contributions in the research and treatment of Phenylketonuria (PKU).[8][9][10][11][12]

Chester B. Whitley
Alma materCarleton College and The University of Minnesota
Known forDiscovery, development and implementation of some enzyme replacement therapies and gene therapies for inherited metabolic disorders
Scientific career
FieldsPediatric Genetics and Metabolism
InstitutionsUniversity of Minnesota
Doctoral advisorRobert J. Desnick

Whitley is a professor in the Department of Pediatrics in the University of Minnesota Medical School in Minneapolis. Additionally, he is a professor in the Department of Experimental and Clinical Pharmacology in the College of Pharmacy of the University of Minnesota. He is also a graduate faculty member of the Molecular, Cellular, Developmental Biology and Genetics graduate program of the University of Minnesota, a member of the Cancer Center of the University of Minnesota, and an associate member of the Genetics Graduate Faculty of the University of Minnesota Graduate School.[13]

Whitley began the seminal clinical trials of bone marrow transplantation for Hurler syndrome (MPS I-H) and other lysosomal diseases.[14][15][16][17][18][19][20][21][22] He continues as a provider of this treatment at the University of Minnesota. He also accomplished one of the few human clinical trials of gene therapy for inherited metabolic disorders.[23][24][25][26]

Whitley is the author of more than 100 peer-reviewed articles in scientific journals and five book chapters. He holds a patent for a "Method for the Detection of Mucopolysaccharide Storage Diseases."[27] He also holds a patent for "Methods of Treating Lysosomal Storage Related Diseases by Gene Therapy."[28] Whitley has been elected by peers for inclusion in Best Doctors in America from 1996 to 2013.

Biography edit

Whitley received his undergraduate degree in biology from Carleton College In Northfield, Minnesota. He earned a Ph.D. in human biochemical genetics from the University of Minnesota Graduate School in 1977 and his M.D. from the University of Minnesota Medical School in 1980. He completed an internship and a residency in pediatrics at the University of Minnesota Hospitals and was awarded a Fellowship in Genetics by the University of Minnesota Department of Pediatrics in 1983.

Whitley is a member of the American College of Medical Genetics, the American Society of Gene Therapy, the American Society of Human Genetics, and the Society for Inherited Metabolic Disorders. He is on the Scientific Advisory Board of the National MPS Society, Inc. Whitley is the Project Director and Principal Investigator of the Lysosomal Disease Network.[29] Whitley has been the organizing chair of each convening of the annual "WORLD Symposium" of the Lysosomal Disease Network since it first convened in 2004. The acronym WORLD represents ''We're organizing research for lysosomal diseases." He is the chair of the WORLD Lysosomal Disease Network Steering Organizing Committee.

Some of Whitley's recent responsibilities include being session organizer and co-chair of the 'Pharmacotherapy' session of the Phenylketonuria (PKU) Scientific Review Conference entitled 'State of the Science and Future Research Needs' convened by the National Institutes of Heath (NIH), Natcher Center, Bethesda, Maryland, 2011–2012; and serving as faculty of the North American Metabolic Academy course provided by the Society for Inherited Metabolic Diseases, 2010-2014. Whitley also currently serves on the clinical advisory board and the global MPS II advisory board of Shire Human Gene Therapies; and the clinical advisory committee of ReGenX Corporation.

Selected Publications edit

Partial list:

  • Eisengart JB, Rudser KD, Tolar J, Orchard PJ, Kivisto T, Ziegler RS, Whitley CB, Shapiro EG. Enzyme replacement is associated with better cognitive outcomes after transplant in Hurler Syndrome. J Pediatr. 2012 Sep 10. pii: S0022-3476(12)00877-3. doi: 10.1016/j.jpeds.2012.07.052. [Epub ahead of print]
  • Shapiro E, Guler OE, Rudser K, Delaney K, Bjoraker K, Whitley CB, Tolar J, Orchard P, Provenzale J, Thomas KM. An exploratory study of brain function and structure in Mucopolysaccharidosis Type I: long term observations following hematopoietic cell transplantation (HCT). Mol Genet Metab. 2012 Sep;107(1-2):116-21. doi: 10.1016/j.ymgme.2012.07.016. Epub 2012 Jul 20. PMID 22867884.
  • Arn P, Whitley CB, Wraith JE, Webb HW, Underhill L, Rangachari L, Cox GF. High rate of postoperative mortality in patients with Mucopolysaccharidosis I: findings from the MPS I Registry. J Pediatr Surg. 2012 Mar;47(3):477-84. doi: 10.1016/j.jpedsurg.2011.09.042. PMID 22424341.
  • Utz JR, Lorentz CP, Markowitz D, Rudser KD, Diethelm-Okita B, Erickson D, Whitley CB. START, a double blind, placebo-controlled pharmacogenetic test of responsiveness to sapropterin dihydrochloride in Phenylketonuria patients. Mol Genet Metab. 2012 Feb;105(2):193-7. doi: 10.1016/j.ymgme.2011.10.014. Epub 2011 Oct 29. PMID 22112818.
  • Wolf DA, Lenander AW, Nan Z, Belur LR, Whitley CB, Gupta P, Low WC, McIvor RS. Direct gene transfer to the CNS prevents emergence of neurologic disease in a murine model of Mucopolysaccharidosis Type I. Neurobiol Dis. 2011 Jul;43(1):123-33. doi: 10.1016/j.nbd.2011.02.015. Epub 2011 Mar 17. PMID 21397026.
  • Najafian B, Svarstad E, Bostad L, Gubler MC, Tøndel C, Whitley CB, Mauer M. Progressive podocyte injury and globotriaosylceramide (GL-3) accumulation in young patients with Fabry disease. Kidney Int. 2011 Mar;79(6):663-70. doi: 10.1038/ki.2010.484. Epub 2010 Dec 15. PMID 21160462.
  • Whitley CB, Utz JR. Maroteaux-Lamy syndrome (Mucopolysaccharidosis Type VI): a single dose of galsulfase further reduces urine glycosaminoglycans after hematopoietic stem cell transplantation. Mol Genet Metab. 2010 Dec;101(4):346-8. doi: 10.1016/j.ymgme.2010.07.015. Epub 2010 Aug 1. PMID 20800524.
  • Decker C, Yu ZF, Giugliani R, Schwartz IV, Guffon N, Teles EL, Miranda MC, Wraith JE, Beck M, Arash L, Scarpa M, Ketteridge D, Hopwood JJ, Plecko B, Steiner R, Whitley CB, Kaplan P, Swiedler SJ, Conrad S, Harmatz P. Enzyme replacement therapy for Mucopolysaccharidosis VI: growth and pubertal development in patients treated with recombinant human N-acetylgalactosamine 4-sulfatase. J Pediatr Rehabil Med. 2010;3(2):89-100. PMID 20634905.
  • Whitley CB. Mucopolysaccharidoses. In: Moser H.W. (ed), Neurodystrophies and Neurolipidoses, Handbook of Clinical Neurology, vol. 66, 1996. ISBN 978-0444812858.
  • Whitley CB, Belani K, Chang PN, Summers CG, Blazar BR, Tsai MY, Latchaw RE, Ramsay NKC, Kersey JH: Long-term outcome of Hurler syndrome following bone marrow transplantation, Am J Med Genet 46:209-218, 1993. PMID 8484412.
  • Whitley CB, Ridnour MD, Draper KM, Dutton CM, Neglia JP: Diagnostic test for mucopolysaccharidosis. I. Direct method for quantifying excessive urinary glycosaminoglycan excretion. Clin Chem 35:374-379, 1989. PMID 2493341.
  • Whitley CB, Draper KM, Dutton CM, Brown PA, Severson SL, France LA: Diagnostic test for mucopolysaccharidosis. II. Rapid quantification of glycosaminoglycan in urine samples collected on paper matrix. Clin Chem 35:2074-2081, 1989. PMID 2507197.
  • Whitley CB, Ramsay NKC, Kersey JH, Krivit W: Bone marrow transplantation for Hurler syndrome. Assessment of metabolic correction. Birth Defects 22:7-24, 1986. PMID 3083887.
  • References edit

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    External links edit