Core B: Neuro-Histology and Behavior Core. The overall objective of the Program Project is to study the effects of various gene constructs for treating lysosomal metabolic disorders in mice. If the gene constructs are capable of correcting the metabolic disorders then the treated mice must be studied to determine their phenotype. This will be done histologically to determine if cells express the appropriate enzymes, and whether the pathology has been corrected. Animal phenotype with respect to neurological/behavioral function and auditory neurophysiology will also be evaluated by this core. These services will aid in addressing the central hypothesis of the program project, i.e., that gene therapy can correct neurological deficits associated with lysosomal storage disorders. Also, mice that are homozygous for these disorders must be genotyped for proper identification. Core B will provide this genotyping service.

Public Health Relevance

The functions of the Neuro-Histology and Behavior Core are integral to the research performed by the Program. This Core provides support for all aspects of animal behavioral assessment, neurohistology, and genotyping for Projects I, II, and III.

Agency
National Institute of Health (NIH)
Institute
Eunice Kennedy Shriver National Institute of Child Health & Human Development (NICHD)
Type
Research Program Projects (P01)
Project #
5P01HD032652-17
Application #
8700142
Study Section
Special Emphasis Panel (ZHD1-DSR-Z)
Project Start
Project End
Budget Start
2014-07-01
Budget End
2015-06-30
Support Year
17
Fiscal Year
2014
Total Cost
$175,732
Indirect Cost
$59,391
Name
University of Minnesota Twin Cities
Department
Type
DUNS #
555917996
City
Minneapolis
State
MN
Country
United States
Zip Code
55455
Ou, Li; Przybilla, Michael J; Whitley, Chester B (2016) Proteomic analysis of mucopolysaccharidosis I mouse brain with two-dimensional polyacrylamide gel electrophoresis. Mol Genet Metab :
Ou, Li; Przybilla, Michael J; Koniar, Brenda L et al. (2016) Elements of lentiviral vector design toward gene therapy for treating mucopolysaccharidosis I. Mol Genet Metab Rep 8:87-93
Aronovich, Elena L; Hackett, Perry B (2015) Lysosomal storage disease: gene therapy on both sides of the blood-brain barrier. Mol Genet Metab 114:83-93
Wolf, Daniel A; Banerjee, Sharbani; Hackett, Perry B et al. (2015) Gene therapy for neurologic manifestations of mucopolysaccharidoses. Expert Opin Drug Deliv 12:283-96
Satzer, David; DiBartolomeo, Christina; Ritchie, Michael M et al. (2015) Assessment of dysmyelination with RAFFn MRI: application to murine MPS I. PLoS One 10:e0116788
Ou, Li; Herzog, Tyler; Koniar, Brenda L et al. (2014) High-dose enzyme replacement therapy in murine Hurler syndrome. Mol Genet Metab 111:116-22
Hackett, Perry B; Aronovich, Elena L (2014) Rational design for enhanced gene therapy with DNA transposons. Mol Ther 22:1575-7
Janson, Christopher G; Romanova, Liudmila G; Leone, Paola et al. (2014) Comparison of Endovascular and Intraventricular Gene Therapy With Adeno-Associated Virus-α-L-Iduronidase for Hurler Disease. Neurosurgery 74:99-111
Ou, Li; Herzog, Tyler L; Wilmot, Carrie M et al. (2014) Standardization of *-L-iduronidase enzyme assay with Michaelis-Menten kinetics. Mol Genet Metab 111:113-5
Carpentier, Claire E; Schreifels, Jeffrey M; Aronovich, Elena L et al. (2014) NMR structural analysis of Sleeping Beauty transposase binding to DNA. Protein Sci 23:23-33

Showing the most recent 10 out of 83 publications