PEROXISOMAL FATTY ACID METABOLISM IN GENETIC AND AGE-RELATED DISORDERS - PROJECT SUMMARY/ABSTRACT PEROXISOM…
Federal financial assistance recorded from USAspending. This is not a procurement contract.
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ASST_NON_R00AG070271_075 · OREGON HEALTH & SCIENCE UNIVERSITY · Department of Health and Human Services
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- Assistance award identifier
- ASST_NON_R00AG070271_075
- FAIN
- R00AG070271
- Recipient
- OREGON HEALTH & SCIENCE UNIVERSITY
- Awarding agency
- Department of Health and Human Services
- Funding agency
- Department of Health and Human Services
- Assistance listing (CFDA)
- 93.866
- Total obligated
- $740,805
- Period
- Feb 1, 2022 – May 31, 2027
- Fiscal year
- 2024
- Place of performance state
- OR
- Source file
- FY2024_All_Assistance_Full_20261006.zip
Description
PEROXISOMAL FATTY ACID METABOLISM IN GENETIC AND AGE-RELATED DISORDERS - PROJECT SUMMARY/ABSTRACT PEROXISOMES ARE UBIQUITOUS EUKARYOTIC ORGANELLES THAT FORM A FOCAL POINT FOR MULTIPLE METABOLIC PATHWAYS. LIPID METABOLISM, AND IN PARTICULAR, FATTY ACID TRANSPORT RELATED TO IT, DEPEND HEAVILY ON PEROXISOMAL MEMBRANE PROTEINS THAT HAVE SPECIFICALLY EVOLVED FOR SUCH PURPOSES. THE ATP-DEPENDENT CASSETTE (ABC) TRANSPORTERS OF âDâ SUBFAMILYâ RESIDE IN THE PEROXISOMAL MEMBRANE AND ARE RESPONSIBLE FOR FATTY ACID IMPORT INTO THE PEROXISOMES, DEFECTS IN WHICH PROCESS ARE RELATED TO VARIOUS METABOLIC DISORDERS. MUTATIONS IN ABCD1 CAUSE X-LINKED ADRENOLEUKODYSTROPHY (X-ALD) WHICH MANIFESTS AS MILD TO SEVERE CENTRAL NERVOUS SYSTEM (CNS) DEMYELINATION. DYSFUNCTION OF ABCD3 AND/OR PEROXISOME BIOGENESIS FACTORS (PEXS) MAY CAUSE ZELLWEGER SYNDROME (ZS), A HETEROGENEOUS GROUP OF PEROXISOME ASSEMBLY DISORDERS. IN ADDITION TO INHERITED DISEASES, REDUCED PEROXISOMAL FUNCTION IS ASSOCIATED WITH AGING AND PATHOGENESIS OF AGE-RELATED ACQUIRED DISEASES LIKE DIABETES, NEURODEGENERATIVE DISORDERS. KEY GAPS IN UNDERSTANDING THE FUNCTION OF ABCDS IN METABOLISM AND DISEASE ARE DUE TO LACK OF STRUCTURAL DETAILS, PARTICULARLY OF THEIR CONFORMATIONAL PLASTICITY DURING SUBSTRATE TRANSPORT, AND HOW THIS IS COMPROMISED DURING AGING AND DISEASE. MY GOAL IS TO UNDERSTAND THE INVOLVEMENT OF PEROXISOMAL FATTY ACID METABOLISM IN AGING AND AGE-RELATED NEURODEGENERATIVE DISEASES, SPECIFICALLY ALZHEIMERâS DISEASE AND ULTIMATELY TO LEAD A RESEARCH GROUP AS AN INDEPENDENT RESEARCHER IN THIS AREA. I PROPOSE TO ELUCIDATE THE STRUCTURAL BASIS FOR FUNCTION OF ABCDS AT ATOMIC LEVEL USING CRYOEM IN THE ABSENCE AND PRESENCE OF SUBSTRATE, AND ATP (AIM 1A AND 1B, MENTORED), COUPLING BETWEEN ATPASE AND THIOESTERASE ACTIVITY, AND FATTY ACID BINDING AND TRANSPORT (AIM 2A AND 2B, MENTORED). I WILL DETERMINE ATOMIC STRUCTURES OF ABCDS IN COMPLEX WITH PEXS (PEX3 AND PEX19) USING CRYOEM (AIM 3A, MENTORED) AND ASSESS ALT