@article{IR,
      author = {Van Grouw, Alexandria and Damekar, Shraddha and Merkens,  Louise and Pappu, Anuradha and Duell, Barton and DeBarber,  Andrea},
      url = {http://digitalcollections.ohsu.edu/record/8222},
      title = {Characterizing blood-brain sterol pathways in individuals  with deficient sterol 27-hydroxylase (CYP27A1) enzyme  activity},
      publisher = {Oregon Health and Science University},
      abstract = {Aberrant cholesterol metabolism is thought to play an  important role in brain pathophysiology for many  neurodegenerative disorders. How sterol metabolism impacts  cerebral accumulation of toxic sterol species is not fully  characterized. The brain houses 20-25% of total body  cholesterol in a de novo synthesized pool unable to  traverse the BBB. Formation of  7a-hydroxy-3-oxo-4-cholestenoic acid (7-HOCA) by the sterol  27-hydroxylase (CYP27A1) enzyme is one route of cerebral  cholesterol removal, with flux of 7-HOCA demonstrated to  occur across the BBB into the periphery. Deficient CYP27A1  activity is associated with cerebrotendinous xanthomatosis  (CTX); a rare genetic disorder that can cause irreversible  neurological decline associated with formation of  cerebellar xanthomas, consisting mostly of cholesterol and  a saturated analogue, cholestanol.},
      number = {IR},
      doi = {https://doi.org/10.6083/bg257f59s},
      recid = {8222},
      address = {2020},
}