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Defining a Transcriptional Regulatory Network for Peroxisome Biogenesis
Author(s) -
Paz Edwin A.,
Gaasterland Terry,
La Spada Albert
Publication year - 2016
Publication title -
the faseb journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.709
H-Index - 277
eISSN - 1530-6860
pISSN - 0892-6638
DOI - 10.1096/fasebj.30.1_supplement.803.5
Subject(s) - zellweger syndrome , peroxisome , biogenesis , adrenoleukodystrophy , biology , transcription factor , peroxisomal disorder , transcriptional regulation , gene , function (biology) , human genetics , organelle biogenesis , computational biology , genetics , bioinformatics
Peroxisomes are highly dynamic organelles that are involved in lipid metabolism and hydrogen peroxide break‐down. Dysregulation of peroxisomal processes are associated with a group of neurodevelopmental disorders including Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum disease. Underlying these disorders are mutations in PEX genes resulting in faulty peroxin production and function. We hypothesize that important contributors to neurodegenerative diseases are dysregulated transcriptional networks affecting peroxisome biology. Using a bioinformatics approach, we have identified potential transcription factors and their consensus transcriptional factor binding site (TFBS) motifs, and are testing if these TFBSs are coordinately regulating peroxisome biogenesis and function. Identifying novel transcriptional regulators of peroxisome biogenesis may reveal novel targets that can be exploited for therapeutic intervention. Support or Funding Information R01 AG033082 K12 GM068524