What are Peroxisomes ABP Board Review

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This is a snippet from Dr. Christina L. Grant’s Metabolic Disorders lecture in MedStudy’s 2023-2024 Pediatrics Video Board Review. https://medstudy.com/products/pediatr... • What are Peroxisomes? • • Ubiquitous organelles in all cells • • Impermeable membrane to keep isolated functions • • Functions • – Fatty acid β-oxidation • • Catalyzes VLCFAs • • Bile acids • • Branched-chain fatty acids • • Eicosanoids • – Ether phospholipid biosynthesis • • Plasmalogens are important part of the brain • – Catalase: degradation of hydrogen peroxide • – Cholesterol synthesis steps • Peroxisomal Disorders — Zellweger Spectrum Disorders • • Do not make normal peroxisomes • – Absent/Reduced function of all enzymes • • Clinical findings • – Characteristic facies, large anterior fontanelle • – Hypotonia • – Cataracts, pigmented retinopathy • – Epiphyseal stippling (pinpoint calcifications) • – Liver function abnormalities, jaundice • • Zellweger spectrum disorders • – Continuum of disease severity • – Includes neonatal adrenoleukodystrophy (ALD) and infantile Refsum disease (IRD) • Continue your review: https://medstudy.com/products/pediatr... • Subscribe to our YouTube channel: / @medstudycom • Follow us on: • Instagram: / medstudystrong • Facebook: / medstudy • Twitter: / medstudy • TikTok: / medstudystrong • #Pediatricians #Pediatrician #Pediatrics #Peds #ABP #AOBP #boardexam #boardprep #meded #medicaleducation

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