{"id":1989,"date":"2023-02-22T16:06:00","date_gmt":"2023-02-22T16:06:00","guid":{"rendered":"https:\/\/www.osmosis.org\/blog\/?p=1989"},"modified":"2025-10-01T11:40:32","modified_gmt":"2025-10-01T19:40:32","slug":"usmle-step-1-question-of-the-day-peroxisomes","status":"publish","type":"post","link":"https:\/\/www.osmosis.org\/blog\/usmle-step-1-question-of-the-day-peroxisomes","title":{"rendered":"USMLE\u00ae Step 1 Question of the Day: Peroxisomes"},"content":{"rendered":"<div id=\"ez-toc-container\" class=\"ez-toc-v2_0_80 ez-toc-wrap-center counter-hierarchy ez-toc-counter ez-toc-custom ez-toc-container-direction\">\n<div class=\"ez-toc-title-container\">\n<p class=\"ez-toc-title\" style=\"cursor:inherit\">In This Article<\/p>\n<span class=\"ez-toc-title-toggle\"><a href=\"#\" class=\"ez-toc-pull-right ez-toc-btn ez-toc-btn-xs ez-toc-btn-default ez-toc-toggle\" aria-label=\"Toggle Table of Content\"><span class=\"ez-toc-js-icon-con\"><span class=\"\"><span class=\"eztoc-hide\" style=\"display:none;\">Toggle<\/span><span class=\"ez-toc-icon-toggle-span\"><svg style=\"fill: #999;color:#999\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" class=\"list-377408\" width=\"20px\" height=\"20px\" viewBox=\"0 0 24 24\" fill=\"none\"><path d=\"M6 6H4v2h2V6zm14 0H8v2h12V6zM4 11h2v2H4v-2zm16 0H8v2h12v-2zM4 16h2v2H4v-2zm16 0H8v2h12v-2z\" fill=\"currentColor\"><\/path><\/svg><svg style=\"fill: #999;color:#999\" class=\"arrow-unsorted-368013\" xmlns=\"http:\/\/www.w3.org\/2000\/svg\" width=\"10px\" height=\"10px\" viewBox=\"0 0 24 24\" version=\"1.2\" baseProfile=\"tiny\"><path d=\"M18.2 9.3l-6.2-6.3-6.2 6.3c-.2.2-.3.4-.3.7s.1.5.3.7c.2.2.4.3.7.3h11c.3 0 .5-.1.7-.3.2-.2.3-.5.3-.7s-.1-.5-.3-.7zM5.8 14.7l6.2 6.3 6.2-6.3c.2-.2.3-.5.3-.7s-.1-.5-.3-.7c-.2-.2-.4-.3-.7-.3h-11c-.3 0-.5.1-.7.3-.2.2-.3.5-.3.7s.1.5.3.7z\"\/><\/svg><\/span><\/span><\/span><\/a><\/span><\/div>\n<nav><ul class='ez-toc-list ez-toc-list-level-1 ' ><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-1\" href=\"https:\/\/www.osmosis.org\/blog\/usmle-step-1-question-of-the-day-peroxisomes\/#The_correct_answer_to_todays_USMLE%C2%AE_Step_1_Question_is%E2%80%A6\" >The correct answer to today&#8217;s USMLE\u00ae Step 1 Question is&#8230;<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-2\" href=\"https:\/\/www.osmosis.org\/blog\/usmle-step-1-question-of-the-day-peroxisomes\/#A_a-oxidation_of_branched-chain_fatty_acids\" >A. \u0251-oxidation of branched-chain fatty acids<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-3\" href=\"https:\/\/www.osmosis.org\/blog\/usmle-step-1-question-of-the-day-peroxisomes\/#Incorrect_answer_explanations\" >Incorrect answer explanations<\/a><ul class='ez-toc-list-level-3' ><li class='ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-4\" href=\"https:\/\/www.osmosis.org\/blog\/usmle-step-1-question-of-the-day-peroxisomes\/#B_Modification_of_proteins_targeted_for_lysosomal_degradation\" >B. Modification of proteins targeted for lysosomal degradation<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-5\" href=\"https:\/\/www.osmosis.org\/blog\/usmle-step-1-question-of-the-day-peroxisomes\/#C_Clearance_of_bronchopulmonary_secretions_via_ciliary_oscillations\" >C. Clearance of bronchopulmonary secretions via ciliary oscillations&nbsp;<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-6\" href=\"https:\/\/www.osmosis.org\/blog\/usmle-step-1-question-of-the-day-peroxisomes\/#D_Synthesis_of_amino_acid_chains\" >D. Synthesis of amino acid chains&nbsp;<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-3'><a class=\"ez-toc-link ez-toc-heading-7\" href=\"https:\/\/www.osmosis.org\/blog\/usmle-step-1-question-of-the-day-peroxisomes\/#E_Synthesis_of_ATP_via_oxidative_phosphorylation\" >E. Synthesis of ATP via oxidative phosphorylation<\/a><\/li><\/ul><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-8\" href=\"https:\/\/www.osmosis.org\/blog\/usmle-step-1-question-of-the-day-peroxisomes\/#Major_Takeaway\" >Major Takeaway<\/a><\/li><li class='ez-toc-page-1 ez-toc-heading-level-2'><a class=\"ez-toc-link ez-toc-heading-9\" href=\"https:\/\/www.osmosis.org\/blog\/usmle-step-1-question-of-the-day-peroxisomes\/#References\" >References<\/a><\/li><\/ul><\/nav><\/div>\n\n<p class=\"wp-block-paragraph\"><em><strong>This week, we are sharing another USMLE\u00ae Step 1-style practice question to test your knowledge of medical topics. Today&#8217;s case involves a 5-month-old infant who was brought to a pediatrician for evaluation of failure to thrive. Upon physical examination, the patient has a weak cry and diffuse hypotonia. Hepatomegaly is present with abdominal palpation. The organelle affected in this patient&#8217;s condition is normally involved in which of the following cellular processes? Can you figure it out?<\/strong><\/em><\/p>\n\n\n\n<p class=\"wp-block-paragraph\">A 5-month-old male infant is brought to a pediatrician for evaluation of failure to thrive. The patient was recently adopted and has never been evaluated by a physician as far as the new parents are aware. According to the parents, the infant cannot grab objects or roll from a prone to supine position. He has also experienced several episodes of generalized seizure activity. He is at the 10th percentile for length and 5th percentile for weight. Temperature is 37.0\u00b0C (98.6\u00b0F), blood pressure is 80\/53 mmHg, and pulse is 92\/min. Upon physical examination, the patient has a weak cry and diffuse hypotonia. Hepatomegaly is present with abdominal palpation. Genetic analysis reveals a mutation in the PEX gene. The organelle affected in this patient&#8217;s condition is normally involved in which of the following cellular processes?<\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>A. &nbsp;\u0251-oxidation of branched-chain fatty acids<\/strong><strong>B. &nbsp;Modification of proteins targeted for lysosomal degradation<\/strong><\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>C. Clearance of bronchopulmonary secretions via ciliary oscillations<br><\/strong><br><strong>D. Synthesis of amino acid chains<br><\/strong><br><strong>E. Synthesis of ATP via oxidative phosphorylation<\/strong><\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Scroll down to find the answer!<\/p>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"The_correct_answer_to_todays_USMLE%C2%AE_Step_1_Question_is%E2%80%A6\"><\/span>The correct answer to today&#8217;s USMLE\u00ae Step 1 Question is&#8230;<span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"A_a-oxidation_of_branched-chain_fatty_acids\"><\/span>A. \u0251-oxidation of branched-chain fatty acids<span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p class=\"wp-block-paragraph\">Before we get to the Main Explanation, let&#8217;s look at the incorrect answer explanations. Skip to the bottom if you want to see the correct answer right away!<\/p>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Incorrect_answer_explanations\"><\/span>Incorrect answer explanations<span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p class=\"wp-block-paragraph\">The incorrect answers to today&#8217;s USMLE\u00ae Step 1 Question are&#8230;<\/p>\n\n\n\n<h3 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"B_Modification_of_proteins_targeted_for_lysosomal_degradation\"><\/span>B. Modification of proteins targeted for lysosomal degradation<span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Incorrect:&nbsp;<\/strong>The Golgi body is responsible for adding mannose-6-phosphate residues to proteins targeted for lysosomal degradation. In I-cell disease, the Golgi body fails to phosphorylate mannose residues, and proteins are secreted into the extracellular space. Patients with I-cell disease often present with coarse facial features, restricted joint mobility, and skeletal abnormalities. This disease is not associated with a&nbsp;<em>PEX<\/em>&nbsp;gene mutation.<\/p>\n\n\n\n<h3 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"C_Clearance_of_bronchopulmonary_secretions_via_ciliary_oscillations\"><\/span>C. Clearance of bronchopulmonary secretions via ciliary oscillations&nbsp;<span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Incorrect:<\/strong>&nbsp;Cilia assist in clearing bronchopulmonary secretions. Primary ciliary dyskinesia is an autosomal recessive condition characterized by immotile cilia. Patients are at risk of airway disease due to impaired mucociliary clearance. Around 50% of patients with this condition also have situs inversus. The patient in this vignette does not have a history of recurrent pulmonary infections.<\/p>\n\n\n\n<h3 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"D_Synthesis_of_amino_acid_chains\"><\/span>D. Synthesis of amino acid chains&nbsp;<span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Incorrect:&nbsp;<\/strong>Ribosomes synthesize amino acid chains from mRNA. However, this patient is presenting with hypotonia, seizures, hepatomegaly, and a mutation in the PEX gene, which is more concerning for Zellweger disease \u2013 a peroxisomal disorder.<\/p>\n\n\n\n<h3 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"E_Synthesis_of_ATP_via_oxidative_phosphorylation\"><\/span>E. Synthesis of ATP via oxidative phosphorylation<span class=\"ez-toc-section-end\"><\/span><\/h3>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Incorrect:&nbsp;<\/strong>The mitochondria contain the enzymes comprising the electron transport chain involved in synthesizing ATP via oxidative phosphorylation. Mitochondrial abnormalities can lead to myopathies and optic neuropathy. However, this patient is presenting with hypotonia, seizures, hepatomegaly, and a mutation in the<em>&nbsp;PEX<\/em>&nbsp;gene, which is more concerning for Zellweger disease \u2013 a peroxisomal disorder.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Main Explanation<\/strong><\/p>\n\n\n\n<p class=\"wp-block-paragraph\">This young child presents with hypotonia, hepatomegaly, and a history of seizures. Genetic testing reveals a&nbsp;<strong><em>PEX<\/em>&nbsp;gene mutation<\/strong>. Together, these findings are consistent with&nbsp;<strong>Zellweger syndrome<\/strong>\u2013a disease that occurs secondary to peroxisomal defects.&nbsp;<\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Peroxisomes<\/strong>&nbsp;are membrane-bound organelles involved in several critical cellular processes, including:<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">1. Neutralization of&nbsp;<strong>free radicals<\/strong>, which in turn protects cellular components such as DNA, lipids, and proteins from oxidative damage.&nbsp;<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">2. Breakdown of&nbsp;<strong>toxic substances<\/strong>&nbsp;including ethanol and formaldehyde. This process is performed by catalase and also helps convert H2O2&nbsp;into water.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">3.&nbsp;<strong>\u0251-oxidation<\/strong>, where branched-chain fatty acids are broken down into linear fatty acids.&nbsp;<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">4.&nbsp;<strong>\u03b2-oxidation<\/strong>, where linear very-long-chain fatty acids (VLCFAs) and medium-chain fatty acids (MCFAs) are enzymatically broken down into progressively smaller chains.&nbsp;<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">5. Breakdown of amino acids6. Synthesis of&nbsp;<strong>bile acids, cholesterol<\/strong>, and&nbsp;<strong>plasmalogens.&nbsp;<\/strong><\/p>\n\n\n\n<p class=\"wp-block-paragraph\">6. Synthesis of\u00a0<strong>bile acids, cholesterol<\/strong>, and\u00a0<strong>plasmalogens.\u00a0<\/strong><strong>plasmalogens.&nbsp;<\/strong><\/p>\n\n\n\n<figure class=\"wp-block-image size-full\"><img loading=\"lazy\" decoding=\"async\" width=\"630\" height=\"406\" src=\"https:\/\/www.osmosis.org\/blog\/wp-content\/uploads\/sites\/2\/2024\/09\/image_e69d0d.png\" alt=\"Peroxisomal Disorder graphic description\" class=\"wp-image-1992\" srcset=\"https:\/\/www.osmosis.org\/blog\/wp-content\/uploads\/sites\/2\/2024\/09\/image_e69d0d.png 630w, https:\/\/www.osmosis.org\/blog\/wp-content\/uploads\/sites\/2\/2024\/09\/image_e69d0d.png?resize=300,193 300w\" sizes=\"auto, (max-width: 630px) 100vw, 630px\" \/><\/figure>\n\n\n\n<h2 class=\"wp-block-heading\"><\/h2>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"Major_Takeaway\"><\/span><strong>Major Takeaway<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p class=\"wp-block-paragraph\"><strong>Peroxisomes&nbsp;<\/strong>are membrane-bound organelles that perform an array of functions including&nbsp;<strong>\u0251-oxidation<\/strong>&nbsp;and&nbsp;<strong>\u03b2-oxidation&nbsp;<\/strong>of fatty acids, neutralization of&nbsp;<strong>free radicals<\/strong>, and breakdown of&nbsp;<strong>toxic substances<\/strong>&nbsp;like alcohol. Zellweger syndrome is a peroxisomal disorder which presents with hypotonia, seizures, hepatomegaly, and a mutation in the&nbsp;<em>PEX<\/em>&nbsp;gene.<\/p>\n\n\n\n<h2 class=\"wp-block-heading\"><span class=\"ez-toc-section\" id=\"References\"><\/span><strong>References<\/strong><span class=\"ez-toc-section-end\"><\/span><\/h2>\n\n\n\n<p class=\"wp-block-paragraph\">Delille, H.K., Bonekamp, N.A., Schrader, M. (2006) Peroxisomes and disease- An Overview.&nbsp;<em>International Journal of Biomedical Sciences<\/em>. 2(4), 308-314. PMID: 23674998.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\">Elumalai, V., Pasrija, D. (2020) \u201cZellweger syndrome\u201d.&nbsp;<em>StatPearls [Internet].<\/em>&nbsp;Web Address:&nbsp;<a href=\"https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK560676\/\" target=\"_blank\" rel=\"noreferrer noopener\">https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK560676\/<\/a>.<\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><em><strong>Want more USMLE\u00ae Step 1 practice questions? Try Osmosis today! Access your&nbsp;<em><strong><a href=\"https:\/\/www.osmosis.org\/login?type=create\" target=\"_blank\" rel=\"noreferrer noopener\">free trial<\/a><\/strong><\/em>&nbsp;and find out why millions of current and future clinicians and caregivers love learning with us.<\/strong><\/em><\/p>\n\n\n\n<figure class=\"wp-block-image size-large\"><a href=\"https:\/\/www.osmosis.org\/plans\/md\" target=\"_blank\" rel=\"noreferrer noopener\"><img loading=\"lazy\" decoding=\"async\" height=\"366\" width=\"1024\" src=\"https:\/\/www.osmosis.org\/blog\/wp-content\/uploads\/sites\/2\/2024\/09\/2024-09-11T101549.044.png?w=1024\" alt=\"Osmosis ad to create an account\" class=\"wp-image-1993\" srcset=\"https:\/\/www.osmosis.org\/blog\/wp-content\/uploads\/sites\/2\/2024\/09\/2024-09-11T101549.044.png 1400w, https:\/\/www.osmosis.org\/blog\/wp-content\/uploads\/sites\/2\/2024\/09\/2024-09-11T101549.044.png?resize=300,107 300w, https:\/\/www.osmosis.org\/blog\/wp-content\/uploads\/sites\/2\/2024\/09\/2024-09-11T101549.044.png?resize=768,274 768w, https:\/\/www.osmosis.org\/blog\/wp-content\/uploads\/sites\/2\/2024\/09\/2024-09-11T101549.044.png?resize=1024,366 1024w\" sizes=\"auto, (max-width: 1024px) 100vw, 1024px\" \/><\/a><\/figure>\n\n\n\n<p class=\"wp-block-paragraph\"><em><strong><em><sub>The United States Medical Licensing Examination (USMLE\u00ae) is a joint program of the Federation of State Medical Boards (FSMB\u00ae) and National Board of Medical Examiners (NBME\u00ae). Osmosis is not affiliated with NBME nor FSMB.<\/sub><\/em><\/strong><\/em><\/p>\n","protected":false},"excerpt":{"rendered":"<p>This week, we are sharing another USMLE\u00ae Step 1-style practice question to test your knowledge of medical topics. Today&#8217;s case involves a 5-month-old infant who was brought to a pediatrician for evaluation of failure to thrive. Upon physical examination, the patient has a weak cry and diffuse hypotonia. Hepatomegaly is present with abdominal palpation. The [&hellip;]<\/p>\n","protected":false},"author":202,"featured_media":1991,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_jetpack_memberships_contains_paid_content":false,"footnotes":""},"categories":[27,20,1369,44],"tags":[],"class_list":["post-1989","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-medicine","category-exam-prep","category-step-1-questions","category-step-1"],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v23.6 - https:\/\/yoast.com\/wordpress\/plugins\/seo\/ -->\n<title>USMLE\u00ae Step 1 Question of the Day: Peroxisomes - Osmosis Blog<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/www.osmosis.org\/blog\/usmle-step-1-question-of-the-day-peroxisomes\" \/>\n<meta property=\"og:locale\" content=\"en_US\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"USMLE\u00ae Step 1 Question of the Day: Peroxisomes - Osmosis Blog\" \/>\n<meta property=\"og:description\" content=\"This week, we are sharing another USMLE\u00ae Step 1-style practice question to test your knowledge of medical topics. Today&#8217;s case involves a 5-month-old infant who was brought to a pediatrician for evaluation of failure to thrive. Upon physical examination, the patient has a weak cry and diffuse hypotonia. Hepatomegaly is present with abdominal palpation. 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