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Foundation Sciences · Biochemistry

Haem Synthesis

⏱️ 45–60 minutes read 📖 Biochemistry

Eight-step pathway from glycine + succinyl-CoA to haem; first and last steps mitochondrial.

📌 Learning Objectives

  • Describe the key principles of haem synthesis.
  • Explain the clinical relevance of haem synthesis.
  • Recognise common conditions linked to haem synthesis in MLA-style scenarios.

📋 Overview

Eight-step pathway from glycine + succinyl-CoA to haem; first and last steps mitochondrial. This topic integrates with pathology, pharmacology and clinical medicine and is frequently tested in UK medical school exams and the MLA.

🔬 Basic Science

Eight-step pathway from glycine + succinyl-CoA to haem; first and last steps mitochondrial. Detailed mechanisms, regulation and molecular interactions underpin both normal physiology and disease.

🏥 Clinical Relevance

Acute intermittent porphyria (porphobilinogen deaminase): abdominal pain, neuro symptoms, dark urine; treat with haem arginate, glucose.

🧪 Investigations

Relevant laboratory tests, imaging or histological examination are used as appropriate to the clinical context.

💊 Management

Management is condition-specific; principles include addressing the underlying biochemical/structural derangement, supportive care and targeted therapy where available.

Revision Resources – expand the sections below for high-yield notes, exam pearls, key facts and further reading.

🎯 MLA High-Yield Notes & Quick Revision ⌄
High-yield topic for the UK MLA — frequently appears in SBA questions linking biochemistry concepts to clinical presentations and management decisions.
Applying biomedical science to clinical practice Diagnosis and investigation Pathophysiology of common conditions
  • Eight-step pathway from glycine + succinyl-CoA to haem
  • first and last steps mitochondrial.
⭐ Exam Pearls ⌄
⭐ High Yield
ALA synthase rate-limiting; requires B6
Lead inhibits ALA dehydratase and ferrochelatase
Porphyrias arise from enzyme defects
Sideroblastic anaemia from disordered haem synthesis
💡 Clinical Pearl
: Acute intermittent porphyria (porphobilinogen deaminase): abdominal pain, neuro symptoms, dark urine; treat with haem arginate, glucose.
⚠️ Exam Tip — Common Mistakes
Confusing haem synthesis with related but distinct mechanisms.
Memorising pathways without linking to clinical disease.
🔑 Key Facts ⌄
ALA synthase rate-limiting; requires B6
Lead inhibits ALA dehydratase and ferrochelatase
Porphyrias arise from enzyme defects
Sideroblastic anaemia from disordered haem synthesis
📚 References ⌄
  1. BMJ Best Practice
  2. Robbins Basic Pathology
  3. Lippincott Illustrated Reviews: Biochemistry
  4. Wheater's Functional Histology
  5. NICE guidance where applicable.

Further Resources

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