Скачать или смотреть 5. Connective Tissue Biochemistry | Elastin Structure, Synthesis & Clinical Aspects | USMLE Step 1

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Connective Tissue Biochemistry | Elastin Structure, Synthesis & Clinical Aspects | MBBS | USMLE Step 1

👋 Hey future doctors and biochem learners! After collagen, the next key structural protein of connective tissue is Elastin — the molecule that gives tissues like lungs, skin, and arteries their stretch and recoil properties. 🫁🩸

In this lecture, we’ll break down the structure of elastin, its biosynthesis, cross-linking, and applied clinical aspects. A must-know topic for MBBS, NEET PG, FMGE, and USMLE Step 1. 🧬📚

🌟 Structure & Synthesis of Elastin

Tropoelastin precursor synthesized by fibroblasts and smooth muscle cells.

Rich in glycine, proline, and unique cross-linking amino acids (desmosine and isodesmosine).

Secreted into extracellular space and deposited on a fibrillin scaffold.

Cross-linked by lysyl oxidase (copper-dependent) → provides elasticity.

🧪 Regulation

Elastase (enzyme) degrades elastin.

Normally inhibited by α1-antitrypsin (AAT).

Balance between elastin synthesis and degradation maintains tissue elasticity.

🩺 Applied Clinical Aspects

1️⃣ Emphysema (COPD) 🫁

Caused by destruction of alveolar elastin by elastase.

Smoking inactivates α1-antitrypsin → unchecked elastase activity.

Result: loss of lung elasticity, enlarged alveoli, breathlessness.

2️⃣ Marfan Syndrome 🧬

Mutation in fibrillin-1 gene → defective scaffold for elastin deposition.

Clinical: tall stature, long limbs, lens dislocation, aortic aneurysms.

3️⃣ Cutis Laxa 🧵

Rare inherited/acquired condition.

Defective cross-linking of elastin → loose, wrinkled skin.

4️⃣ α1-Antitrypsin Deficiency

Inherited deficiency of AAT → premature emphysema, liver disease.

Clinical clue: young patient with COPD + liver dysfunction.

🎯 Exam Integration

Elastin = stretch & recoil protein.

Cofactor for lysyl oxidase = Copper.

Disorders: Emphysema (elastase), Marfan (fibrillin), Cutis laxa (cross-linking defect), AAT deficiency.
👉 A classic case-based topic in USMLE Step 1 and NEET PG.

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❓Want us to create a Collagen vs Elastin comparative lecture (differences in structure, synthesis, and clinical disorders) for your Connective Tissue playlist? Drop your request in the comments below! 😊

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