Podcast
Questions and Answers
Which protein is NOT a type of ECM proteins?
Which protein is NOT a type of ECM proteins?
- Structural proteins:
- Specialized proteins
- Proteoglycans
- Tropocollagen (correct)
Match the protein type to its proteins
Match the protein type to its proteins
Structural proteins = Collagen, Elastin, Fibrillin Specialized proteins = Fibronectin, Laminin Proteoglycans = GAGs x = x
What is needed for the synthesis of Collagen
What is needed for the synthesis of Collagen
Fibroblasts
What is the structural unit of collagen?
What is the structural unit of collagen?
What does collagen mainly consist of?
What does collagen mainly consist of?
Why does collagen have a very firm structure?
Why does collagen have a very firm structure?
What is the first step to the synthesis of collagen?
What is the first step to the synthesis of collagen?
How does the first step in the intracellular synthesis of collagen end?
How does the first step in the intracellular synthesis of collagen end?
What happens in the second step in the intracellular synthesis of collagen?
What happens in the second step in the intracellular synthesis of collagen?
How does the intracellular synthesis of collagen end?
How does the intracellular synthesis of collagen end?
How does the formation of tropocollagen from procollagen proceed?
How does the formation of tropocollagen from procollagen proceed?
How do the interchain cross links form?
How do the interchain cross links form?
What does a genetic defect in collagen lead to?
What does a genetic defect in collagen lead to?
What is the cause of Osteogenesis imperfecta aka Brittle bone syndrome
What is the cause of Osteogenesis imperfecta aka Brittle bone syndrome
Which answer does not correctly state the manifestations of Osteogenesis imperfecta?
Which answer does not correctly state the manifestations of Osteogenesis imperfecta?
What is the cause of Ehlers Danlos syndrome?
What is the cause of Ehlers Danlos syndrome?
What are the manifestations of Ehlers Danlos syndrome?
What are the manifestations of Ehlers Danlos syndrome?
What is the cause of Lathyrism?
What is the cause of Lathyrism?
What are the manifestations of Lathyrism?
What are the manifestations of Lathyrism?
Elastin is a … like protein
Elastin is a … like protein
Where is collagen found in the body?
Where is collagen found in the body?
Where is elastin found in the body?
Where is elastin found in the body?
What is the main unit of elastin?
What is the main unit of elastin?
Why does elastin have elastic properties?
Why does elastin have elastic properties?
What is the shape of elastin?
What is the shape of elastin?
What is the importance of a-1 anti-trypsin deficiency?
What is the importance of a-1 anti-trypsin deficiency?
Why do proteinases like trypsin and elastase get secreted?
Why do proteinases like trypsin and elastase get secreted?
What happens in the absence of a-1 anti-trypsin?
What happens in the absence of a-1 anti-trypsin?
What is fibronectin?
What is fibronectin?
Where is fibronectin found?
Where is fibronectin found?
What is fibronectin's structure?
What is fibronectin's structure?
What does fibronectin bind with?
What does fibronectin bind with?
What is fibronectin's importance?
What is fibronectin's importance?
What is fibronectin glomerulopathy?
What is fibronectin glomerulopathy?
What are the effects of fibronectin glomerulopathy?
What are the effects of fibronectin glomerulopathy?
What are the manifestations of fibronectin glomerulopathy?
What are the manifestations of fibronectin glomerulopathy?
What is fibrillin?
What is fibrillin?
Where is fibrillin found?
Where is fibrillin found?
What is the importance of fibrillin?
What is the importance of fibrillin?
What is the cause of Marfan’s syndrome?
What is the cause of Marfan’s syndrome?
What are the manifestations of Marfan’s syndrome?
What are the manifestations of Marfan’s syndrome?
What are cartilage proteins and how are they classified?
What are cartilage proteins and how are they classified?
What is the main collagen protein?
What is the main collagen protein?
What is collagen type II?
What is collagen type II?
What is the most important non-collagenous protein in cartilage?
What is the most important non-collagenous protein in cartilage?
What do the GAGs in cartilage look like?
What do the GAGs in cartilage look like?
Who is the binder in cartilage proteins?
Who is the binder in cartilage proteins?
What are bone proteins and how are they classified?
What are bone proteins and how are they classified?
What is the most important collagen protein in bone proteins?
What is the most important collagen protein in bone proteins?
What is the major non-collagenous protein in bone proteins?
What is the major non-collagenous protein in bone proteins?
Who is the binder in bone proteins?
Who is the binder in bone proteins?
What is osteoporosis?
What is osteoporosis?
What are risk factors of osteoporosis?
What are risk factors of osteoporosis?
Which protein is NOT a Glycoprotein?
Which protein is NOT a Glycoprotein?
Match the protein with its common sites
Match the protein with its common sites
Match the protein with its structure
Match the protein with its structure
Match the protein with its function
Match the protein with its function
Match the protein with its disorders
Match the protein with its disorders
Study Notes
Collagen & Elastin
- Collagen is found in various tissues, including skin, bone, cartilage, tendons, ligaments, blood vessels, and cornea.
- Elastin is found in ligaments, lungs, and the walls of blood vessels.
- Tropoelastin is the main unit of elastin.
- Elastin's elastic properties stem from the formation of desmosine, a molecule created from the interaction of three allysine residues and one unoxidized lysine.
- Elastin exhibits fibrous shape when extended and a globular shape when relaxed.
α-1 Antitrypsin Deficiency
- α-1 antitrypsin is a protein that protects tissues from elastase enzymes released by inflammatory cells like neutrophils.
- During acute inflammation, such as bacterial infection, the body releases proteinases like trypsin and elastase.
- In the absence of α-1 antitrypsin, neutrophil elastase breaks down elastin, damaging lung elasticity and leading to respiratory complications like emphysema, COPD, and cirrhosis of the liver.
Fibronectin
- Fibronectin is a major protein of the extracellular matrix (ECM).
- It's found in connective tissue and plasma.
- Fibronectin consists of two identical subunits connected by two disulfide bonds and has several binding sites.
- It binds to heparin, collagen, and cell surface receptors like integrin.
- Fibronectin's adhesive characteristics allow it to bind to transmembrane receptors like integrin, contributing to cell communication between the extracellular and intracellular environments.
- Fibronectin glomerulopathy is a genetic disorder caused by a mutation in the fibronectin gene, leading to abnormal fibronectin production in kidney glomeruli and impaired glomerular function.
- Fibronectin glomerulopathy can manifest as hematuria, proteinuria, hypertension, renal tubular acidosis, and renal failure.
Fibrillin
- Fibrillin is a glycoprotein secreted into the ECM by fibroblasts.
- It is found in the suspensory ligaments of the lens and the aorta.
- Fibrillin forms the scaffold for elastin deposition.
- Marfan syndrome is caused by a mutation in the gene encoding fibrillin, leading to various complications.
- Marfan syndrome can lead to lens dislocation, aortic dilation, and joint hyperextensibility.
Cartilage Proteins
- Cartilage is avascular connective tissue containing collagen and non-collagenous proteins.
- Collagen type II is the main collagen protein in cartilage, accounting for 98% of the total collagen content.
- Proteoglycans, particularly aggrecans containing glycosaminoglycans (GAGs), are the most important non-collagenous proteins in cartilage.
- GAGs in cartilage have a bottle brush appearance, with three GAGs attached to a core protein.
- Chondronectin, a binder protein, binds to type II collagen in cartilage.
Bone Proteins
- Bone is mineralized connective tissue containing collagen and non-collagenous proteins.
- Collagen type I is the most important collagen protein in bone, making up 90% of the total collagen content.
- Osteocalcin is the major non-collagenous protein in bone.
- Osteonectin, a binder protein, binds to collagen in bone.
Osteoporosis
- Osteoporosis is a progressive condition characterized by reduced bone mass and density.
- Risk factors for osteoporosis include: aging, female sex, family history, sedentary lifestyle, caffeine and soda consumption, low intake of calcium, magnesium, and vitamin D, smoking, and corticosteroid use.
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