Lecture 7 Fibrous Proteins and Connective Tissues (CHATGPT)
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49 Terms
Q: What is the primary function of fibrous proteins?
To provide strength and/or flexibility to structures.
Q: Why are fibrous proteins water-insoluble?
Due to their high concentration of hydrophobic residues.
Q: What secondary structure is typical of α-keratin?
α-helix cross-linked by disulfide bonds.
Q: What fibrous protein is found in hair, feathers, and nails
alpha-keratin
Q: What is the structure of collagen?
A triple helix of three polypeptides.
Q: What makes collagen fibers strong?
Covalent cross-links involving lysine, hydroxylysine, or histidine.
Q: What protein is predominantly found in silk?
Fibroin.
Q: What is the secondary structure of fibroin?
Predominantly β-conformation.
Q: What residues are common in fibroin
Ala and Gly
Q: What role do hydrophobic residues play in α-keratin?
They stabilize the protein through disulfide bonds.
Q: Where is collagen type I found?
Skin, tendon, vascular ligature, organs, bone
Q: What is the function of collagen type II?
It is found in cartilage.
Q: What condition is caused by mutations in type I collagen?
Osteogenesis imperfecta.
Q: What mutation is suspected to cause chondrodysplasias?
Type II collagen mutation.
Q: What condition is associated with a mutation in type III collagen?
Ehlers-Danlos Syndrome.
Q: What vitamin is crucial for collagen synthesis?
Vitamin C.
Q: What disease results from a deficiency in vitamin C?
Scurvy.
Q: What is the molecular defect in Alport syndrome?
Mutations in type IV collagen, affecting kidneys, eyes, and hearing
Q: What is the role of collagen in the sclera?
It provides strength due to its random fiber interweaving.
Q: What is the secondary structure of the collagen triple helix?
Left-handed repeating tripeptide structure.
Q: What are the main components of connective tissue?
Cells, fibers, and a gel-like ground substance
Q: What is the function of connective tissue?
Provides support, protection, and structure to tissues and organs
Q: What is connective tissue proper?
It includes loose and dense connective tissues found in various body regions.
Q: What is an example of dense regular connective tissue?
Tendons, which have ordered collagen patterns
Q: What is a key characteristic of Marfan syndrome?
Mutations in fibrillin-1 leading to elongated limbs and hyperextendable joints.
Q: What condition is caused by a mutation in fibrillin-1?
Marfan syndrome.
Q: What autoimmune disease affects tear and saliva glands?
Sjögren’s syndrome.
Q: What is a key symptom of ankylosing spondylitis?
Fusion of the spine and sacroiliac joints.
Q: What genetic mutation is responsible for Ehlers-Danlos syndrome?
Mutations in COL5A1 and COL5A2.
Q: What tissue type stores fat and helps repair damaged tissue?
Connective tissue.
Q: What is amyloid fiber?
A misfolded protein that forms insoluble extracellular fibers.
Q: What conditions are associated with amyloid diseases?
Type 2 diabetes, Alzheimer’s, Huntington’s, and Parkinsons diseases
Q: What neurodegenerative disease involves amyloid-β deposition?
Alzheimer’s disease.
Q: What protein is involved in Parkinson’s disease?
Misfolded α-synuclein forms Lewy bodies.
Q: What disease involves intracellular aggregation of huntingtin protein?
Huntington’s disease.
Q: What is the primary cause of cystic fibrosis?
Defects in the cystic fibrosis transmembrane conductance regulator (CFTR) protein.
Q: What type of disease is cystic fibrosis?
A genetic disorder caused by protein misfolding.
Q: What are prions?
Misfolded proteins that cause other proteins to misfold, leading to prion diseases
Q: What is an example of a prion disease in humans?
Creutzfeldt-Jakob disease.
Q: What is a hallmark feature of prion diseases?
Formation of transmissible spongiform encephalopathies.
What are examples of Connective tissue?
Bone, Cartilage, Fat, Blood and Lymphatic tissue
What does connective tissue do?
Provides support, protection, and structure to other tissues and organs
What are the types of connective tissue proper?
Loose (Areolar)-lots of fibroblasts
Dense (Irregular)-Dermis, predominantly collagen
What are the types of specialized connective tissue?
Dense (regular)-tendons
Cartilage, bone, adipose tissue, blood, hematopoietic tissue
What are the big Connective Tissue Diseases?
Marfan syndrome, Ehlers-Danlos syndrome, Osteogenesis imperfecta, Sjogren syndrome, Ankylosing spondylitis, Psoriatic arthritis, Reactive arthritis
What is the CRAP gene?
HLA-B27
Where is type I collagen found?
Skin, tendon, vascular ligature, organs, bone
Where is type II collagen found?
Cartilage
Where is type V collagen found?
Cell surfaces, hair, and placenta