Fibrous Proteins- Keagan (week 3) Flashcards

1
Q

Structure of collagen

A

Right-handed triple helix of alpha-chains

Most abundant protein in the body

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2
Q

What are alpha chains and their repeats

A

Individual poly peptides composing tropocollagen

GLY-X-Y repeats

Proline and hydroxyproline/hydroxylysine are usually X and Y

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3
Q

Three polypeptide alpha chains of collagen held together by

A

Hydrogen bonds

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4
Q

Type I collagen
Composition
Tissues
Genes

A

Composition- a1[I], a2[I]
Tissues- skin, bone, cornea, tendons
Genes- COL1A1, COL1A2

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5
Q

Type II collagen

Composition
Tissues
Genes

A

Composition- a1[II]
Tissues- cartilage, intervertebral disks, vitreous body
Genes- COL2A1

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6
Q

Type III collagen

Composition
Tissues
Genes

A

Composition- a1[III]
Tissues- blood vessels, dermis
Genes- COL3A1

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7
Q

Type IV collagen

Composition
Tissues
Genes

A

Composition a1, a2, a3, a4, a5, a6
Tissues- basement membranes
Genes- COL4A1,2,3,4,5,6

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8
Q

Type V collagen

Composition
Tissues
Genes

A

Composition- a1 or a1[V]a2[V]
Tissues- interstitial tissue (associated with type I)
Genes- COL5A1,2,3

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9
Q

Collagen made by what three cells

A

Osteoblasts
Chondroblasts
Fibroblasts

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10
Q

Biosynthesis of collagen

A

Idk watch video or something

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11
Q

Vitamin C importance
Deficiency causes what

A

Reducing agent (coenzyme) to hydroxylate PRO and LYS
PRO—> PRO-OH

Deficiency causes scurvy

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12
Q

Osteogenesis imperfecta

A

Type I collagen disorder (mutation of type I collagen)
-brittle bone disease, fractures, blue sclera, hearing loss

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13
Q

Ehlers-danlos syndrome

A

Hyperelasticity of skin, hyper extensive joints, rupture of colon, skin hemorrhage

Mutations of type I, III, V collagen

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14
Q

Menkes Disease

A

X-linked recessive
Deficient lysyl oxidase (cross linking)
Copper deficiency

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15
Q

Synthesis of elastin

A

Tropoelastin + fibrillin-1 (microfibrillar proteins)

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16
Q

Lysyl oxidase importance

A

Forms interchain cross link of collagen and elastin
Without it= menkes disease

17
Q

Emphysema

A

Excess air in lung
Mutations in A1AT

Elastin and fibrillin defect

18
Q

Marfans syndrome

A

Elongation of bones, ocular & cardiovascular abnormalities

Mutations in fibrillin-1 (maintenance of elastic fibers)

19
Q

What is keratin

A

Tough fibers (hair, nails, outer epidermis)
Rich in cysteine

20
Q

Keratin structure

A
  1. Two a-helices form protofilaments
    Protofilaments —> intermediate filaments—> microfibrils—> macro fibrils—> a single cell
21
Q

What causes hair to curl

A

Oxidation of disulfide bonds in keratin

22
Q

Epidermolysis bullosa simplex

A

Keratin-related disease
Blister formation in hands
Mutations of genes encoding keratin 5 or keratin 14