Protein Structure and Function Flashcards
Specialized group of proteins required for the PROPER FOLDING OF MANY SPECIES OF PROTEINS
Prevents aggregation and misfolding
Chaperones
Due to UNFOLDING AND DISORGANIZATION OF A PROTEIN
Denaturation
does NOT affect primary structure
Globular Proteins
hemoglobin myoglobin albumin globulin most enzymes
spherical
MORE soluble
has DYNAMIC functions
Fibrous Proteins
collagen fibers
elastin fibers
keratin
elongated or needle shaped
MINIMALLY soluble
STRUCTURAL proteins
Most abundant protein in the body
Collagen
3 polypeptides (alpha-chains) wound around in triple helix formation (stabilized by hydrogen bonds)
MC form of collagen
Type 1 collagen
Usual pattern of AA in collagen
Gly - glycine
X - proline
Y - hydroxyproline, hydroxylysine
Type I collagen
BONE
OI
Type 2 collagen
Cartilage
Achondrogenesis type II
Type 3 collagen
Reticulin fibers, blood vessels, granulation tissue
Ehlers Danlos (vascular type) aortic rupture, aortic aneurysms
Type 4 collagen
Basement Membrane
Alport Syndrome
Goodpasture Syndrome
Type 5 collagen
bone, skin, fetal tissues, placenta
Ehler Danlos (classical type)
Type 7 collagen
Anchoring fibrils
Dystrophic epidermolysis bullosa
Connective tissue with rubber like properties allowing extensibility and elastic recoil
Elastin
non hydroxylated PROLINE, LYSINE - major AA
Scaffold for the synthesis of elastin fibers
FIBRILLIN
Cross links precursor tropoelastin together
DESMOSINE
Defective in Marfan Syndrome
skeletal change aortic dissection ectopic lentis (upward, outward)
Fibrillin
Triad of Marfan’s Syndrome
Skeletal changes
Ectopic lentis - upward and temporal subluxatiob
aortic aneurysm
FIBRILLIN 1
zonular fibers in the lens
periosteum
aorta
International Criteria to classify Marfan syndrome
Ghent Criteria
Mutation in TGR Beta Receptors
Characterized by aortic aneurysms, cleft palate, hypertelorism
Loeys-Dietz
Alpha helix coiled structure wind arpund each other to form a super helix
Rich in HYDROPHOBIC AA - Ala, Leu, Met, Val, Phe
KERATIN
Major protein component of Renal Glomerular and other Basal Laminas
LAMININ