Study Guide for Fibrous Proteins and Membrane Transport

Α-keratin

Structural protein in hair, nails, and skin.

Scurvy

Disease from vitamin C deficiency, affects collagen synthesis.

Osteogenesis Imperfecta

Genetic disorder causing brittle bones due to collagen defects.

Transmissible Spongiform Encephalopathies (TSE)

Prion diseases causing neurodegeneration and spongiform changes.

Creutzfeldt-Jakob Disease (CJD)

Rapid dementia and muscle stiffness; can be inherited.

Bovine Spongiform Encephalopathy (BSE)

Mad Cow Disease affecting cattle, transmissible to humans.

Prions

Misfolded proteins causing neurodegenerative diseases.

Fibrous Proteins

Long chains of amino acids providing structural support.

Collagen

Protein with Gly-XY repeat, crucial for structure.

Hydroxyproline

Uncommon amino acid in collagen, derived from proline.

Prion Protein (PrP)

Normal brain protein that misfolds into a pathogenic form.

Hydrophobic Nature

Misfolded prions aggregate due to hydrophobic interactions.

Fluid Mosaic Model

Describes dynamic membranes with fluid lipids and proteins.

Integral Proteins

Membrane proteins embedded within the lipid bilayer.

Peripheral Proteins

Loosely attached membrane proteins, separable by pH changes.

Hydropathy Plots

Used to predict protein hydrophobic/hydrophilic regions.

Passive Diffusion

Molecule movement across membranes based on concentration gradients.

Uniport

Transport of a single substance across a membrane.

Symport

Simultaneous transport of two substances in the same direction.

Antiport

Transport of two substances in opposite directions across a membrane.

Sodium-Glucose Symporter

Uses sodium gradient to transport glucose actively.

GLUT Transporters

Facilitate passive glucose transport down concentration gradient.

Active Transport

Energy-requiring movement of substances against concentration gradient.

Na+/K+ Pump

Transports three sodium ions out, two potassium ions in.

P-type ATPases

Cation transporters phosphorylated during transport cycle.

F-type ATPases

Generate proton gradients for cellular functions.

Chloride/Bicarbonate Exchanger

Exchanges Cl- for HCO3- in red blood cells.

Amyloid Fold

Stable protein structure forming aggregates in diseases.

Gerstmann-Straussler-Scheinker Syndrome (GSS)

Rare inherited prion disease causing coordination problems.

Fatal Familial Insomnia (FFI)

Inherited disease causing severe insomnia and dementia.

Kuru

Prion disease transmitted through cannibalism, causing tremors.

Scrapie

Prion disease in sheep, causes itching and coordination loss.