Human biochem lecture 10

Prion Disease

A spectrum of infectious proteinopathies caused by misfolded proteins, leading to various neurological diseases.

PrP(C)

The normal variant of prion proteins that can be converted into the infectious form (PrP(Sc)).

Alpha Helices

A common secondary structure in proteins characterized by a right-handed coiling formed by hydrogen bonding between amino acid residues.

Beta Sheets

Secondary structures formed by hydrogen bonds between adjacent strands, which can run in parallel or antiparallel directions.

Kuru

A well-known prion disease historically associated with ritualistic cannibalism.

sCJD

Spontaneous Creutzfeldt-Jakob disease, which is one of the most common human prion diseases.

Fibrous Proteins

Proteins characterized by elongated structures and primarily secondary formations, such as keratin, fibroin, and collagen.

Collagen

The most abundant fibrous protein in animals, forming connective tissues and composed of a triple helix structure.

Ramachandran Plots

Graphs that depict the allowed angles of rotation between bonds in a protein structure, helping to predict secondary structural elements.

Tertiary Structure

The three-dimensional structure of a protein formed by folding and interactions between secondary structures.

Quaternary Structure

The assembly of multiple folded polypeptide chains into a complex protein structure.

310 Helix

A less common type of helix in proteins, stabilized by hydrogen bonds between three residues per turn.

Steric Clashing

Occurs when atoms within a molecule are too close together, resulting in an unstable conformation.

Beta Sheet Arrangement

Refers to the orientation of beta strands in beta sheets, either parallel or antiparallel.

Glycine

An amino acid often found in fibrous proteins due to its small, flexible side chain.

Proline

An amino acid that influences the stability and structure of polypeptides, particularly in helix formation.

Van der Waals Interactions

Weak attractions between molecules that contribute to the structural integrity of fibrous proteins.

Hemoglobin

A globular protein in red blood cells responsible for oxygen transport in the body.

Myoglobin

A globular protein that stores oxygen in muscle cells.

Destruction/Degradation Enzymes

Proteins that help in breaking down unnecessary or harmful substances in biological systems.

Neurotransmitter Receptors

Proteins that receive chemical signals from neurotransmitters in the nervous system.

Familial Fatal Insomnia (FFI)

A genetic prion disease characterized by debilitating insomnia and eventual death.