A first-stop reference on proteins associated with amyloidosis.
This book is the first to present a systematic overview of all known fibril-forming proteins, including their biochemical characteristics and pathophysiology. It considers the clinically recognized amyloid proteins that are known to be associated with the amyloid protein folding disorders, dealing with their common structural and thermodynamic features that lead to amyloid fibril formation and disease. Emphasis is on the thermodynamics of protein folding, the structure and physiologic effects of common oligomeric and subfibrillar intermediates and the influence of the extracellular matrix and cellular trafficking and metabolism on the genesis and catabolism of beta pleated sheet proteins.

The chapters on specific amyloid proteins all follow a common structure, allowing quick access to the desired biochemical and medical data, making this an invaluable tool for clinicians and researchers alike.

PART I Overview of Amyloidosis and Amyloid Proteins
Brief history of amyloidosis
Anatomic and clinical clues to in vivo mechanisms of amyloidogenesis

PART II Protein Structure and the Beta Pleated Sheet Conformation
Brief history of the beta pleated sheet conformation including glossary of terms

PART III Protein Folding, Unfolding and Refolding
Thermodynamics and protein folding
The role of post-translational chemical modifications in amyloid fibril formation
Lipid modulators of protein misfolding and aggregation
Extracellular matrix-heparan sulfate proteoglycans
Serum amyloid P component: Structural features and amyloid recognition
Apolipoprotein E

PART IV Pathways to Amyloid Fibril Formation
Partially folded intermediates in the fibrillation of natively unfolded proteins
Partially folded intermediates, random coil
Chemical interactions in amyloid fibril formation

PART V Pathophysiology of Amyloid Fibril Formation
Oligomers and cellular toxicity
Fibrils and organ dysfunction
Brain dysfunction associated with amyloid fibrils and other aggregated proteins

PART VI Amyloid Proteins
Beta amyloid protein
Prion protein
Familial dementia
Immunoglobulin
Transthyretin
Apolipoprotein, AApoAII and Amyloid A
Gelsolin
Lysozyme
Fibrinogen
Beta-2-microglobulin
Cystatin C
IAPP and Others