Cæruloplasmin - the Pathology

Motor Neurone Disease

Or amyotrophic lateral sclerosis (ALS), is a progressive, fatal neurological disease affecting as many as 2 people in 100,000. Copper is implicated via a mutant of superoxide dismutase. In the U.S., ALS is known as Lou Gehrig's disease due to the fact that the famous baseball player, Lou Gehrig, died from the disease in 1941. The most famous person living with ALS is noted British physicist Stephen Hawking. He has been living with ALS for more than 35 years. He is able to move only two fingers. ALS was first described in scientific literature in 1869 by the French neurologist Jean-Martin Charcot. The French refer to ALS as Maladie de Charcot.

The Motor Neurone Disease (MND) Association.
The ALS Association.
ALS Survival Guide
LEF Site papers
See "A novel glycosylphosphatidylinositol-anchored form of ceruloplasmin is expressed by mammalian astrocytes"

by B.N.Patel and S.David, 'The GPI-anchored form of ceruloplasmin expressed by astrocytes is likely to be the major form of this molecule in the central nervous system because serum ceruloplasmin does not cross the blood-brain barrier. Lack of this form of ceruloplasmin in the central nervous system could lead to the generation of highly toxic free radicals, which can cause neuronal degeneration as seen in aceruloplasminemia and other neurodegenerative diseases such as Parkinson's and Alzheimer's disease'