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June 22, 2005

Faulty enzyme sparks Parkinson's disease

Topics: Medicine

alpha-Synuclein is a major component of Lewy bodies, the pathological hallmark of Parkinson disease, dementia with Lewy bodies, and related disorders. Misfolding and aggregation of alpha-synuclein is thought to be a critical cofactor in the pathogenesis of certain neurodegenerative diseases.

Put another way, Parkinson disease and other alpha-synucleinopathies are characterized by the deposition of intraneuronal alpha-synuclein (alphaSyn) inclusions, or a clumping, involving "accumulation of a protein in an aberrant form - according to Philip Thomas, lead researcher at the University of Texas' Southwestern Medical Center.

- UPI via Science Daily
(...) Parkinson's is "a disease involving accumulation of a protein in an aberrant form," said Philip Thomas, lead researcher at the University of Texas'

(...) Normally, the protein involved in Parkinson's, alpha-synuclein, unfolds when it is stressed and is chewed into harmless bits by an enzyme.

(...) However, that enzyme malfunctions in Parkinson's patients and creates protein fragments that clump, and those protein clusters cause more clumps in "vicious cycle" of the degenerative disease, scientists said.

Thomas, reporting in The Journal of Biological Chemistry(abstract), said future research may involve ways to inhibit the malicious form of the enzyme while leaving the normal form alone since the enzyme is essential for cells to survive.

Related reading:
Effects of Parkinson's disease-linked mutations on the structure of lipid-associated alpha-synuclein.

Cross-linking of ubiquitin, HSP27, parkin, and alpha-synuclein by gamma-glutamyl-epsilon-lysine bonds in Alzheimer's neurofibrillary tangles.

Cognitive status correlates with neuropathologic stage in Parkinson disease.

Cross posted at NewHopeBlog

Posted by Hyscience at June 22, 2005 9:30 PM

Will this help those who are already diagnosed with Parkinsons Disease? Does this indicate that there might be a genetic component in the disease.

My mother has Parkinsons Disease. She rarely talks about some of its effects but she told me something on the telephone the other night and my first comment happened to be "that was your Parkinsons Disease"

I get neuropathic pain and that is another reason to be wary and on the look out to find out more about this disease.

Posted by: Maggie4life at June 24, 2005 5:24 PM



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