New Insight Into Cause Of Amyotrophic Lateral Sclerosis

Main Category: Biology / Biochemistry
Also Included In: Muscular Dystrophy / ALS;  Genetics
Article Date: 31 May 2007 - 12:00 PDT

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Researchers have discovered a new cellular mechanism that may better explain what causes amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's Disease.

ALS is characterized by the death of neurons that control voluntary muscles, leading to muscle weakness and atrophy. Around 25 percent of the familial forms of the disease - forms that occurs repeatedly in family members but is not necessarily inherited - have been linked to mutation in a gene that makes a protein called superoxide dismutase 1 (SOD1). But how the defective variants of the SOD1 protein might interact with other cellular components to cause the disease has remained unclear.

Haining Zhu and colleagues looked at the components of proteins that interact with SOD1 and identified a protein involved in the intracellular transport of molecules in neurons. Previous studies had shown that such transport processes were slowed down in ALS patients and related animal models, but how such deficiency was connected with defective SOD1 has remained unknown. The new results show that this slower transport of molecules is probably due to an abnormal interaction between the newly-identified transport protein and defective components of SOD1, thus providing new insight into ways to prevent or slow down the disease.

Article: "Interaction between Familial Amyotrophic Lateral Sclerosis (ALS)-linked SOD1 Mutants and the Dynein Complex" by Fujian Zhang, Anna-Lena Strom, Kei Fukada, Sangmook Lee, Lawrence J. Hayward, and Haining Zhu

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