Motor Neurons Derived From Embryonic Stem Cells Mimic The Progress Of ALS

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Motor Neurons Derived From Embryonic Stem Cells Mimic The Progress Of

Familial ALS

24 Feb 2009

http://www.medicalnewstoday.com/articles/139989.php

Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's

disease, is a devastating condition in which motor neuron

degeneration causes progressive loss of movement and muscle tone,

leading to death. Overcoming the limited success of previous models,

a report published in Disease Models & Mechanisms (DMM),

dmm.biologists.org describes how neurons can be derived from human

stem cells, and engineered to mimic inherited ALS.

Researchers at the University of California Los Angeles developed an

optimized protocol to generate motor neurons from human embryonic

stem cells (ES cells), which express normal or mutant forms of the

SOD-1 gene, which is linked to inherited, familial ALS. Resulting

cells exhibit hallmark characteristics of motor nerve cells, and

neurons expressing mutant SOD-1 display abnormalities typical of ALS.

Defects included shortened cell projections and a reduced life span

compared to cells containing the normal SOD-1 gene.

This human cell-derived model of ALS provides a new method of

studying this disease and testing novel therapeutics. This is

especially helpful as only one drug is approved to help slow ALS

progression, and animal models currently used in drug development

have had limited success. Additionally, this research may aid other

gene-linked neurodegenerative diseases, as they too may benefit from

studies in a human cell-derived model.