Perry, George

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Amyotrophic lateral sclerosis: a novel hypothesis involving a gained 'loss of function' in the JNK/SAPK pathway.

Xiongwei Zhu; George Perry; Mark A Smith (Profiled Authors: Smith, Mark A; Zhu, Xiongwei; Perry, George)

Institute of Pathology, Case Western Reserve University, Cleveland, Ohio 44106, USA. xxz12@po.cwru.edu
Redox report : communications in free radical research 2003;8(3):129-33.

Amyotrophic lateral sclerosis (ALS) is a progressive and fatal neurodegenerative disease that mainly affects motor neurons. Despite intensive research efforts inspired by the mile-stone discovery linking the Cu/Zn superoxide dismutase 1 (SOD1) gene to a subset of familial cases, the mechanisms underlying disease pathogenesis are still largely unknown. Nonetheless, the recent finding of a second gene associated with familial form of the disease, ALS2, is likely to be of great help in elucidating the key pathways involved in motor neuron degeneration. Here, we provide evidence that the JNK/SAPK pathway plays a critical neuroprotective role in susceptible motor neurons in ALS. The involvement of the JNK/SAPK pathway integrates our knowledge about these two known genetic factors into a single pathogenic pathway involved in both sporadic and familial ALS.

1 Originating Grant

• 1.

  Smith, Mark A

  METABOLIC ABNORMALITIES IN ALZHEIMER DISEASE

  1 May 1999 - 30 April 2006

  NATIONAL INSTITUTE OF NEUROLOGICAL DISORDERS AND STROKE

  Total Funding: $ 750,386

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