Mechanisms of Disease: astrocytes in neurodegenerative disease.
The term neurodegenerative disease refers to the principal pathology associated with disorders such as amyotrophic lateral sclerosis, Alzheimer's disease, Huntington's disease and Parkinson's disease, and it is presumed that neurodegeneration results in the clinical findings seen in patients with these diseases. Decades of pathological and physiological studies have focused on neuronal abnormalities in these disorders, but it is becoming increasingly evident that astrocytes are also important players in these and other neurological disorders. Our understanding of the normative biology of astrocytes has been aided by the development of animal models in which astrocyte-specific proteins and pathways have been manipulated, and mouse models of neurodegenerative diseases have also revealed astrocyte-specific pathologies that contribute to neurodegeneration. These models have led to the development of targeted therapies for pathways in which astrocytes participate, and this research should ultimately influence the clinical treatment of neurodegenerative disorders.
Maragakis
Nicholas J
NJ
Department of Neurology, Johns Hopkins University School of Medicine, 600 N Wolfe Street, Meyer 6-119, Baltimore, MD 21287, USA. nmaragak@jhmi.edu
Rothstein
Jeffrey D
JD
D000818:Animals; D001253:Astrocytes / Q000473:pathology* / Q000502:physiology*; D004195:Disease Models, Animal; D006801:Humans; D019636:Neurodegenerative Diseases / Q000145:classification / Q000235:genetics / Q000473:pathology*