TY - JOUR
T1 - Pigment epithelium-derived factor is elevated in CSF of patients with amyotrophic lateral sclerosis
AU - Kuncl, Ralph W.
AU - Bilak, Masako M.
AU - Bilak, Stephan R.
AU - Corse, Andrea M.
AU - Royal, Walter
AU - Becerra, S. Patricia
PY - 2002
Y1 - 2002
N2 - Pigment epithelium-derived factor (PEDF), a recently defined retinal trophic factor and anti-angiogenic factor for the eye, is also present in the CNS and is a motor neuron protectant. We asked whether PEDF levels in CSF are altered in patients with amyotrophic lateral sclerosis (ALS). Pigment epithelium-derived factor protein was detected by quantitative western blot analysis with a PEDF-specific antiserum. Levels of PEDF in CSF, expressed as a ratio to total CSF protein, were significantly elevated 3.4-fold in 15 patients with ALS compared with neurologic disease controls (p < 0.0003). This increase does not seem likely to reflect up-regulation of PEDF synthesis in muscle in response to denervation, as CSF PEDF was not elevated in severe denervating diseases other than ALS. Nor does the increase represent some non-specific release in neurodegeneration, as CSF PEDF was not elevated in other neurodegenerative diseases. While the mechanism of this presumably reactive increase is not known, the distinctive, surprisingly elevated level of PEDF in the CSF may be an autoprotective reaction in ALS.
AB - Pigment epithelium-derived factor (PEDF), a recently defined retinal trophic factor and anti-angiogenic factor for the eye, is also present in the CNS and is a motor neuron protectant. We asked whether PEDF levels in CSF are altered in patients with amyotrophic lateral sclerosis (ALS). Pigment epithelium-derived factor protein was detected by quantitative western blot analysis with a PEDF-specific antiserum. Levels of PEDF in CSF, expressed as a ratio to total CSF protein, were significantly elevated 3.4-fold in 15 patients with ALS compared with neurologic disease controls (p < 0.0003). This increase does not seem likely to reflect up-regulation of PEDF synthesis in muscle in response to denervation, as CSF PEDF was not elevated in severe denervating diseases other than ALS. Nor does the increase represent some non-specific release in neurodegeneration, as CSF PEDF was not elevated in other neurodegenerative diseases. While the mechanism of this presumably reactive increase is not known, the distinctive, surprisingly elevated level of PEDF in the CSF may be an autoprotective reaction in ALS.
KW - Amyotrophic lateral sclerosis
KW - Cerebrospinal fluid
KW - Neurotrophic factors
KW - Pigment epithelium-derived factor
KW - Serine protease inhibitors
KW - Spinal cord
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U2 - 10.1046/j.1471-4159.2002.00813.x
DO - 10.1046/j.1471-4159.2002.00813.x
M3 - Article
C2 - 12067231
AN - SCOPUS:0036074844
SN - 0022-3042
VL - 81
SP - 178
EP - 184
JO - Journal of Neurochemistry
JF - Journal of Neurochemistry
IS - 1
ER -