title: Pigment epithelium-derived factor is elevated in CSF of patients with amyotrophic lateral sclerosis.
abstract: 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.
authors.affiliation: Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland 21218, USA. rkuncl1@jhu.edu
authors.forename: Ralph W
authors.initials: RW
authors.lastname: Kuncl
authors.forename: Masako M
authors.initials: MM
authors.lastname: Bilak
authors.forename: Stephan R
authors.initials: SR
authors.lastname: Bilak
authors.forename: Andrea M
authors.initials: AM
authors.lastname: Corse
authors.forename: Walter
authors.initials: W
authors.lastname: Royal
authors.forename: S Patricia
authors.initials: SP
authors.lastname: Becerra
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000134:cerebrospinal fluid; D015153:Blotting, Western; D003720:Densitometry; D005136:Eye Proteins; D005260:Female; D006801:Humans; D016014:Linear Models; D008297:Male; D008875:Middle Aged; D009414:Nerve Growth Factors; D019636:Neurodegenerative Diseases / Q000134:cerebrospinal fluid*; D011506:Proteins; D015203:Reproducibility of Results; D015843:Serpins / Q000134:cerebrospinal fluid*; D015996:Survival Rate; D000099174:Pigment Epithelium-Derived Factor
chemical_list: D005136:Eye Proteins; D009414:Nerve Growth Factors; D011506:Proteins; D015843:Serpins; D000099174:Pigment Epithelium-Derived Factor