title: Cobalamin as a regulator of serum and cerebrospinal fluid levels of normal prions.
abstract: We have previously demonstrated that the concentration of normal prion proteins (PrP(C)) is increased in the serum and cerebrospinal fluid (CSF) of rats deficient in vitamin B(12) (cobalamin, Cbl). In this study, we investigated whether similar increases also occur in the serum and CSF of patients deficient in Cbl (Cbl-D), and whether the increase in serum levels can be corrected by Cbl therapy. The study involved two sample populations. The first consisted of 45 patients (13 patients with pernicious anemia [PA], 19 with other forms of anemia, and 13 healthy controls); and the second, 68 patients (five with subacute combined degeneration [SCD], 18 with amyotrophic lateral sclerosis, 22 with multiple sclerosis [MS], and 23 neurological controls). Serum PrP(C) levels were measured using an enzyme-linked-immunosorbent-assay before as well as after Cbl therapy. The mean serum PrP(C) levels in patients with PA were significantly higher than those of the controls (p=0.0017) but normalized after Cbl therapy; there was no significant change in the patients with other forms of anemia. Mean CSF PrP(C) levels in the patients with SCD were significantly higher than in the neurological controls (p<0.03). The serum and CSF PrP(C) levels of patients with PA and those with SCD were correlated significantly with serum (p=0.004) and CSF (p=0.0018) Cbl levels. In patients with MS, CSF PrP(C) concentrations were significantly lower than those of the controls regardless of their CSF Cbl levels. We found a correlation between Cbl and PrP(C) levels in the serum and CSF of Cbl-D patients, which suggests that Cbl may regulate the PrP(C) levels in the serum and CSF in humans.
authors.affiliation: Città Studi Department, Laboratory of Neuropathology, University of Milan, Milan, Italy. giuseppe.scalabrino@unimi.it
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Scalabrino
authors.forename: Daniela
authors.initials: D
authors.lastname: Veber
authors.forename: Chiara
authors.initials: C
authors.lastname: Briani
authors.forename: Silvano
authors.initials: S
authors.lastname: Milani
authors.forename: Antonietta
authors.initials: A
authors.lastname: Terralavoro
authors.forename: Sergio
authors.initials: S
authors.lastname: Brenna
authors.forename: Luca
authors.initials: L
authors.lastname: Valenti
authors.forename: Vincenzo
authors.initials: V
authors.lastname: Silani
authors.forename: Claudia
authors.initials: C
authors.lastname: Morelli
authors.forename: Maddalena
authors.initials: M
authors.lastname: Peracchi
mesh_terms: D000328:Adult; D000690:Amyotrophic Lateral Sclerosis / Q000097:blood / Q000134:cerebrospinal fluid / Q000188:drug therapy*; D000704:Analysis of Variance; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000097:blood / Q000134:cerebrospinal fluid / Q000188:drug therapy*; D009460:Neurologic Examination; D011328:Prions / Q000097:blood* / Q000134:cerebrospinal fluid*; D013223:Statistics as Topic; D052879:Subacute Combined Degeneration / Q000097:blood / Q000134:cerebrospinal fluid / Q000188:drug therapy*; D014805:Vitamin B 12 / Q000627:therapeutic use*; D014803:Vitamin B Complex / Q000627:therapeutic use*; D017086:beta-Thalassemia / Q000097:blood / Q000134:cerebrospinal fluid / Q000188:drug therapy
chemical_list: D011328:Prions; D014803:Vitamin B Complex; D014805:Vitamin B 12