title: Alteration of Motor Protein Expression Involved in Bidirectional Transport in Peripheral Blood Mononuclear Cells of Patients with Amyotrophic Lateral Sclerosis.
abstract: BACKGROUND
  Sporadic amyotrophic lateral sclerosis (SALS) is a fatal motor neuron degenerative disease of unclear pathogenesis. Disturbances of intracellular transport are possible causes of the disease.
  
  
  OBJECTIVE
  We evaluated the expression of motor proteins involved in the anterograde (kinesins KIF1B, KIF5C) and retrograde (KIFC3, dynactin subunits DCTN1 and DCTN3) intracellular transport in peripheral blood mononuclear cells (PBMCs).
  
  
  METHODS
  PBMCs were obtained from 74 SALS patients with different clinical phenotypes, 65 blood donors (healthy control I), and 29 cases with other neurological diseases (disease control II) divided into subgroups IIA (atypical parkinsonism) and IIB (ALS-mimicking disorders). mRNA expression was studied by real-time qPCR, and protein level by Western blotting.
  
  
  RESULTS
  In SALS, KIF5C and KIFC3 expression was significantly lower and DCTN1 higher than in control I, and dependent of age. KIF1B expression was significantly higher in SALS than in subgroup IIB, whereas DCTN1 and DCTN3 were higher in SALS than in subgroup IIA. All changes in the studied proteins were statistically significant in classic ALS but not in progressive muscular atrophy.
  
  
  CONCLUSIONS
  In SALS, and especially in classic ALS, the changes in motor protein expression may alter bidirectional intracellular transport in PBMCs. More studies are needed to find out whether the levels of KIF5C and DCTN1 may be useful in ALS diagnosis, and whether KIF1B expression may discriminate ALS from ALS-mimicking disorders.
authors.affiliation: Department of Neurology, Medical University of Warsaw, Warsaw, Poland.
authors.forename: Magdalena
authors.initials: M
authors.lastname: Kuźma-Kozakiewicz
authors.forename: Beata
authors.initials: B
authors.lastname: Kaźmierczak
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Chudy
authors.forename: Beata
authors.initials: B
authors.lastname: Gajewska
authors.forename: Anna
authors.initials: A
authors.lastname: Barańczyk-Kuźma
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000375:Aging / Q000378:metabolism; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000378:metabolism*; D015415:Biomarkers / Q000097:blood; D015153:Blotting, Western; D000072159:Dynactin Complex / Q000378:metabolism*; D005260:Female; D006801:Humans; D016547:Kinesins / Q000378:metabolism*; D007963:Leukocytes, Mononuclear / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D012333:RNA, Messenger / Q000378:metabolism; D060888:Real-Time Polymerase Chain Reaction; D055815:Young Adult
chemical_list: D015415:Biomarkers; C000606360:DCTN1 protein, human; C000606362:DCTN3 protein, human; D000072159:Dynactin Complex; C091053:KIF1B protein, human; C586505:Kifc3 protein, human; D012333:RNA, Messenger; C514435:KIF5C protein, human; D016547:Kinesins