title: Selective vulnerability in amyotrophic lateral sclerosis: no evidence for a contribution of annexins, a family of calcium binding proteins.
abstract: Clinically, amyotrophic lateral sclerosis (ALS) usually presents as a pure motor system disorder, whereas oculomotor and sphincter muscle control of the anus and the bladder appear to be spared. Previously, a lacking expression of calcium binding proteins (CBPs) was demonstrated in vulnerable motor neurons in contrast to spared neuronal populations, e.g., the motor neurons of the cranial nerve III (NO) and the Onufrowicz nucleus (ON), suggesting a potential role of CBPs in the selective motoneuronal vulnerability in ALS. The annexins comprise a multigene family of CBPs, constituting a significant amount of total cellular protein and presumably involved in calcium-homeostasis and intracellular calcium-regulated pathways. We immunohistochemically investigated the expression patterns of annexins A1, A2, A4, A5, A6, and A7 in spinal cord and midbrain tissues from 24 ALS patients and 5 age-matched controls to test the hypothesis that annexins also contribute to the selective vulnerability in ALS. There was no difference in the expression patterns of ALS cases and normal controls. Annexin A1 was expressed in ependymal cells and motor neurons. Annexin A2 could be detected in ependymal and endothelial cells and motor neurons. Annexins A4 and A5 were found in both ependymal and glial cells, whereas annexin A6 was strongly expressed in motor neurons. Annexin A7 was totally absent from central nervous system tissue. A contribution of annexins to the selective vulnerability in ALS could not be derived from our results.
authors.affiliation: Centre of Neuromuscular Disorders, Department of Neurology Friedrich-Alexander-University Erlangen-Nuremberg Schwabachanlage 6, D-91054 Erlangen, Germany. stefan.probst-cousin@neuro.imed.uni-erlangen.de
authors.forename: Stefan
authors.initials: S
authors.lastname: Probst-Cousin
authors.forename: Markus
authors.initials: M
authors.lastname: Bergmann
authors.forename: Christian
authors.initials: C
authors.lastname: Maihöfner
authors.forename: Bernhard
authors.initials: B
authors.lastname: Neundörfer
authors.forename: Dieter
authors.initials: D
authors.lastname: Heuss
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism*; D017302:Annexins / Q000145:classification* / Q000378:metabolism*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry / Q000379:methods; D008297:Male; D008875:Middle Aged; D009457:Neuroglia / Q000378:metabolism; D009474:Neurons / Q000378:metabolism; D013116:Spinal Cord / Q000166:cytology / Q000378:metabolism*
chemical_list: D017302:Annexins