title: Increased progranulin in the skin of amyotrophic lateral sclerosis: an immunohistochemical study.
abstract: It has been demonstrated that progranulin (PGRN) is a neurotrophic factor that enhances neuronal survival and axonal growth. Several lines of evidence have indicated that PGRN plays a role in the pathomechanism of amyotrophic lateral sclerosis (ALS). However, there has no study of PGRN in ALS skin. We made a quantitative immunohistochemical study of the expression of PGRN in the skin from 18 patients with sporadic ALS and 13 control subjects. Immunohistochemistry for PGRN demonstrated cytoplasmic activity in the epidermis and in some blood vessels and glands. Numerous PGRN-positive (PGRN+) cells were observed in the epidermis in ALS patients, which became more marked as ALS progressed. PGRN immunoreactivity of PGRN+cells was markedly positive in the epidermis in ALS patients. The proportion of PGRN+cells in the epidermis in ALS patients was significantly higher (p<0.001) than in controls. There was a significant positive relationship (r = 0.83, p<0.001) between the proportion and duration of illness in ALS patients. These data suggest that changes of PGRN in ALS skin are related to the disease process and that metabolic alteration of PGRN may take place in the skin of patients with ALS.
authors.affiliation: Department of Neurology, Teikyo University Chiba Medical Center, 3426–3 Anesaki, Ichihara, Japan.
authors.forename: Kanako
authors.initials: K
authors.lastname: Yasui
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Oketa
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Higashida
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Fukazawa
authors.forename: Seiitsu
authors.initials: S
authors.lastname: Ono
mesh_terms: D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000378:metabolism*; D015415:Biomarkers / Q000378:metabolism; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D036341:Intercellular Signaling Peptides and Proteins / Q000096:biosynthesis*; D008297:Male; D008875:Middle Aged; D000077153:Progranulins; D011498:Protein Precursors / Q000096:biosynthesis*; D012867:Skin / Q000737:chemistry / Q000378:metabolism*; D015854:Up-Regulation / Q000502:physiology
chemical_list: D015415:Biomarkers; C492314:GRN protein, human; D036341:Intercellular Signaling Peptides and Proteins; D000077153:Progranulins; D011498:Protein Precursors