Increased progranulin in the skin of amyotrophic lateral sclerosis: An immunohistochemical study

• Published in: Journal of the neurological sciences Vol. 309; no. 1; pp. 110 - 114
• Main Authors: Yasui, Kanako, Oketa, Yoshihiko, Higashida, Kazuhiro, Fukazawa, Hiroyuki, Ono, Seiitsu
• Format: Journal Article
• Language: English
• Published: Amsterdam Elsevier B.V 15.10.2011; Elsevier
• Subjects: Adult; Aged; Amyotrophic lateral sclerosis; Amyotrophic Lateral Sclerosis - diagnosis; Amyotrophic Lateral Sclerosis - metabolism; Biological and medical sciences; Biomarkers - metabolism; Epidermis; Female; Humans; Immunohistochemistry; Intercellular Signaling Peptides and Proteins - biosynthesis; Male; Medical sciences; Middle Aged; Multiple sclerosis and variants. Guillain barré syndrome and other inflammatory polyneuropathies. Leukoencephalitis; Neurology; Progranulin; Protein Precursors - biosynthesis; Skin; Skin - chemistry; Skin - metabolism; Up-Regulation - physiology; Immunohistochemistry; Amyotrophic lateral sclerosis; Epidermis; Skin; Progranulin; Nervous system diseases; Central nervous system disease; Degenerative disease; Spinal cord disease
• ISSN: 0022-510X; 1878-5883; 1878-5883
• DOI: 10.1016/j.jns.2011.07.003

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.