Accelerated Lipofuscinosis and Ubiquitination in Granulin Knockout Mice Suggest a Role for Progranulin in Successful Aging

• Published in: The American journal of pathology Vol. 177; no. 1; pp. 311 - 324
• Main Authors: Ahmed, Zeshan, Sheng, Hong, Xu, Ya-fei, Lin, Wen-Lang, Innes, Amy E., Gass, Jennifer, Yu, Xin, Hou, Harold, Chiba, Shuichi, Yamanouchi, Keitaro, Leissring, Malcolm, Petrucelli, Leonard, Nishihara, Masugi, Hutton, Michael L., McGowan, Eileen, Dickson, Dennis W., Lewis, Jada
• Format: Journal Article
• Language: English
• Published: Bethesda, MD Elsevier Inc 01.07.2010; American Society for Investigative Pathology
• Subjects: Aging - physiology; Animals; Biological and medical sciences; Brain - cytology; Brain - metabolism; Brain - pathology; Errors of metabolism; Humans; Intercellular Signaling Peptides and Proteins - genetics; Intercellular Signaling Peptides and Proteins - metabolism; Investigative techniques, diagnostic techniques (general aspects); Lipids (lysosomal enzyme disorders, storage diseases); Medical sciences; Metabolic diseases; Mice; Mice, Knockout; Neuronal Ceroid-Lipofuscinoses - genetics; Neuronal Ceroid-Lipofuscinoses - metabolism; Neuronal Ceroid-Lipofuscinoses - pathology; Neurons - cytology; Neurons - metabolism; Pathology; Pathology. Cytology. Biochemistry. Spectrometry. Miscellaneous investigative techniques; Progranulins; Regular; Ubiquitin - metabolism; Ubiquitination; Progranulin; Ageing; Metabolic diseases; Enzymopathy; Lipofuscinosis; Genetic disease; Anatomic pathology; Ubiquitination; Animal; Knockout mouse; Pigments; Lipoidosis; Age

Progranulin (PGRN) is involved in wound repair, inflammation, and tumor formation, but its function in the central nervous system is unknown. Roles in development, sexual differentiation, and long-term neuronal survival have been suggested. Mutations in the GRN gene resulting in partial loss of the...