TY - JOUR
T1 - Progranulin
T2 - A proteolytically processed protein at the crossroads of inflammation and neurodegeneration
AU - Cenik, Basar
AU - Sephton, Chantelle F.
AU - Cenik, Bercin Kutluk
AU - Herz, Joachim
AU - Yu, Gang
PY - 2012/9/21
Y1 - 2012/9/21
N2 - GRN mutations cause frontotemporal lobar degeneration with TDP-43-positive inclusions. The mechanism of pathogenesis is haploinsufficiency. Recently, homozygous GRN mutations were detected in two patients with neuronal ceroid lipofuscinosis, a lysosomal storage disease. It is unknown whether the pathogenesis of these two conditions is related. Progranulin is cleaved into smaller peptides called granulins. Progranulin and granulins are attributed with roles in cancer, inflammation, and neuronal physiology. Cell surface receptors for progranulin, but not granulin peptides, have been reported. Revealing the cell surface receptors and the intracellular functions of granulins and progranulin is crucial for understanding their contributions to neurodegeneration.
AB - GRN mutations cause frontotemporal lobar degeneration with TDP-43-positive inclusions. The mechanism of pathogenesis is haploinsufficiency. Recently, homozygous GRN mutations were detected in two patients with neuronal ceroid lipofuscinosis, a lysosomal storage disease. It is unknown whether the pathogenesis of these two conditions is related. Progranulin is cleaved into smaller peptides called granulins. Progranulin and granulins are attributed with roles in cancer, inflammation, and neuronal physiology. Cell surface receptors for progranulin, but not granulin peptides, have been reported. Revealing the cell surface receptors and the intracellular functions of granulins and progranulin is crucial for understanding their contributions to neurodegeneration.
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U2 - 10.1074/jbc.R112.399170
DO - 10.1074/jbc.R112.399170
M3 - Short survey
C2 - 22859297
AN - SCOPUS:84866536050
SN - 0021-9258
VL - 287
SP - 32298
EP - 32306
JO - Journal of Biological Chemistry
JF - Journal of Biological Chemistry
IS - 39
ER -