Progranulin

A proteolytically processed protein at the crossroads of inflammation and neurodegeneration

Basar Cenik, Chantelle F. Sephton, Bercin Kutluk Cenik, Joachim Herz, Gang Yu

Research output: Contribution to journalArticle

104 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)32298-32306
Number of pages9
JournalJournal of Biological Chemistry
Volume287
Issue number39
DOIs
StatePublished - Sep 21 2012

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Inflammation
Cell Surface Receptors
Proteins
Ceroid
Frontotemporal Lobar Degeneration
Neuronal Ceroid-Lipofuscinoses
Lysosomal Storage Diseases
Haploinsufficiency
Peptides
Mutation
Physiology
granulin precursor protein
Neoplasms

ASJC Scopus subject areas

  • Biochemistry
  • Cell Biology
  • Molecular Biology

Cite this

Progranulin : A proteolytically processed protein at the crossroads of inflammation and neurodegeneration. / Cenik, Basar; Sephton, Chantelle F.; Cenik, Bercin Kutluk; Herz, Joachim; Yu, Gang.

In: Journal of Biological Chemistry, Vol. 287, No. 39, 21.09.2012, p. 32298-32306.

Research output: Contribution to journalArticle

Cenik, Basar ; Sephton, Chantelle F. ; Cenik, Bercin Kutluk ; Herz, Joachim ; Yu, Gang. / Progranulin : A proteolytically processed protein at the crossroads of inflammation and neurodegeneration. In: Journal of Biological Chemistry. 2012 ; Vol. 287, No. 39. pp. 32298-32306.
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