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progranulin
Function:
- role in wound healing
- skin injury stumulates progranulin mRNA production
- increases proliferation of fibroblasts in culture
- increases proliferation of endothelial cells in culture
- increases endothelial cell migration through collagen
- applied to wounds, progranulin increases numbers of:
a) neutrophils
b) macrophages
c) fibroblasts
- stimulates angiogenesis
- does not accelerate wound healing
Compartment: secreted
Alternative splicing: named isoforms=3
Expression:
- expressed in myelogenous leukemic cell lines of promonocytic, promyelocytic, & proerythroid lineage
- expressed in fibroblasts
- strong expression in epithelial cell lines
- present in inflammatory cells & bone marrow
- highest levels in kidney
Pathology:
- mutations in gene associated with frontotemporal dementia, ubiquitin-positive
- defects associated with neuronal ceroid lipofuscinosis 11
Laboratory:
- GRN gene mutation
Related
progranulin gene
General
glycoprotein
peptide precursor
secreted protein
Properties
SIZE: entity length = 593 aa
COMPARTMENT: extracellular compartment
MOTIF: N-glycosylation site {N118}
cysteine-rich region {C126-C416} (12)
proteolytic site
N-glycosylation site {N530}
PRECURSOR-FOR: granulin
Database Correlations
OMIM correlations
MORBIDMAP 138945
UniProt P28799
Pfam PF00396
Entrez Gene 2896
Kegg hsa:2896
References
- Journal Watch 23(6):51, 2003
He Z et al, Nat Med 9:225, 2003
- Baker M et al,
Mutations in progranulin cause tau-negative frontotemporal
dementa linked to chromosome 17.
Nature 2006, 442:916
PMID: 16862116
- Crus M et al,
Null mutations in progranulin cause ubiquitin-positive
frontotemporal dementia linked to chromosome 17q21
Nature 2006, 442:920
PMID: 16862115
- UniProt :accession P28799
- Atlas of Genetics & Cytogenetics in Oncology & Haematology
http://atlasgeneticsoncology.org/genes/GRNID40757ch17q21.html