| First Author | Pandey MK | Year | 2014 |
| Journal | Mol Genet Metab | Volume | 111 |
| Issue | 2 | Pages | 163-71 |
| PubMed ID | 24079945 | Mgi Jnum | J:211409 |
| Mgi Id | MGI:5575421 | Doi | 10.1016/j.ymgme.2013.09.002 |
| Citation | Pandey MK, et al. (2014) Gaucher disease: chemotactic factors and immunological cell invasion in a mouse model. Mol Genet Metab 111(2):163-71 |
| abstractText | Gaucher disease results from mutations in GBA1 that cause functional disruption of the encoded lysosomal enzyme, acid beta-glucosidase. The consequent excess accumulation of glucosylceramide and glucosylsphingosine in lysosomes is central to the disease pathogenesis with classical involvement of macrophage (Msmall ef, Cyrillics) lineage cells of visceral organs, bone, or brain. Several studies have implicated the increased secretion of chemokines and infiltration of a variety of immunological cells into tissues of Gaucher disease patients. Trafficking of immunological cells to the sites of inflammation requires the presence of chemokines. Although increases of different immunological cells and several chemokines are present in Gaucher disease, the specific chemoattractants that cause the increased influx of immunological cells are not fully defined. Here, increased levels of I-309, MCP-5, CXCL-2, CXCL-9, CXCL-10, CXCL-11, CXCL-13, and their corresponding leukocytes, i.e., MOs (monocytes), Msmall ef, Cyrillics, dendritic cells (DCs), polymorphonuclear neutrophils (PMNs), and T, and B cells were identified in the circulation of mice with Gba1 mutations (D409V/null). Sera from D409V/null mice contained chemoattractants for a variety of immunological cells as shown by ex vivo chemotaxis studies and by flow cytometry. Enhanced chemotaxis towards 9V/null sera was found for 9V/null lung-, spleen-, liver-, and bone marrow-derived Msmall ef, Cyrillics (CD11b(+) F480(+)), PMNs (Gr1(high) CD11b(+)), DCs (CD11c(+) CD11b(+)), T lymphocytes (CD3(+) TCRB(+)), and B lymphocytes (B220(+) CD19(+)). These data support these chemotactic factors as causative to increased tissue infiltration of leukocytes in Gaucher disease. |
