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  • severe
  • Brpf1 -deficient mice display postnatal lethality and severe hematopoietic hypoplasia. (jci.org)
  • Candidates for HSCTs include pediatric cases where the patient has an inborn defect such as severe combined immunodeficiency or congenital neutropenia with defective stem cells, and also children or adults with aplastic anemia who have lost their stem cells after birth. (wikipedia.org)
  • Since more than one type of immune cell is affected, this disease is classified as a severe combined immunodeficiency disease (SCID). (wikipedia.org)
  • protein
  • If there is a mutation in the protein, that means that the mitochondria metabolism most likely will be altered and will not be able to provide enough energy to the hematopoietic stem cells. (wikipedia.org)
  • Ubiquitin is a small (76-amino acid) protein highly conserved among eukaryotic cells. (wikipedia.org)
  • Jeggo is particularly well known for identifying two components of an enzyme called DNA-dependent protein kinase (DNA-PK) as being important in DNA non-homologous end joining (NHEJ), a pathway by which mammalian cells repair themselves. (wikipedia.org)
  • Reactive
  • Aplastic anemia (AA) is an immune-mediated and life-threatening form of acquired bone marrow failure (BMF), characterized by development and expansion of self-reactive T cells. (springer.com)
  • Standard treatment options for patients with AA include: (1) immunosuppressive therapy (IST) with anti-thymocyte globulin and cyclosporine A which targets self-reactive T cells, or (2) matched sibling or unrelated BM transplant (BMT). (springer.com)
  • clinical
  • Shizuru JA, Negrin RS, Weissman IL (2005) Hematopoietic stem and progenitor cells: clinical and preclinical regeneration of the hematolymphoid system. (springer.com)
  • Funding for this research came, in part, from the National Institutes of Health (R21-NS090066, RO1-DK090058) the Cystinosis Research Foundation the Sanford Stem Cell Clinical Center and the California Institute of Regenerative Medicine. (newswise.com)
  • In a small human clinical trial to evaluate the safety and efficacy of fucoidan ingestion (brown seaweed extract), 3g daily of 75% w/w oral fucoidan for 12 days increased the proportion of CD34+CXCR4+ from 45 to 90% and the serum SDF-1 levels, which could be useful in CD34+ cells homing/mobilization via SDF-1/CXCR4 axis. (wikipedia.org)
  • Zinc finger nucleases have also been used in a mouse model of haemophilia and a clinical trial found CD4+ human T-cells with the CCR5 gene disrupted by zinc finger nucleases to be safe as a potential treatment for HIV/AIDS. (wikipedia.org)
  • US FDA 2008) for clinical use (to mobilize hematopoietic stem cells). (wikipedia.org)
  • June 2016 BL-8040, a Peptidic CXCR4 Antagonist, Induces Leukemia Cell Death and Specific Leukemia Cell Mobilization in Relapsed/Refractory Acute Myeloid Leukemia Patients in an Ongoing Phase IIa Clinical Trial. (wikipedia.org)
  • epithelial
  • G. Nigro, R. Rossi, P. H. Commere, P. Jay, P. J. Sansonetti, The cytosolic bacterial peptidoglycan sensor Nod2 affords stem cell protection and links microbes to gut epithelial regeneration. (sciencemag.org)
  • Adult
  • In a healthy adult person, approximately 1011-1012 new blood cells are produced daily in order to maintain steady state levels in the peripheral circulation. (wikipedia.org)
  • Blood
  • The discovery has implications for developing stem cell-based therapies for diseases like leukemia and congenital blood disorders. (redorbit.com)
  • The gene turns on key binding molecules, or ligands, that are required to definitively establish blood cell production. (redorbit.com)
  • Image Caption: A scanning electron microscope image depicting normal circulating human blood, which includes round, dimpled red blood cells and several types of white blood cells, including lymphocytes, a monocyte and a neutrophil, and many small, disc-shaped platelets. (redorbit.com)
  • They are an essential support mechanism of blood cells and the immune system. (redorbit.com)
  • Also, elevated expression of Cdc42 has been found in immune system white blood cells in older humans. (redorbit.com)
  • In particular, they are testing red blood cell production, endurance and immune response in the mice. (redorbit.com)
  • It must balance enormous production needs (more than 500 billion blood cells are produced every day) with the need to precisely regulate the number of each blood cell type in the circulation. (wikipedia.org)
  • The patient is then treated with high-dose chemotherapy with or without radiotherapy with the intention of eradicating the patient's malignant cell population at the cost of partial or complete bone marrow ablation (destruction of patient's bone marrow's ability to grow new blood cells). (wikipedia.org)
  • As these cells are short-lived, there needs to be a steady turnover of new blood cells and the maintenance of an HSC pool. (wikipedia.org)
  • However, Ueno and Weissman provided the earliest contradiction to the hemangioblast theory when they saw that distinct ES cells mixed into a blastocyst resulted in more than 1 ES cell contributing to the majority of the blood islands found in the resultant embryo. (wikipedia.org)
  • This model has received support from studies showing subpopulations of blood cells harboring initiating but not late somatic mutations in patients with chronic lymphocytic leukemia (CLL), hairy cell leukemia (HCL), and AML. (wikipedia.org)
  • Monocytes are a type of leukocyte, or white blood cell. (wikipedia.org)
  • There are at least three types of monocytes in human blood: The classical monocyte is characterized by high level expression of the CD14 cell surface receptor (CD14++ CD16− monocyte) The non-classical monocyte shows low level expression of CD14 and additional co-expression of the CD16 receptor (CD14+CD16++ monocyte). (wikipedia.org)
  • Hematopoietic stem cells give rise to blood cells. (wikipedia.org)
  • GM-CSF stimulates production of white blood cells. (wikipedia.org)
  • Stem cells are taken from an affected child's blood or bone marrow. (wikipedia.org)
  • G-CSF is a growth factor for neutrophils (a common type of white blood cells), and may act by increasing the activity of neutrophil-derived proteases such as neutrophil elastase in the bone marrow leading to proteolytic degradation of SDF-1. (wikipedia.org)
  • Following the transplant procedure, the patient's CD4+ T-cells circulating in the blood were homozygous for CCR5-Δ32. (wikipedia.org)
  • Noting the reduction in detectable HIV in the patient's blood and the return of the patient's circulating CD4+ T-cell population to normal levels, with all of the cells expressing the resistant variant of CCR5, Levy cautions that calling this treatment a cure would be premature. (wikipedia.org)
  • All cellular blood components are derived from haematopoietic stem cells. (wikipedia.org)
  • Erythroid cells are the oxygen carrying red blood cells. (wikipedia.org)
  • The lymphoid lineage is primarily composed of T-cells and B-cells (types of white blood cells). (wikipedia.org)
  • In developing embryos, blood formation occurs in aggregates of blood cells in the yolk sac, called blood islands. (wikipedia.org)
  • When bone marrow develops, it eventually assumes the task of forming most of the blood cells for the entire organism. (wikipedia.org)
  • Infection
  • A. Burberry, M. Y. Zeng, L. Ding, I. Wicks, N. Inohara, S. J. Morrison, G. Núñez, Infection mobilizes hematopoietic stem cells through cooperative NOD-like receptor and Toll-like receptor signaling. (sciencemag.org)
  • Because "most of HIV strains" use the CCR5 receptor to enter a host cell, the mutation confers resistance to HIV infection. (wikipedia.org)
  • signaling pathway
  • Philip A. Beachy has examined the Hedgehog signaling pathway and its role in embryonic development and cell formation. (wikipedia.org)
  • Matthew P. Scott has identified the human homolog PTCH1 as a key tumor suppressor gene for the Hedgehog signaling pathway and the causative gene for Nevoid basal-cell carcinoma syndrome. (wikipedia.org)
  • function
  • That NOD signaling influences stem cell function was also reported by Nigro et al . (sciencemag.org)
  • Pharmacologic inhibition of Cdc42 reversed HSC aging and restored function similar to that of younger stem cells, explained Hartmut Geiger, PhD, the study's principal investigator and a researcher in the Division of Experimental Hematology/Cancer Biology at Cincinnati Children's, and the Department of Dermatology and Allergic Diseases, Ulm University Medicine. (redorbit.com)
  • Apart from the proper design of the nucleases to recognize and cut the selected site in the cell genome, two main goals need to be adequately addressed to optimize its function: the delivery of the tools into the desired cells and the selection of those where the gene editing process has occurred correctly. (intechopen.com)
  • Meng A, Wang Y, Brown SA, Van Zant G, Zhou D. Ionizing radiation and busulfan inhibit murine bone marrow cell hematopoietic function via apoptosis-dependent and -independent mechanisms. (springer.com)
  • The genetic background, derived from inbred NOD mouse strain NOD/ShiLtJ, contributes reductions in innate immunity that include an absent hemolytic complement system, reduced dendritic cell function, and defective macrophage activity. (wikipedia.org)
  • distinct
  • This view was first challenged by the 2002 discovery by the Muller-Sieburg group in San Diego, who illustrated that different stem cells can show distinct repopulation patterns that are epigenetically predetermined intrinsic properties of clonal Thy-1lo Sca-1+ lin− c-kit+ HSC. (wikipedia.org)
  • tissue
  • These change into macrophages after entering into appropriate tissue spaces, and can transform into foam cells in endothelium. (wikipedia.org)
  • The bedrock of what it means to be a living entity is an understanding of how a cell or tissue functions as an integrated system. (wikipedia.org)
  • inhibit
  • Another explanation of this observation is provided by a study that shows the ability of CXCL12 (and CCL2) producing tumors to entrain neutrophils that inhibit seeding of tumor cells in the lung. (wikipedia.org)
  • diseases
  • This chapter will review and discuss how researchers are trying to solve them, all attempts that are ongoing and the potential application of the technology to the patients affected with hematopoietic genetic diseases. (intechopen.com)
  • Correction
  • The availability of these tools has allowed a precise gene editing, such as knockout of a specific gene or the correction of a defective gene by means of homologous recombination (HR), taking advantage of the endogenous cell repair machinery. (intechopen.com)
  • human
  • It is a very efficient inducer of hematopoietic stem cell mobilization in animal and human studies. (wikipedia.org)
  • The compound immunodeficiencies in NSG branded mice permit the engraftment of a wide range of primary human cells, and enable sophisticated modeling of many areas of human biology and disease. (wikipedia.org)
  • CXCR4
  • HIV isolates that use CXCR4 are traditionally known as T-cell tropic isolates. (wikipedia.org)
  • WHIM like mutations in CXCR4 were recently identified in patients with Waldenstrom's macroglobulinemia, a B-cell malignancy. (wikipedia.org)
  • However, in breast cancer where SDF1/CXCL12 is also expressed by the cancer cells themselves along with CXCR4, CXCL12 expression is positively correlated with disease free (metastasis free) survival. (wikipedia.org)
  • CXCR4 antagonists are especially in important for hindering cancer progression because one of the downstream effects initiated by CXCR4 receptor activation is cell movement which helps the spread of cancer, known as metastasis. (wikipedia.org)
  • genetic
  • it is thought that this subpopulation is "clonally" derived from a single founding cell and is therefore made of genetic "clones" of the founder. (wikipedia.org)
  • As the HUMARA assay is based on the epigenetic state of cells, the underlying genetic determinants of the clonal expansion remained to be uncovered. (wikipedia.org)
  • Patients with RD have a genetic defect that affects the T cells and at least one other type of immune cell. (wikipedia.org)