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Role of the Leucine Zipper Domain of CCAAT/ Enhancer Binding Protein-Epsilon (C/EBPepsilon) in Neutrophil-Specific Granule Deficiency.
Wada T, Akagi T. Wada T, et al. Crit Rev Immunol. 2016;36(4):349-358. doi: 10.1615/CritRevImmunol.2017019385. Crit Rev Immunol. 2016. PMID: 28322138 Review.
Neutrophil-specific granule deficiency (SGD) is a rare autosomal recessive primary immunodeficiency characterized by bilobed neutrophil nuclei and lack of neutrophil-specific granule proteins such as lactoferrin. ...A recent observation showed that a homozygo …
Neutrophil-specific granule deficiency (SGD) is a rare autosomal recessive primary immunodeficiency characterized by bi …
Neutrophil granule constituents and their release in health and disease.
Boxer LA, Smolen JE. Boxer LA, et al. Hematol Oncol Clin North Am. 1988 Mar;2(1):101-34. Hematol Oncol Clin North Am. 1988. PMID: 2831184 Review.
In the latter case, specific granule constituents are released directly into the extracellular environment, demonstrating that these granules can function as secretory organelles. Studies of patients with specific granule deficiency have indicated the role of …
In the latter case, specific granule constituents are released directly into the extracellular environment, demonstrating that these granule …
The role of C/EBP(epsilon) in the terminal stages of granulocyte differentiation.
Lekstrom-Himes JA. Lekstrom-Himes JA. Stem Cells. 2001;19(2):125-33. doi: 10.1634/stemcells.19-2-125. Stem Cells. 2001. PMID: 11239167 Review.
As a consequence of its characterization using both in vitro and knockout mouse models, the myeloid-specific transcription factor, CCAAT/enhancer binding protein (C/EBP)epsilon, has been identified as a critical regulator of terminal granulopoiesis and one of the causative mutati …
As a consequence of its characterization using both in vitro and knockout mouse models, the myeloid-specific transcription factor, CCAAT/enh …
White blood cell defects: molecular discoveries and clinical management.
Uzel G, Holland SM. Uzel G, et al. Curr Allergy Asthma Rep. 2002 Sep;2(5):385-91. doi: 10.1007/s11882-002-0071-5. Curr Allergy Asthma Rep. 2002. PMID: 12165204 Review.
We have also included NF-kappaB essential modifier (NEMO) defects, which lead to X-linked ectodermal dysplasia, with or without lymphedema and osteopetrosis, and a wide range of involvement of the immune system, which can mimic the hyper-IgM phenotype. Neutrophil-specific
We have also included NF-kappaB essential modifier (NEMO) defects, which lead to X-linked ectodermal dysplasia, with or without lymphedema a …
The molecular biology of selected phagocyte defects.
Lomax KJ, Malech HL, Gallin JI. Lomax KJ, et al. Blood Rev. 1989 Jun;3(2):94-104. doi: 10.1016/0268-960x(89)90004-0. Blood Rev. 1989. PMID: 2673450 Review.
The absence of lactoferrin associated with hereditary specific granule deficiency is a result of decreased transcription of the lactoferrin gene in myeloid cells. ...
The absence of lactoferrin associated with hereditary specific granule deficiency is a result of decreased transcriptio …