Neutrophil-specific Granule Deficiency Results from a Novel Mutation with Loss of Function of the Transcription Factor CCAAT/Enhancer Binding Protein ε

Simplified mammalian DNA isolation procedure

1991 • 1,508 citations

A liver-enriched transcriptional activator protein, LAP, and a transcriptional inhibitory protein, LIP, are translated from the sam mRNA

1991 • 1,019 citations

Absence of granulocyte colony-stimulating factor signaling and neutrophil development in CCAAT enhancer binding protein α-deficient mice

1997 • 929 citations

Biological Role of the CCAAT/Enhancer-binding Protein Family of Transcription Factors

1998 • 799 citations

A family of C/EBP-related proteins capable of forming covalently linked leucine zipper dimers in vitro.

1991 • 604 citations

CCAAT/Enhancer Binding Protein α Is a Regulatory Switch Sufficient for Induction of Granulocytic Development from Bipotential Myeloid Progenitors

1998 • 483 citations

Mutation in the signal-transducing chain of the interferon-gamma receptor and susceptibility to mycobacterial infection.

1998 • 427 citations

Impaired granulopoiesis, myelodysplasia, and early lethality in CCAAT/enhancer binding protein ɛ-deficient mice

1997 • 379 citations

CCAAT/enhancer-binding protein mRNA is translated into multiple proteins with different transcription activation potentials.

1993 • 372 citations

PU.1 (Spi-1) and C/EBP alpha regulate the granulocyte colony- stimulating factor receptor promoter in myeloid cells

1996 • 341 citations

Biosynthesis of granule proteins in normal human bone marrow cells. Gelatinase is a marker of terminal neutrophil differentiation

1995 • 232 citations

Human neutrophil-specific granule deficiency: a model to assess the role of neutrophil-specific granules in the evolution of the inflammatory response

1982 • 198 citations

Microbicidal/cytotoxic proteins of neutrophils are deficient in two disorders: Chediak-Higashi syndrome and "specific" granule deficiency.

1988 • 195 citations

CCAAT/enhancer binding protein ɛ is preferentially up-regulated during granulocytic differentiation and its functional versatility is determined by alternative use of promoters and differential splicing

1997 • 185 citations

Lactoferrin Deficiency Associated with Altered Granulocyte Function

1982 • 172 citations

Characterization of 18 New Mutations in COL7A1 in Recessive Dystrophic Epidermolysis Bullosa Provides Evidence for Distinct Molecular Mechanisms Underlying Defective Anchoring Fibril Formation

1997 • 159 citations

Cloning of the Novel Human Myeloid-Cell-Specific C/EBP-ε Transcription Factor

1997 • 141 citations

Lactoferrin deficiency as a consequence of a lack of specific granules in neutrophils from a patient with recurrent infections. Detection by immunoperoxidase staining for lactoferrin and cytochemical electron microscopy.

1980 • 134 citations

An Anomaly of Neutrophil Morphology with Impaired Function

1974 • 124 citations

A Novel Human CCAAT/Enhancer Binding Protein Gene, C/EBPϵ, Is Expressed in Cells of Lymphoid and Myeloid Lineages and Is Localized on Chromosome 14q11.2 Close to the T-Cell Receptor α/δ Locus

1996 • 109 citations

CCAAT/Enhancer Binding Protein ɛ Is Critical for Effective Neutrophil-Mediated Response to Inflammatory Challenge

1999 • 105 citations

Ca2+-Dependent Production and Release of IL-8 in Human Neutrophils

1998 • 97 citations

Granulocyte colony-stimulating factor regulates myeloid differentiation through CCAAT/enhancer-binding protein epsilon

2001 • 76 citations

Abnormal neutrophil maturation in a neutrophil defect with morphologic abnormality and impaired function

1979 • 71 citations

C/EBPε Is a Myeloid-specific Activator of Cytokine, Chemokine, and Macrophage-Colony-stimulating Factor Receptor Genes

1998 • 69 citations

Premature Termination Codon Mutations in the Type VII Collagen Gene in Recessive Dystrophic Epidermolysis Bullosa Result in Nonsense-Mediated mRNA Decay and Absence of Functional Protein

1997 • 59 citations

Congenital neutropenia: neutrophil proliferation with abnormal maturation

1975 • 59 citations

Selective defect in myeloid cell lactoferrin gene expression in neutrophil specific granule deficiency.

1989 • 58 citations

Neutrophil-specific granule deficiency includes eosinophils

1993 • 58 citations

Defective bactericidal activity and absence of specific granules in neutrophils from a patient with recurrent bacterial infections

1984 • 49 citations

Absence of the Largest Platelet-von Willebrand Multimers in a Patient with Lactoferrin Deficiency and a Bleeding Tendency

1992 • 22 citations

Glandular secretion of lactoferrin in a patient with neutrophil lactoferrin deficiency

1989 • 19 citations

Anomalous neutrophil granule distribution in a patient with lactoferrin deficiency: Pertinence to the respiratory burst

1985 • 16 citations

C/EBP-ε: Chromosomal mapping and mutational analysis of the gene in leukemia and preleukemia

1997 • 16 citations

A marked decrease in defensin mRNA in the only case of congenital neutrophil-specific granule deficiency reported in Japan.

1994 • 13 citations

Ultrastructure of neutrophilic phagosome of autologous platelet in vivo in specific granule deficiency

1993 • 3 citations