Allelic Heterogeneity in Hereditary Surfactant Protein B (SP-B) Deficiency

RNA splice junctions of different classes of eukaryotes: sequence statistics and functional implications in gene expression

1987 • 2,333 citations

Recommendations for a nomenclature system for human gene mutations

1998 • 936 citations

Targeted disruption of the surfactant protein B gene disrupts surfactant homeostasis, causing respiratory failure in newborn mice.

1995 • 639 citations

Deficiency of Pulmonary Surfactant Protein B in Congenital Alveolar Proteinosis

1993 • 630 citations

A mutation in the surfactant protein B gene responsible for fatal neonatal respiratory disease in multiple kindreds.

1994 • 561 citations

Hyaline membrane disease

1956 • 413 citations

Pulmonary Surfactant Protein B (SP-B): Structure-Function Relationships

1991 • 307 citations

Structure and properties of surfactant protein B

1998 • 238 citations

Human surfactant protein B: structure, function, regulation, and genetic disease

1995 • 192 citations

Aberrant processing of surfactant protein C in hereditary SP-B deficiency

1995 • 186 citations

Lung transplantation for treatment of infants with surfactant protein B deficiency

1997 • 180 citations

Synthesis, processing and secretion of surfactant proteins B and C

1998 • 175 citations

Ontogeny of Surfactant Proteins A and B in Human Amniotic Fluid as Indices of Fetal Lung Maturity

1991 • 155 citations

Partial Deficiency of Surfactant Protein B in an Infant With Chronic Lung Disease

1995 • 152 citations

Surfactant protein SP-B induces ordering at the surface of model membrane bilayers

1990 • 151 citations

Decreased lung compliance and air trapping in heterozygous SP-B-deficient mice.

1997 • 147 citations

Structure and Organization of the Gene Encoding Human Pulmonary Surfactant Proteolipid SP-B

1989 • 139 citations

Molecular and phenotypic variability in the congenital alveolar proteinosis syndrome associated with inherited surfactant protein B deficiency

1994 • 134 citations

Surfactant protein B: disulfide bridges, structural properties and kringle similarities

1991 • 125 citations

Ultrastructure of lung in surfactant protein B deficiency.

1994 • 117 citations

Intracellular processing of pulmonary surfactant protein B in an endosomal/lysosomal compartment

1992 • 108 citations

Cystic Fibrosis as a Disease of Misprocessing of the Cystic Fibrosis Transmembrane Conductance Regulator Glycoprotein

1999 • 107 citations

Isolation of a cDNA clone encoding a high molecular weight precursor to a 6-kDa pulmonary surfactant-associated protein.

1987 • 105 citations

Surfactant Protein-B–Deficient Mice Are Susceptible to Hyperoxic Lung Injury

1999 • 105 citations

Intracellular pH modulates the activity of chloride channels in isolated lacrimal gland acinar cells

1995 • 97 citations

An SP-B Gene Mutation Responsible for SP-B Deficiency in Fatal Congenital Alveolar Proteinosis: Evidence for a Mutation Hotspot in Exon 4

1998 • 84 citations

Biosynthetic routing of pulmonary surfactant proteins in alveolar type II cells

1993 • 84 citations

Transient surfactant protein B deficiency in a term infant with severe respiratory failure

1998 • 80 citations

Structural Requirements for Targeting of Surfactant Protein B (SP-B) to Secretory Granules in Vitro and in Vivo

1996 • 72 citations

Rescue of SP-B Knockout Mice with a Truncated SP-B Proprotein

1997 • 70 citations

Surfactant Protein-B Deficiency

1997 • 54 citations

Surfactant protein B deficiency: Clinical, histological and molecular evaluation

1999 • 33 citations

Human Surfactant Protein-A Contains Blood Group A Antigenic Determinants

1992 • 28 citations

Cell-specific posttranslational processing of the surfactant-associated protein SP-B

1993 • 26 citations

Impaired Cotranslational Processing as a Mechanism for Type I Antithrombin Deficiency

1998 • 22 citations