Normal Lung Function in Subjects Heterozygous for Surfactant Protein-B Deficiency

1999 ◽  
Vol 159 (2) ◽  
pp. 411-414 ◽  
Author(s):  
ROGER D. YUSEN ◽  
ALAN H. COHEN ◽  
AARON HAMVAS
Keyword(s):  
Lung Function ◽  
Surfactant Protein ◽  
Normal Lung ◽  
Surfactant Protein B ◽  
Normal Lung Function ◽  
Surfactant Protein B Deficiency ◽  
Protein B

Surfactant protein B processing in human fetal lung

1998 ◽  
Vol 275 (3) ◽  
pp. L559-L566 ◽  
Author(s):  
Susan H. Guttentag ◽  
Michael F. Beers ◽  
Bert M. Bieler ◽  
Philip L. Ballard
Keyword(s):  
Polyclonal Antibodies ◽  
Fetal Lung ◽  
Surfactant Protein ◽  
Explant Culture ◽  
Normal Lung ◽  
Surfactant Protein B ◽  
Multistep Process ◽  
Human Fetal Lung ◽  
Protein B

Surfactant protein B (SP-B8), an 8-kDa hydrophobic protein essential for surfactant and normal lung function, is produced from the intracellular processing of preproSP-B. To characterize SP-B processing in human type 2 cells, we used human fetal lung in explant culture and polyclonal antibodies to human SP-B8(Phe201–Met279) and to specific epitopes within the NH2- and COOH-terminal propeptide domains (Ser145–Leu160, Gln186–Gln200, and Gly284–Ser304). Western blot analysis revealed a novel intermediate at ∼9 kDa, representing mature SP-B8, with a residual NH2-terminal peptide of ∼10 amino acids. Pulse-chase studies showed a precursor-product relationship between the 9- and 8-kDa forms. During differentiation of type 2 cells in explant culture, the rate of proSP-B conversion to 25-kDa intermediate remained constant, whereas the rate of 25-kDa intermediate conversion to SP-B8increased, resulting in a net increase in tissue SP-B8. Dexamethasone did not affect the rate of proSP-B processing but markedly enhanced the rate of SP-B8 accumulation. We conclude that NH2-terminal propeptide cleavage of proSP-B is a multistep process and that more distal processing events are rate limiting and both developmentally and hormonally regulated.

scholarly journals Surfactant Phosphatidylcholine Metabolism in Patients with Congenital Surfactant Protein-B Deficiency Studied with Stable Isotopes

1999 ◽  
Vol 45 (4, Part 2 of 2) ◽  
pp. 306A-306A
Author(s):  
Daphne J M T Janssen ◽  
Virgilio P Carnielli ◽  
Jan Erik H Bunt ◽  
Ingrid H T Luijendijk ◽  
Dick Tibboel ◽  
...  
Keyword(s):  
Stable Isotopes ◽  
Surfactant Protein ◽  
Surfactant Protein B ◽  
Surfactant Protein B Deficiency ◽  
Phosphatidylcholine Metabolism ◽  
Protein B

Hereditary surfactant protein B deficiency resulting from a novel mutation

2000 ◽  
Vol 26 (1) ◽  
pp. 97-100 ◽  
Author(s):  
M. Somaschini ◽  
S. Wert ◽  
G. Mangili ◽  
A. Colombo ◽  
L. Nogee
Keyword(s):  
Novel Mutation ◽  
Surfactant Protein ◽  
Surfactant Protein B ◽  
Surfactant Protein B Deficiency ◽  
Protein B

Congenital surfactant protein B deficiency - emphasis on imaging

2001 ◽  
Vol 31 (5) ◽  
pp. 327-331 ◽  
Author(s):  
B. Newman ◽  
Jerald P. Kuhn ◽  
Sandra S. Kramer ◽  
Joseph A. Carcillo
Keyword(s):  
Surfactant Protein ◽  
Surfactant Protein B ◽  
Surfactant Protein B Deficiency ◽  
Protein B

SURFACTANT PROTEIN B DEFICIENCY

2002 ◽  
Vol 21 (5) ◽  
pp. 507-511
Author(s):  
Barbara K. Fulton ◽  
Mary M. Davis
Keyword(s):  
Surfactant Protein ◽  
Surfactant Protein B ◽  
Surfactant Protein B Deficiency ◽  
Protein B

Surfactant protein B deficiency: Antenatal diagnosis and prospective treatment with surfactant replacement

1994 ◽  
Vol 125 (3) ◽  
pp. 356-361 ◽  
Author(s):  
Aaron Hamvas ◽  
F. Sessions Cole ◽  
Daphne E. deMello ◽  
Michael Moxley ◽  
Jeffrey A. Whitsett ◽  
...  
Keyword(s):  
Antenatal Diagnosis ◽  
Surfactant Protein ◽  
Surfactant Replacement ◽  
Surfactant Protein B ◽  
Surfactant Protein B Deficiency ◽  
Protein B

Increased serum SP-D in identification of high-risk smokers at high risk of COPD

2021 ◽  
Author(s):  
Christine Dalgård ◽  
Fang Wang ◽  
Ingrid Louise Titlestad ◽  
Kirsten Ohm Kyvik ◽  
Jørgen Vestbo ◽  
...  
Keyword(s):  
High Risk ◽  
Lung Function ◽  
Surfactant Protein ◽  
Innate Immune ◽  
Normal Lung ◽  
Surfactant Protein D ◽  
Lung Function Decline ◽  
Normal Lung Function ◽  
Danish Twin

Pulmonary surfactant protein D (SP-D) is an important component of the pulmonary innate immune system with the ability to dampen cigarette smoke-induced lung inflammation. However, cigarette smoking mediates translocation of SP-D from the lung to the blood, and serum SP-D (sSP-D) has therefore previously been suggested as marker for smoke-induced lung injury. In support of this notion, associations between high sSP-D and low lung function measurements have previously been demonstrated in smokers and in COPD. The present investigations employ a 12-year longitudinal Danish twin study to test the hypothesis that baseline sSP-D variation has the capacity to identify smokers with normal baseline lung function who are in high risk of significant future smoke-induced lung function decline. We find that sSP-D is significantly increased in those with normal lung function at baseline that develop lung function decline during follow up compared to those who stay lung healthy. Moreover, we demonstrate that it is the smoke-induced baseline sSP-D level, and not the constitutional level, which has capacity as biomarker, and which is linearly increased with the decline in lung function during follow up. In conclusion, we here present first observation of increased sSP-D for identification of high-risk smokers.

Long-term outcomes after infant lung transplantation for surfactant protein B deficiency related to other causes of respiratory failure

2006 ◽  
Vol 149 (4) ◽  
pp. 548-553 ◽  
Author(s):  
Lisanne M. Palomar ◽  
Lawrence M. Nogee ◽  
Stuart C. Sweet ◽  
Charles B. Huddleston ◽  
F. Sessions Cole ◽  
...  
Keyword(s):  
Respiratory Failure ◽  
Lung Transplantation ◽  
Surfactant Protein ◽  
Long Term Outcomes ◽  
Surfactant Protein B ◽  
Surfactant Protein B Deficiency ◽  
Protein B
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