INT186461

Context Info Confidence 0.73 First Reported 2005 Last Reported 2008 Negated 0 Speculated 0 Reported most in Body Documents 10 Total Number 16 Disease Relevance 8.69 Pain Relevance 1.09

extracellular space (SFTPB) extracellular region (SFTPB) lipid metabolic process (SFTPB) lysosome (SFTPB) cytoplasm (SFTPB)

Anatomy Link Frequency Clara cells 2 external 1 epithelial cells 1 neutrophil 1 SFTPB (Homo sapiens)

Pain Link Frequency Relevance Heat fibrosis 239 97.52 Inflammation 236 93.24 corticosteroid 6 92.92 depression 2 80.56 Angina 3 16.56 tolerance 1 10.00 anesthesia 13 5.00 Pain 2 5.00 imagery 2 5.00

Disease Link Frequency Relevance Heat Disease 112 99.56 Pulmonary Disease 124 99.00 Pneumonia 121 98.88 Bronchitis 128 98.32 Pulmonary Alveolar Proteinosis 229 97.84 Cystic Fibrosis 230 97.52 Adult Respiratory Distress Syndrome 139 96.84 Congenital Anomalies 39 95.12 INFLAMMATION 222 93.24 Respiratory Distress 96 91.20

Sentences Mentioned In

Key:

Protein

Mutation

Event

Anatomy

Negation

Speculation

Pain term

Disease term

Although SP-B was abundantly present and mutations of SFTPB were excluded [18], alterations of SP-B processing from other causes have not been excluded.

Gene_expression (present) of SP-B

1) Confidence 0.73 Published 2005 Journal Respir Res Section Body Doc Link PMC1224872 Disease Relevance 0.50 Pain Relevance 0.05

Thus, this intermediate represents a normal pro-SP-B processing intermediate of SP-B biosynthesis and could result from either constitutive secretion of this form by type II cells or from the physiologic release of 25 kD pro-SP-B into the airways by Clara cells.

Gene_expression (biosynthesis) of SP-B in Clara cells

2) Confidence 0.73 Published 2005 Journal Respir Res Section Body Doc Link PMC1224872 Disease Relevance 0.20 Pain Relevance 0

SP-B is synthesized as a proprotein by alveolar type II epithelial cells and non-ciliated bronchiolar (Clara) cells; however, complete processing of the precursor to the biologically active, mature peptide occurs only in type II cells.

Gene_expression (synthesized) of SP-B in epithelial cells

3) Confidence 0.73 Published 2005 Journal Respir Res Section Body Doc Link PMC1224872 Disease Relevance 0.33 Pain Relevance 0

In addition, mutations of surfactant protein B gene and other known external or syndromic causes of PAP, including LPI, were ruled out in this cohort of paediatric patients.

Gene_expression (mutations) of surfactant protein B in external associated with pulmonary alveolar proteinosis

4) Confidence 0.65 Published 2007 Journal Orphanet J Rare Dis Section Body Doc Link PMC1845139 Disease Relevance 0.70 Pain Relevance 0

Of the 6 patients with SP-B deficiency defined as a lack of mature SP-B on Western blotting, 4 had mutations in SFTPB (Tab. 3).

Gene_expression (deficiency) of SP-B

5) Confidence 0.63 Published 2005 Journal Respir Res Section Body Doc Link PMC1224872 Disease Relevance 0.58 Pain Relevance 0

Mature SP-B was regularly detected in all lavages from normal children and from those with chronic bronchitis at a median molecular weight of 7 kDa (Tab. 1, Fig. 2).

Gene_expression (detected) of SP-B associated with bronchitis

6) Confidence 0.63 Published 2005 Journal Respir Res Section Body Doc Link PMC1224872 Disease Relevance 0.64 Pain Relevance 0

SP-B is synthesized by alveolar type II cells to the mature 8 kDa protein and secreted into the alveolar space, where it is mainly found as a dimer [5].

Gene_expression (synthesized) of SP-B

7) Confidence 0.60 Published 2008 Journal BMC Pulm Med Section Body Doc Link PMC2364613 Disease Relevance 0.32 Pain Relevance 0.06

Pro-SP-B was found in 60 of 64 samples and was therefore commonly abundant in all groups, which corresponds to previous results [10].

Gene_expression (found) of SP-B

8) Confidence 0.58 Published 2008 Journal BMC Pulm Med Section Body Doc Link PMC2364613 Disease Relevance 0.71 Pain Relevance 0.18

Pro-SP-B was found in 60 of 64 samples and was therefore commonly abundant in all groups, which corresponds to previous results [10].

Gene_expression (abundant) of SP-B

9) Confidence 0.58 Published 2008 Journal BMC Pulm Med Section Body Doc Link PMC2364613 Disease Relevance 0.79 Pain Relevance 0.21

SP-B was detected in BAL of all 64 patients, whereas SP-C was found in BAL of all but 3 children; those three BAL fluids had more than 80% neutrophils, and in two patients, who were re-lavaged later, SP-C was then present and the neutrophil count was lower.

Gene_expression (detected) of SP-B in neutrophil

10) Confidence 0.52 Published 2008 Journal BMC Pulm Med Section Abstract Doc Link PMC2364613 Disease Relevance 0.84 Pain Relevance 0.20

SP-B was mainly present as a dimer, SP-C as a monomer.

Gene_expression (present) of SP-B

11) Confidence 0.52 Published 2008 Journal BMC Pulm Med Section Abstract Doc Link PMC2364613 Disease Relevance 0.78 Pain Relevance 0.19

SP-B was detected regularly at molecular weights of 8 kDa in monomeric respectively 16–18 kDa in dimeric form, additional partly at approximately 30 kDa under non reducing conditions, suggesting the presence of oligomers in smaller amounts.

Gene_expression (detected) of SP-B

12) Confidence 0.52 Published 2008 Journal BMC Pulm Med Section Body Doc Link PMC2364613 Disease Relevance 0.37 Pain Relevance 0.09

Bronchoalveolar Clara cells also express pro-SP-B, but do not process it to the mature form.

Gene_expression (express) of SP-B in Clara cells

13) Confidence 0.52 Published 2008 Journal BMC Pulm Med Section Body Doc Link PMC2364613 Disease Relevance 0.39 Pain Relevance 0.07

In line with our data, they found decreased SP-A and SP-B levels compared to controls, but SP-A remained consistently low during the observation period (14 days).

Gene_expression (levels) of SP-B

14) Confidence 0.51 Published 2007 Journal Respir Res Section Body Doc Link PMC1950506 Disease Relevance 0.94 Pain Relevance 0

An important finding of this study is the regular detection of certain pro-SP-B peptides in BAL from children without bronchoalveolar disease.

Gene_expression (detection) of SP-B associated with disease

15) Confidence 0.49 Published 2005 Journal Respir Res Section Body Doc Link PMC1224872 Disease Relevance 0.44 Pain Relevance 0

It has also to be noted that our currently applied technique for measurement of SP-B and SP-C does not allow for the differentiation between intact mature SP-B/C and degradation products of these proteins, the generation of which may additionally exert a detrimental effect on surface activity of the LA fraction.

Gene_expression (products) of SP-B

16) Confidence 0.40 Published 2007 Journal Respir Res Section Body Doc Link PMC1950506 Disease Relevance 0.15 Pain Relevance 0.04

General Comments

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