Type III Collagen Aminopropeptide Levels in Serum of Patients With Progressive Systemic Scleroderma

Krieg, Thomas; Langer, I.; Gerstmeier, H.; Keller, J.; Mensing, H.; Goerz, G; Timpl, Rupert

doi:10.1111/1523-1747.ep12459865

Cited by 55 publications

(16 citation statements)

References 20 publications

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“…Furthermore, a similar increase in ICTP has been found in two patients with a generalized form of morphoea, supporting the correlation between the extent of connective tissue involvement and ICTP levels in SSc. The finding of an increased turnover of type I collagen in SSc may be further supported by the increased levels of the amino terminal propeptide of type III procollagen in this disease reported by several authors, 6,23,24 as recent studies indicate that some of the assays used previously also measure degradation products of type III collagen. 21 Moreover, a recent study reporting increased urinary excretion of two mature cross-links of collagen, hydroxylysyl and lysyl pyridinoline, in patients with SSc, suggests that in SSc more fibrillar collagens are degraded than in the normal state.…”

Section: Discussionmentioning

confidence: 52%

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Circulating type I collagen degradation products: a new serum marker for clinical severity in patients with scleroderma?

Hunzelmann

Risteli

Sacher

et al. 1998

British Journal of Dermatology

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Systemic sclerosis (SSc; scleroderma) results in the excessive deposition of extracellular matrix components in affected organs. This is partly due to enhanced synthesis; however, the role of degradative processes in this disease is still poorly understood. Sera of 32 patients with SSc (22 with the diffuse, 10 with the limited form) and of six patients with morphoea were assessed using radioimmunoassays for the cross-linked carboxy terminal telopeptide of type I collagen (ICTP) and for the amino terminal propeptide of type I procollagen (PINP) reflecting type I collagen degradation and synthesis, respectively. In 27 of the 32 patients with SSc, the concentration of ICTP was above the upper limit of the normal value (4.6 micrograms/L) and the mean level was clearly elevated at 7.92 micrograms/L. The ICTP concentration correlated with the skin score measuring the extent of the lesions, whereas no such correlation was found for PINP. The ICTP antigen in serum, studied by immunoblotting, had a molecular weight of about twice that of the trypsin-generated fragment isolated from human bone collagen. The mean concentration of serum PINP was 43.9 micrograms/L and no patient exceeded the upper limit of the normal range (80 micrograms/L). We report here for the first time that the concentration of the type I collagen degradation product ICTP in serum shows a close correlation with the extent of skin fibrosis in patients with SSc. We conclude that the increased deposition of type I collagen in this disease is accompanied by an increased turnover of this molecule, indicating a more complex derangement of synthetic and degradative processes than previously acknowledged.

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Section: Discussionmentioning

confidence: 52%

“…liver fibrosis, SSc and lung fibrosis. [5][6][7] However, relatively little is known about the rate and mechanism of collagen turnover in these diseases in vivo.…”

mentioning

confidence: 99%

Circulating type I collagen degradation products: a new serum marker for clinical severity in patients with scleroderma?

Hunzelmann

Risteli

Sacher

et al. 1998

British Journal of Dermatology

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“…Proteolytic enzymes may well be also activated in psoriasis and cause rapid and increased degradation of PIlIP. A low affinity for degraded forms of PIlIP in the assay we used in this study (16,17) might explain why PIlIP levels in psoriasis were lower than those in the control. Increased turnover of collagen without its accumulation may occur in SLE.…”

Section: Discussionmentioning

confidence: 93%

“…Since most cases of GM have a selfhealing tendency as the mean disease duration of our cases (2 ± 1 years) was much shorter than those of the other groups including MCTD and PSS, it may well be that the connective tissue activation discussed in regard to psoriasis and SLE is also present in GM. A more complete Fab assay which measures not only intact but also degraded forms (16,17) would be helpful in verifying these speculations.…”

Section: Discussionmentioning

confidence: 99%

Prolyl Hydroxylase and Procollagen III Peptide Levels in the Sera from Patients with Collagen Diseases and Psoriasis

Sasaki

Nakajima

1989

The Journal of Dermatology

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Prolyl hydroxylase (PH) and procollagen III peptide (PIIIP) levels in 43 serum samples from various collagen diseases including 19 progressive systemic sclerosis (PSS) cases were simultaneously assayed and compared to those of 22 age-matched psoriasis patients and 20 healthy controls in order to examine whether they can serve as useful parameters of fibrotic changes, especially in the skin. Serum PH levels were determined with enzyme immunoassay and serum PIIIP levels with radioimmunoassay. The average values of PH were 109% (n = 3, (p less than 0.001; generalized morphea, GM), 104% (n = 1; overlap syndrome, OS), 57% (n = 6; mixed connective tissue disease, MCTD), 48% (p less than 0.05; PSS), 33% (n = 5; dermatomyositis, DM), 28% (psoriasis), and 9% (n = 9; systemic lupus erythematosus, SLE), higher than the control levels, while those of PIIIP were 82% (OS), 57% (MCTD), 42% (p less than 0.01; PSS), 11% (GM), and 5% (DM) higher and 22% (psoriasis) and 28% (SLE), lower than the controls. These results indicate that both values were higher in the diseases with skin fibrosis and lower in those without it, suggesting that both assays generally reflect the state of actual progression of fibrosis at the point of determination. These two values had considerable correlation; however, GM, psoriasis and SLE patients had relatively higher values of PH than of PIIIP. This result suggests that the PH assay may also reflect the state of connective tissue activation; therefore the PIIIP assay may be more specific for pathological fibrotic process.(ABSTRACT TRUNCATED AT 250 WORDS)

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“…Type I11 collagen is synthesized by fibroblasts, smooth muscle, and endothelial cells as a soluble precursor form, type I11 procollagen, which is secreted and then undergoes a maturation process in which the amino-terminal propeptide, P-111-NP, is cleaved and released into the extracellular space [Timpl and Glanville, 19811. The peptide serves as an indicator of type I11 collagen synthesis; increased serum levels are detectable during rapid growth, puberty, pregnancy, hyperthyroidism, and in fibrotic conditions such as chronic liver diseases and systemic sclerosis [Rohde et al, 1979;Timpl and Glanville, 1981;Trivedi et al, 1985Trivedi et al, , 1986Krieg et al, 1986;Charrie et al, 1986;Risteli et al, 19871. In EDS type IV patients, the serum P-111-NP level appears to be determined by the nature of the underlying molecular defect. For example, fibroblasts from patient 6 synthesize a thermally unstable type I11 procollagen, which is poorly secreted [ Superti-Furga and Steinmann, 19881, while fibroblasts from patient 1, who carries a deletion in one of the genes coding for type I11 procollagen, secrete reduced amounts of mutant type I11 procol-lagen with a structural abnormality that interferes with cleavage of P-111-NP [Superti-Furga et al, 19881. In both patients, serum P-111-NP levels were abnormally low.…”

Section: Discussionmentioning

confidence: 99%

Ehlers‐Danlos syndrome type IV: A subset of patients distinguished by low serum levels of the amino‐terminal propeptide of type III procollagen

et al. 1989

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Serum levels of procollagen type III aminopropeptide (P-III-NP), a peptide released during conversion of type III procollagen to collagen, were abnormally low in six of 10 patients with Ehlers-Danlos syndrome (EDS) type IV (arterial-ecchymotic type) and low-normal in 4. The serum P-III-NP levels correlated with the amount of type III procollagen secreted by the patients' cultured fibroblasts. Serum P-III-NP determination is a simple test that identifies a major subgroup of patients with this life-threatening, dominantly inherited connective tissue disorder and may be especially helpful in making the diagnosis in children.

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Type III Collagen Aminopropeptide Levels in Serum of Patients With Progressive Systemic Scleroderma

Cited by 55 publications

References 20 publications

Circulating type I collagen degradation products: a new serum marker for clinical severity in patients with scleroderma?

Circulating type I collagen degradation products: a new serum marker for clinical severity in patients with scleroderma?

Prolyl Hydroxylase and Procollagen III Peptide Levels in the Sera from Patients with Collagen Diseases and Psoriasis

Ehlers‐Danlos syndrome type IV: A subset of patients distinguished by low serum levels of the amino‐terminal propeptide of type III procollagen

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