prolidase deficiency/proline

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Leathanach 1 ó 61 torthaí

Collagen biosynthesis anomalies in prolidase deficiency: effect of glycyl-L-proline on the degradation of newly synthesized collagen.

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Logáil Isteach / Cláraigh

Prolidase deficiency is a rare hereditary disease characterized by an iminodipeptiduria especially composed by glycyl-L-proline which is not further degraded. The study of collagen metabolism in fibroblast cultures from three prolidase-deficient patients showed an increase in the rapidly degraded

[Effective therapy with a glycine-proline ointment in a patient with recurrent ulcers from prolidase deficiency].

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú

Logáil Isteach / Cláraigh

BACKGROUND Prolidase deficiency is a rare disease. Lower leg recalcitrant ulcerations are the most characteristic symptoms. METHODS Woman diagnosed of prolidase deficiency with leg recalcitrant and infected ulcerations. Dermatology service solicits a proline and glycline containing ointment after

Human prolidase and prolidase deficiency: an overview on the characterization of the enzyme involved in proline recycling and on the effects of its mutations.

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú

Logáil Isteach / Cláraigh

Here we summarized what is known at the present about function, structure and effect of mutations in the human prolidase. Among the peptidases, prolidase is the only metalloenzyme that cleaves the iminodipeptides containing a proline or hydroxyproline residue at the C-terminal end. It is relevant in

Normal hydroxylation of proline in collagen synthesized by skin fibroblasts from a patient with prolidase deficiency.

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú

Logáil Isteach / Cláraigh

The extent of hydroxylation of proline in collagen synthesized and secreted into the culture medium by skin fibroblasts derived from a patient with prolidase deficiency has been examined and found to be normal. It would seem likely that to a considerable extent the urinary proline-containing

Prolidase deficiency: the use of topical proline for treatment of leg ulcers.

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú

Logáil Isteach / Cláraigh

A 41-year-old man with prolidase deficiency has had chronic leg ulcers and recurrent cellulitis for most of his life. Until recently he had been hospitalized at least annually for this and suffered significant morbidity as a result. Since commencing topical 5% proline in white soft paraffin ointment

Effect of topical application of glycine and proline on recalcitrant leg ulcers of prolidase deficiency.

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú

Logáil Isteach / Cláraigh

Topical proline therapy in prolidase deficiency.

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú

Logáil Isteach / Cláraigh

Liquid chromatography-mass spectrometry for simultaneous analyses of iminodipeptides containing an N-terminal or a C-terminal proline.

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú

Logáil Isteach / Cláraigh

Simultaneous analyses of synthetic iminodipeptides containing an N-terminal proline or a C-terminal proline have been demonstrated using liquid chromatography-mass spectrometry with an atmospheric pressure ionization interface system. The separation of iminodipeptides was carried out on a

N-benzyloxycarbonyl-L-proline: an in vitro and in vivo inhibitor of prolidase.

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú

Logáil Isteach / Cláraigh

Prolidase deficiency (PD) is a recessive disorder of the connective tissue caused by mutations in the prolidase, a specific peptidase, cleaving the dipeptides with a C-terminal prolyl and hydroxyprolyl residue. PD is a complex syndrome characterized mainly by intractable skin lesions, recurrent

Liquid chromatography-mass spectrometry for the qualitative analyses of iminodipeptides in the urine of patients with prolidase deficiency.

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú

Logáil Isteach / Cláraigh

Analyses of standard iminodipeptides and iminodipeptides in the urine of patients with prolidase deficiency have been demonstrated using liquid chromatography-mass spectrometry with an atmospheric pressure ionization interface system. The separation was carried out on a reversed-phase column using

Prolidase deficiency in two dermatological patients in western Sicily.

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Logáil Isteach / Cláraigh

Prolidase deficiency is a rare disorder inherited through an autosomal recessive gene. The hallmark of the disorder are iminodipeptiduria, chronic skin ulcers, recurring infections, mental retardation and characteristic facial appearance, although prolidase deficiency can occur with no clinical

Normal production, nature, and extent of intracellular degradation of newly synthesized collagen in fibroblasts from a patient with prolidase deficiency.

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú

Logáil Isteach / Cláraigh

We have examined the extent of intracellular degradation of newly synthesized collagen occurring in fibroblasts from a patient with prolidase deficiency, a rare, autosomal recessively inherited disorder, in which a lack of prolidase, which normally cleaves imidodipeptides with a C-terminal Pro or

The use of liquid chromatography-mass spectrometry for the identification and quantification of urinary iminodipeptides in prolidase deficiency.

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú

Logáil Isteach / Cláraigh

It has been reported that the urine of patients with prolidase deficiency contains various iminodipeptides with a carboxyl-terminal proline (hydroxyproline). These iminodipeptides have hitherto been detected indirectly by acid hydrolysis or enzymatic digestion, followed by amino acid analysis. In

Measurement of iminodipeptides in the serum of patients with prolidase deficiency using liquid chromatography-mass spectrometry.

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú

Logáil Isteach / Cláraigh

Iminodipeptides containing C-terminal proline or hydroxyproline were determined in sera from patients with prolidase deficiency, in their mother's serum, and in the sera of unrelated controls, using liquid chromatography-mass spectrometry with an atmospheric pressure ionization interface system. The

The effects of serum iminodipeptides and prednisolone on superoxide generation and tyrosyl phosphorylation of proteins in neutrophils from a patient with prolidase deficiency.

Ní féidir ach le húsáideoirí cláraithe ailt a aistriú

Logáil Isteach / Cláraigh

The aim of this study was to examine the effects of serum iminodipeptides and prednisolone on superoxide generation and tyrosyl phosphorylation of proteins in neutrophils from a patient with prolidase deficiency, and also to find the causative effects of superoxide on inflammatory skin lesions. When

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