Estrogen modulates plasminogen promoter activity.

Postmenopausal women treated with estrogen hormone replacement therapy and female patients with hypoplasminogenemia receiving oral contraceptives show increasing plasminogen (PLG) concentrations. The elevated PLG levels are in contrast to the estrogen dependent decline of lipoptrotein(a) [Lp(a)], whose main protein component apolipoprotein(a) [APO(a)] is highly homologous to PLG in protein and gene structure and is also located in its immediate vicinity on chromosome 6q26. The intergenic region between both genes comprises several transcription-regulatory regions with enhancer sequences that increase the basal activity of the PLG core promoter.

Duodenal ulceration in a patient with celiac disease and plasminogen I deficiency: coincidence or cofactors?

Celiac disease (CD) is a gluten-dependent inflammatory disease of the small bowel that affects up to 1% of the worldwide population. Despite severe mucosal abnormalities including total villous atrophy and autoantibody deposition, duodenal ulcer is not a feature of CD. However, a recent study found an elevated rate of peptic ulcer disease in patients with CD.

Transcriptional regulation of the human KiSS1 gene.

Kisspeptin, the product of the KiSS1 gene, has emerged as a key component of the mechanism by which the hypothalamus controls puberty and reproductive development. It does so by stimulating the secretion of gonadotropin releasing hormone (GnRH). Little is known about the transcriptional control of the KiSS1 gene.

Isoelectric focusing pattern of plasminogen mutants of patients with hypoplasminogenemia: correlation of in-vitro data with computer-predicted isoelectric points (pI).

Plasminogen (plg), the circulating proenzyme of plasmin in blood, is a polymorphic protein and most of these natural variants have been identified using isoelectric focusing (IEF) gel electrophoresis. Here, we show that a rare plg gene polymorphism 504R/W is associated with IEF phenotype A3 on the protein level. One healthy individual with homozygous plg gene polymorphism 504W studied so far exhibited low normal plg antigen and slightly decreased plg activity, suggesting that this polymorphism is associated with (mild) hypoplasminogenemia.

Inherited plasminogen deficiency presenting as ligneous vaginitis: a case report with molecular correlation and review of the literature.

Type 1 plasminogen deficiency is an inherited and potentially life-threatening systemic disease in which patients develop pseudomembranous lesions of mucosal surfaces exposed to minor trauma. It is most commonly clinically encountered as ligneous conjunctivitis. We report the case of a 39-year-old woman with extensive involvement of the female genital tract.

Ligneous inflammation involving the female genital tract.

Ligneous inflammation is a rare disease characterized by progressive growth of ligneous plaques on mucosal surfaces. Involvement of the female genital tract is an unusual condition. We present a patient with multifocal ligneous inflammation involving her genital tract, oral mucosa and conjunctiva.

Hypoplasminogenemia with ligneous periodontitis: a failed local therapeutic approach.

Background: Hypoplasminogenemia is a rare condition that is associated with ligneous conjunctivitis, a form of chronic conjunctivitis characterized by firm, fibrin-rich, pseudomembranous lesions on the tarsal conjunctivae and oral lesions. Pseudomembranes may develop on the gingivae, and there may be periodontal involvement.

Methods: Several therapeutic approaches have been developed to treat such patients, but they have had limited effect.

Molecular and clinical spectrum of type I plasminogen deficiency: A series of 50 patients.

Severe type I plasminogen (PLG) deficiency has been causally linked to a rare chronic inflammatory disease of the mucous membranes that may be life threatening. Here we report clinical manifestations, PLG plasma levels, and molecular genetic status of the PLG gene of 50 patients. The most common clinical manifestations among these patients were ligneous conjunctivitis (80%) and ligneous gingivitis (34%), followed by less common manifestations such as ligneous vaginitis (8%), and involvement of the respiratory tract (16%), the ears (14%), or the gastrointestinal tract (2%).

Characterization of plasminogen variants in healthy subjects and plasminogen mutants in patients with inherited plasminogen deficiency by isoelectric focusing gel electrophoresis.

Plasmin(ogen) plays an important role in fibrinolysis and wound healing. Severe hypoplasminogenemia has recently been linked to ligneous conjunctivitis. Plasminogen (plg) is known as a polymorphic protein and most of these variants have been identified using isoelectric focusing (IEF) gel electrophoresis.

Ligneous (pseudomembranous) inflammation involving the female genital tract associated with type-1 plasminogen deficiency.

Ligneous (pseudomembranous) inflammation of the female genital tract is a rare and unusual condition characterized by extensive subepithelial fibrin deposition and associated inflammation. Ligneous inflammation in extragenital sites, predominantly the conjunctiva, has been linked to plasminogen deficiency. Individuals with plasminogen deficiency are unable to remove fibrin deposited in injured mucosal tissue.

A K19E missense mutation in the plasminogen gene is a common cause of familial hypoplasminogenaemia.

The prevalence of familial plasminogen deficiency in Scotland has recently been calculated at 2.9/1000. However, little is known of the molecular genetic background and the frequency of plasminogen gene mutations in most cases of inherited plasminogen deficiency.

Ligneous conjunctivitis, hydrocephalus, hydrocele, and pulmonary involvement in a child with homozygous type I plasminogen deficiency.

Unlabelled: Ligneous conjunctivitis is a rare and unusual form of chronic pseudomembranous conjunctivitis which usually starts in early infancy. Plasminogen deficiency has recently been associated with ligneous conjunctivitis. The disease may be associated with pseudomembranous lesions of other mucous membranes in the mouth, nasopharynx, trachea, and female genital tract and also with congenital hydrocephalus.