Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.4.24.3 (collagenase)
 18,340 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The diagnosis of hereditary epidermolysis bullosa dystrophica (Hallopeau-Siemens) in three patients was confirmed by clinical and histological means as well as by electron microscopy. The clinical course was followed for many years. Since (+)-cyanidanol-3 has been shown to reduce the susceptibility of collagen to mammalian collagenase under in vitro conditions, (+)-cyanidanol-3 was used to treat the three patients for a period of 27 weeks. A decrease in the amount of blistering was observed in all three patients.
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PMID:[Epidermolysis bullosa hereditaria dystrophica (Hallopeau-Siemens). Case report and therapy trial with (+)-cyanidanol-3 in 3 patients]. 367 17

Recent biochemical advances have contributed to clarification of certain skin diseases and metabolic disturbances with predominantly cutaneous symptoms. This is illustrated by the various forms of porphyria. Today we differentiate four hepatic forms: acute intermittent porphyria, variegate porphyria, hereditary coproporphyria and porphyria cutanea tarda, and two erythropoietic forms: congenital erythropoietic porphyria and erythropoietic protoporphyria, all of which are due to an inborn enzymatic deficiency of the heme biosynthesis. From the different forms of ichthyosis, the X-recessive ichthyosis has an underlying enzymatic deficiency of the steroid sulfatase, which seems of significance in the disturbance of keratinization. In epidermolysis bullosa dystrophica type Hallopeau-Siemens an increased collagenase activity was detected. Inhibition of this enzyme by phenytoin results in improvement of the blistering in this genodermatosis. The etiology and pathogenesis of psoriasis are unclear despite extensive efforts. The recently detected deficiency of the arylhydrocarbon-hydroxylase and its inducibility must be confirmed, additionally its significance in the pathogenesis of this disease is yet to be evaluated.
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PMID:[Progress in dermatology: new biochemical aspects]. 629 Mar 59

In epidermolysis bullosa dystrophica recessiva Hallopeau-Siemens, blister formation is due to an excessive synthesis and secretion of a genetically altered collagenase which, under mechanical trauma, becomes activated out of its pro-enzyme and leads to the destruction of collagen in the papillary dermis. There is evidence that phenytoin can inhibit the production of collagenase in the fibroblasts. This paper reports on experiences with the treatment of 6 patients suffering from this epidermolysis bullosa type with phenytoin since 2 years and more. Problems of pharmacokinetics and dosage, possible signs of intoxication, interactions with other drugs as well as side effects of phenytoin are discussed and a therapy regimen schedule is proposed on the basis of these experiences.
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PMID:[Phenytoin therapy in recessive dystrophic epidermolyses (epidermolysis bullosa dystrophica type Hallopeau-Siemens and epidermolysis bullosa dystrophica inversa)]. 685 83