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Science The Struwwelpeter is in the genes
The Struwwelpeter is known from the fairy tale of the same name for his wild hairstyle and his little obedient behavior. Some children naturally suffer from completely disheveled hair that is practically incurable, which is known as Struwwelpeter syndrome, scientists at the University of Bonn explain. In a recent study, they identified the genes responsible for the phenomenon of Struwwelpeter syndrome.
Few children worldwide suffer from Struwwelpeter syndrome (also known as "uncurable hair syndrome"), the causes of which remain largely unclear. A research team led by the University of Bonn was able to identify mutations in three genes that lead to hair that can not be combed, according to the Bonn University Communication.
Some people have hair like a Struwwelpeter, which practically can not be combed. The cause lies in the genes. (Image: www.uni-bonn.de/ Photo: private) Comb and brush without chance
Children with Struwwelpeter syndrome have completely disheveled hair that can not be combed. "Many children know from their own experience that children are not always easy to style," the researchers report. But with patience and strong nerves usually the most tenacious knot can be solved. In the Struwwelpeter syndrome "brush or comb, however, have not the slightest chance," the experts continue. Affected show an extremely frizzy, dry, mostly fair-haired main hair with a characteristic shine, explain the researchers.
Struwwelpeter syndrome very rare
Most pronounced are the disheveled hairs of childhood Struwwelpeter syndrome, and then gradually decline, the experts report. In adulthood, the hair is finally usually more or less normal hairstyle. The fact that little is known about the causes of the syndrome, the scientists also point out that the phenomenon is relatively rare.
Well one hundred cases so far documented
The syndrome was first described in the literature in 1973 and since then more than a hundred cases have been documented worldwide, the researchers say. "But we assume that there are significantly more people affected," says Professor. Regina Betz from the Institute of Human Genetics at the University of Bonn. Because who suffers from unkämmbaren hair, so do not necessarily seek a doctor or a clinic.
Suspected genetic cause
It was already known that the anomaly in many families occurs more frequently, which is why the suspicion of a genetic causes was close. A few years ago, the Bonn human geneticist first heard about a family with two affected children from a British colleague at a congress. "Through contacts with colleagues from all over the world, we were able to find nine more children," reports Prof. Betz.
Mutations in three hereditary triggers
Subsequently, the Bonn scientists sequenced all the genes of those affected and, when compared with large databases, they came across mutations in three genes that are involved in the formation of hair, reports the University of Bonn. The genes with the abbreviations PADI3, TGM3 and TCHH are crucial for the development of the syndrome. In the first two genes building instructions for enzymes are included, in the third / TCHH) the instructions for an important protein of the hair shaft.
Proteins of high importance for healthy hair
"In healthy hair, the TCHH proteins are cross-linked by extremely fine horn filaments, which are responsible for the shape and structure of the hair," explain the researchers. The two other genes found are also important here. "PADI3 alters the hair shaft protein TCHH so that the horn filaments can attach to it" and "the TGM3 enzyme then creates the actual link," explains first author Dr. Fitnat Buket Basmanav Ünalan.
Cross-linkage of hair proteins disturbed
In studies on cell cultures, the researchers found that in the present mutation, the cross-linkage of the hair proteins is disturbed. "Even if only one of the three components is not functional, this has fundamental effects on the structure and stability of the hair," reports the University of Bonn. Also, mice in which the PADI3 or TGM3 gene was defective would have developed characteristic coat abnormalities that are very similar to the human phenotype.
Struwwelpeter syndrome without health consequences
Based on the mutations found, a lot can be learned about the mechanisms "involved in the formation of healthy hair, and why it sometimes causes disturbances," emphasizes Prof. Betz. At the same time, the clinical diagnosis "Struwwelpeter syndrome" can now be verified using molecular genetic methods. This is good news for affected people with hair disorders. Because other hair anomalies are quite associated with severe comorbidities that manifest themselves in later years of life. The Struwwelpeter syndrome, however, usually remain without further health impairments. The unkämmbaren hair are annoying, but "otherwise, sufferers do not have to worry," says Prof. Betz.