Share:
Child's mane can hardly be tamed Where the Struwwelpeter syndrome comes from
For some children, the decomposed hair can not be combed despite all efforts. Researchers have now discovered that certain genes are responsible for Struwwelpeter syndrome. However, the scientists do not have a solution to the problem.
Syndrome of unbreakable hair
Although many parents know that children are often not easy to style, even the most stubborn knots can usually be solved with a lot of patience and strong nerves. Sometimes the brush just does not stand a chance. In German, this phenomenon is referred to as "syndrome of unkämmbaren hair" or as "Struwwelpeter syndrome". Almost nothing was known about the causes - probably because there are only very few cases.
Some children can only be styled with much patience and strong nerves. In children with the "Struwwelpeter syndrome", the brush just has no chance. Researchers have discovered that there are certain genes behind this hair anomaly. (Image: bonzodog / fotolia.com) Symptoms most pronounced in childhood
Researchers from the Universities of Bonn and Toulouse have now identified mutations in three genes responsible for this. Altogether, scientists from eight countries participated in the work. The results have now been published in the journal "American Journal of Human Genetics".
As the University of Bonn writes in a statement, sufferers have "extremely frizzy, dry, mostly fair-haired main hairs with a characteristic shine, which successfully resist any effort to tame them. These symptoms are most pronounced in childhood and then subside with time. "In adulthood, the hair can usually according to the experts hairdressing more or less normal.
Around 100 cases documented worldwide
In the literature, the "Struwwelpeter syndrome" was first described in 1973; now over one hundred cases worldwide have been documented. "But we assume that there are significantly more people affected," explains Professor Dr. med. Regina Betz from the Institute of Human Genetics at the University of Bonn.
"Anyone who suffers from hair that can not be combed, therefore, does not necessarily seek out a doctor or a clinic." However, it is well known that the anomaly is common in some families - so it seems to have genetic causes.
Mutations in three hereditary triggers
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.
Subsequently, the 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. 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," the researchers say. 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.
Why it sometimes comes to disruptions
Disturbances in just one of the components have fundamental effects on the structure and stability of the hair, according to the researchers. Thus, they found that mice with defective PADI3 or TGM3 genes develop characteristic coat abnormalities that are very similar to the human Struwwelpeter.
"From the mutations found, a lot can be learned about the mechanisms involved in the formation of healthy hair, and why it sometimes comes to disturbances," said Prof. Regina Betz. "At the same time, we can now secure the clinical diagnosis of" unkämmbare hair "with molecular genetic methods."
Hair anomalies with serious comorbidities
The last point is good news for affected people with hair disorders, because some hair anomalies are associated with severe comorbidities, which sometimes manifest in later life years.
The Struwwelpeter syndrome, however, usually occurs in isolation without further health problems. According to Betz, the unkämmbaren hair are annoying and possibly also a psychological burden. "Otherwise, however, those affected need not worry." (Ad)