This is caused by mutations in one of three possible genes; PADI3, TGM, or TCHH. These genes code for proteins involved in hair shaft formation and improvement often occurs in later childhood. Uncombable hair syndrome is mainly autosomal recessive, but it can also be autosomal dominant because there are other involved genes that have yet to be identified.By early adulthood, phenotypic symptoms of UHS spontaneously improve or disappear. The hair of people with UHS may eventually lie flat and appear near normal in texture by adolescence or early adulthood for unknown reasons. Basmanav et al. have suggested that either different, non-mutated isoforms of the hair-forming enzymes are produced as we age, or that aging-related mechanistic changes in hair such as greater diameter and length help resolve UHS. Symptoms
Coarse hair
Trichodysplasia
White hair
Woolly hair
Patchy baldness

Cause
As one would expect from the name, individuals affected by this disorder have difficulty managing and grooming their hair. Affected individuals usually possess blonde or silver hair that may be present at birth or develop over time. The hair is wiry, frizzy, and stands straight out from the scalp. It is often dry but not fragile or brittle. This is due to several structural abnormalities in the hair shaft. A cross-section of the hair shaft from affected individuals reveals a kidney bean, triangular, or oval diameter, unlike the circular shaft shape displayed in straight hair. This irregularity in the diameter of the hair shaft prevents the hair from lying neatly against adjacent hair fibers.
Current clinical trials and research opportunities can be found on their website. See also
List of cutaneous conditions
TGM3
PADI3
Trichohyalin

References
External links

Uncombable hair syndrome at NIHs Office of Rare Diseases