Fragile X syndrome is a genetic disorder caused by an expansion of a repeated DNA sequence within the FMR1 gene on the X chromosome, which silences the gene and eliminates the protein it normally produces, a protein needed for typical brain development. It is the most common inherited cause of intellectual disability and the most common known single-gene cause of autism spectrum disorder, with affected individuals often also showing large ears, a long face, and anxiety or attention difficulties. The condition takes its name from a fragile-appearing constriction on the X chromosome first observed under the microscope in the 1960s and 1970s, before the FMR1 gene itself was identified in 1991.
Facts
Classification
ICD-10 ChapterXVII: Congenital Malformations, Deformations and Chromosomal Abnormalities 2 Communicable or Non-communicable Transmission Route First DescribedThe fragile site itself was identified later (1969 Lubs, 1970 Hecht coined the term); 1943 is recorded here as the article's own earliest dated description of the condition's pedigree. Prevalence Band Causative Agent
Causative Agent (category) Sources
1. Fragile X syndrome (Wikipedia)
WikipediaLead section, body-system statement
Fragile X syndrome (FXS) is a genetic neurodevelopmental disorder.
- Fragile X syndrome (FXS) is a genetic neurodevelopmental disorder.
Wikipedia: Fragile X Syndrome, History section
In 1943, British neurologist James Purdon Martin and British geneticist Julia Bell described a pedigree of X-linked intellectual disability, without considering the macroorchidism (larger testicles).
View the Source 2. Wikipedia: ICD-10
WikipediaList of chapters, Chapter XVIIQuote, List of chapters, Chapter XVII
Congenital malformations, deformations and chromosomal abnormalities
View the Source 3. Wikipedia: Non-communicable disease
WikipediaLead section, transmission-route statement (inferred from disease-category)
Genetic disorders are caused by errors in genetic information that produce diseases in the affected people.
Inherited/genetic diseases section
Genetic disorders are caused by errors in genetic information that produce diseases in the affected people.
View the Source 4. Orphadata (Orphanet): Orphadata: Epidemiology of rare diseases (en_product9_prev)
Orphadata (Orphanet)ORPHA:908Quote, ORPHA:908
Point prevalence Value and class 1-5 / 10 000
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