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Sex-linked dominant

Definition

Sex-linked dominant is a rare way that a trait or disorder can be passed down through families. One abnormal gene on the X chromosome can cause a sex-linked dominant disease.

Related terms and topics include:

Alternative Names

Inheritance - sex-linked dominant; Genetics - sex-linked dominant; X-linked dominant; Y-linked dominant

Information

Inheritance of a specific disease, condition, or trait depends on the type of chromosome that is affected. It can be either an autosomal chromosome or a sex chromosome. It also depends on whether the trait is dominant or recessive. Sex-linked diseases are inherited through one of the sex chromosomes, which are the X and Y chromosomes.

Dominant inheritance occurs when an abnormal gene from one parent can cause a disease, even though a matching gene from the other parent is normal. The abnormal gene dominates the gene pair.

For an X-linked dominant disorder: If the father carries the abnormal X gene, all of his daughters will inherit the disease and none of his sons will have the disease. That is because daughters always inherit their father's X chromosome. If the mother carries the abnormal X gene, half of all their children (daughters and sons) will inherit the disease tendency.

For example, if there are four children (two boys and two girls) and the mother is affected (she has one abnormal X and has the disease) but the father does not have the abnormal X gene, the expected odds are:

  • Two children (one girl and one boy) will have the disease
  • Two children (one girl and one boy) will not have the disease

If there are four children (two boys and two girls) and the father is affected (he has one abnormal X and has the disease) but the mother is not, the expected odds are:

  • Two girls will have the disease
  • Two boys will not have the disease

These odds do not mean that the children who inherit the abnormal X will show severe symptoms of the disease.

References

Feero WG, Zazove P, Chen F. Clinical genomics. In: Rakel RE, Rakel D, eds. Textbook of Family Medicine. 9th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 43.

Groden J, Gocha AS, Croce CM. Human basic genetics and patterns of inheritance. In: Creasy RK, Resnik R, Iams JD, Lockwood CJ, Moore TR, Greene MF, eds. Creasy and Resnik's Maternal-Fetal Medicine: Principles and Practice. 7th ed. Philadelphia, PA: Elsevier Saunders; 2014:chap 1.

Korf BR. Principles of genetics. In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 40.

Review Date:1/25/2016
Reviewed By:Chad Haldeman-Englert, MD, FACMG, Fullerton Genetics Center, Asheville, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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