Affected individuals are usually homozygous for the abnormal gene; each unaffected parent will be a heterozygous carrier. Children affected with AR disorders are usually born to unaffected parents, each of whom carries one copy of the variant. If both members of a couple are carriers (or heterozygotes) for this variant, each of their offspring has a 25% chance of being affected. Risk of these disorders varies between populations and is increased by consanguinity.

Examples of autosomal recessive disorders:

  • Sickle cell disease
  • Thalassemia
  • Congenital adrenal hyperplasia
  • Cystic fibrosis
  • Friedreich ataxia
  • Galactosemia
  • Glycogen storage diseases
  • Hurler syndrome
  • Oculocutaneous albinism
  • Phenylketonuria

Autosomal recessive disorders often affect metabolic pathways, whereas autosomal dominant disorders often affect structural proteins