How is limb-girdle muscular dystrophy type 2B (LGMD2B) inherited?
Limb-girdle muscular dystrophy type 2B (LGMD2B), and all subtypes of LGMD type 2, are inherited in an autosomal recessive manner. This means that people with LGMD2B have pathogenic variations (changes, formerly known as mutations) in both copies of the DYSFgene in each cell of the body. We inherit one copy of each gene from our mother and the other from our father. People who have only one changed copy of the DYSF gene are known as carriers of the disease. Carriers of LGMD2B typically do not have any signs or symptoms of the disease. When two carriers of LGMD2B have children, each child has a:
25% chance to have LGMD2B
50% chance to be a carrier of LGMD2B like each parent
25% chance to have inherited two working copies of the DYSF gene, so he or she is not a carrier and is not affected with the disease
Last updated: 12/26/2017
How common or rare is limb-girdle muscular dystrophy, type 2B?
The exact prevalence of limb-girdle muscular dystrophy, type 2B (LGMD2B) is not known.
Several studies throughout the world have estimated the frequency of LGMDs. Different populations often have different frequencies of the various LGMDs. The overall frequency of all LGMDs has been estimated to be 5-70 people per 1 million in several countries. This is approximately 1 in 14,286 to 1 in 200,000. Some have estimated a slightly smaller prevalence range, from 1 in 14,500 to 1 in 123,000 people.
LGMD2B is thought to account for 3-19% of all LGMDs. In many studies, LGMD2A is the most common (accounting for 8-26% of all LGMDs).
Last updated: 3/21/2016
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