Abstract
Pendred syndrome is an autosomal-recessive disorder characterized by congenital sensorineural hearing loss combined with goiter. This disorder may account for up to 10% of cases of hereditary deafness. The disease gene (PDS/SLC26A4) has been mapped to chromosome 7q22-q31 and encodes a chloride-iodide transport protein. Mutations in this gene are also a cause of non-syndromic autosomal recessive hearing impairment (DFNB4). We have analyzed the PDS/SLC26A4 gene in Spanish and Italian families and we have detected five novel mutations (X781W, T132I, IVS2-2A>G, Y556H and 406del5).
Copyright 2002 Wiley-Liss, Inc.
Publication types
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Research Support, Non-U.S. Gov't
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Corrected and Republished Article
MeSH terms
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Alleles
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Alternative Splicing / genetics
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Carrier Proteins / genetics*
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DNA / chemistry
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DNA / genetics
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DNA Mutational Analysis
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Family Health
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Genotype
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Goiter / complications
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Goiter / genetics
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Hearing Loss, Sensorineural / complications
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Hearing Loss, Sensorineural / genetics*
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Humans
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Italy
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Mediterranean Region
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Membrane Transport Proteins*
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Mutation
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Mutation, Missense
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Phenotype
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Sequence Deletion
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Spain
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Sulfate Transporters
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Syndrome
Substances
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Carrier Proteins
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Membrane Transport Proteins
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SLC26A4 protein, human
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Sulfate Transporters
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DNA