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PMID: 7937874 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, P.H.S.

Human adhalin is alternatively spliced and the gene is located on chromosome 17q21.

McNally EM, Yoshida M, Mizuno Y, Ozawa E, Kunkel LM

Abstract

Mutations in the dystrophin gene cause the X chromosome-linked, recessive Duchenne and Becker muscular dystrophies. Dystrophin, a large cytoskeletal protein, copurifies with a complex of dystrophin-associated proteins which serve to anchor dystrophin to the sarcolemma. One of these associated proteins, adhalin, has been implicated as a candidate for severe childhood autosomal recessive muscular dystrophy (SCARMD) due to absence of anti-adhalin staining in muscle biopsy samples taken from SCARMD patients. Furthermore, the Duchenne-like dystrophic phenotype seen in the SCARMD families was shown to be tightly linked to chromosome 13 markers. To determine the genetic mutation responsible for autosomal dystrophy, we characterized the human adhalin gene. Contrary to our expectation, human adhalin was mapped to chromosome 17q21, excluding adhalin as the gene causing chromosome 13-associated SCARMD. Additionally, a splice form of adhalin message was found that predicts a 35-kDa nontransmembrane adhalin. The expression of both adhalin splice forms is exclusively restricted to striated muscle, unlike other components of the dystrophin-glycoprotein complex.

MeSH Terms
Alternative Splicing Amino Acid Sequence Animals Base Sequence Blotting, Northern Chromosome Mapping Chromosomes, Human, Pair 17 Cloning, Molecular Cytoskeletal Proteins/biosynthesis,genetics DNA Primers DNA, Complementary/isolation & purification Gene Expression Humans Karyotyping Membrane Glycoproteins/biosynthesis,genetics Molecular Sequence Data Muscular Dystrophies/genetics Polymerase Chain Reaction RNA, Messenger/biosynthesis Rabbits Sarcoglycans
Chemicals
Cytoskeletal Proteins DNA Primers DNA, Complementary Membrane Glycoproteins RNA, Messenger Sarcoglycans
Authors & Affiliations
5 authors, click to expand affiliations / ORCID
McNally E M
Division of Genetics, Children's Hospital, Boston, MA.
Yoshida M
Mizuno Y
Ozawa E
Kunkel L M
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32 references, click to expand
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Article Info
Journal
Proceedings of the National Academy of Sciences of the United States of America
Abbr.
Proc Natl Acad Sci U S A
ISSN
0027-8424
Published
1994-10-11
Pages
9690-4
Language
English
Region
United States
NLM ID
7505876
PMCID
PMC44882
Subset
IM
Grants
NINDS NIH HHS · NS 23740 · United States
Databases
GENBANK
L34355
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