Amish, Mennonite, and Hutterite
Genetic Disorder Database

Muscular dystrophy, limb-girdle, autosomal recessive 4

Disorder
OMIM #: #604286  (Click to access OMIM database)
Disorder: Muscular dystrophy, limb-girdle, autosomal recessive 4 
Also known as: Muscular dystrophy, limb-girdle, type 2
LGMDR4
LGMD2E 
Clinical
Phenotype: Progressive weakness, atrophy which predominates in the shoulder, pelvic girdle, and trunk, facial muscles are unaffected, hypotonia, muscle weakness, decreased sarcyglycan proteins, increased serum creatine kinase 
Seen In: Amish
Old Order Mennonite
Old Colony Mennonite
Unknown/Other Mennonite
Hutterite
Remarks: Autosomal recessive 
Mutations
1   Amish  
Gene: SGCB
Base Change: C>G, at nucleotide 461
Amino Acid Change: thr 151 --> arg
Last updated: 2024-09-24 

References
Lim L, Duclos F, Broux O, Bourg N, Sunada Y, Allamand V, Meyer J, Richard I, Moomaw C, Slaughter C, Tome F, Fardeau M, Jackson C, Beckmann J, and Campbell K. (1995) B-sarcoglycan: characterization and role in limb-gridle muscular dystrophy linked to 4q12. Nature genetics 11: 257-265.
PubMed ID: 7581448 

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