Sarcomeric α-actinins and their role in human muscle disease

PJ Houweling, KN North - Future Neurology, 2009 - Future Medicine
Future Neurology, 2009Future Medicine
In skeletal muscle, the sarcomeric α-actinins (α-actinin-2 and-3) are a major component of
the Z-line and crosslink actin thin filaments to maintain the structure of the sarcomere. Based
on their known protein binding partners, the sarcomeric α-actinins are likely to have a
number of structural, signaling and metabolic roles in skeletal muscle. In addition, the α-
actinins interact with many proteins responsible for inherited muscle disorders. In this paper,
we explore the role of the sarcomeric α-actinins in normal skeletal muscle and in the …
In skeletal muscle, the sarcomeric α-actinins (α-actinin-2 and -3) are a major component of the Z-line and crosslink actin thin filaments to maintain the structure of the sarcomere. Based on their known protein binding partners, the sarcomeric α-actinins are likely to have a number of structural, signaling and metabolic roles in skeletal muscle. In addition, the α-actinins interact with many proteins responsible for inherited muscle disorders. In this paper, we explore the role of the sarcomeric α-actinins in normal skeletal muscle and in the pathogenesis of a range of neuromuscular disorders.
Future Medicine