Quality Standard:【产品介绍】The alpha-actinins are a multigene family
of four actin-binding proteins related to dystrophin.
The two skeletal muscle isoforms of alpha-actinin, ACTN2
and ACTN3, are major structural components of the
Z-line involved in anchoring the actin-containing
thin filaments. In humans, ACTN2 is expressed in all
muscle fibers, while ACTN3 expression is restricted to
a subset of type 2 fibers. Absence of alpha-actinin-3 is
not associated with an obvious disease phenotype,
raising the possibility that ACTN3 is functionally
redundant in humans, and that alpha-actinin-2 is able
to compensate for alpha-actinin-3 deficiency (1).
Alpha-Actinin-3 is specifically expressed in
fast-twitch myofibers responsible for generating force
at high velocity. The absence of a disease
phenotype secondary to alpha-actinin-3 deficiency is
likely due to compensation by the homologous protein,
alpha-actinin-2. However, the high degree of
evolutionary conservation of ACTN3 suggests
function(s) independent of ACTN2. Highly
significant associations between ACTN3 genotype and
athletic performance have been demonstrated in both male
and female athletes.
Recommended Applications
WB
Applications and Recommended Dilution Factors
WB IHC ICC FC IP
1:1,000 - 10,000
Species Reactivity*
Human Mouse Rat
Positive Positive Positive
Western blot analysis on (A) human skeletal muscle,
(B) mouse skeletal muscle, and (C) rat skeletal
muscle lysates using anti-Alpha-Actinin 3 RabMAb
(cat. # 2952-1).
Specificity
A synthetic peptide corresponding to residues in human
Alpha-Actinin 3 was used as an immunogen.