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 isof o r ms of alpha-actinin
(ACTN2 a n d ACTN3) are maj o r structural components of
the Z-line involved in anch o r ing the
actin-containing thin filaments. In humans, ACTN2
is expressed in all muscle fibres, 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, a n d that
alpha-actinin-2 is able to compensate f o r
alpha-actinin-3 deficiency (1). Alpha-Actinin-3
is specifically expressed in fast-twitch
myofibers responsible f o r generating f o r ce 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
a n d athletic perf o r mance have been demonstrated in
both male a n d female athletes. Specificity: A
synthetic peptide c o r responding to residues on
the N-terminus of human alpha-actinin 3 was used as
an immunogen.
Recommended Applications
WB, IHC, ICC, FC
Applications and Recommended Dilution Factors
WB: 1:5,000-10,000
IHC: 1:100 - 250
ICC: 1:100 - 250
FC: 1:200
Species Reactivity *
Human, Mouse, Rat
*
Products Data
Western blot analysis on human skeletal muscle lysate
using anti-alpha-actinin 3 RabMAb (cat. # 2316-1),
dilution 1:10,000.
Immunohistochemical analysis of paraffin-embedded
human muscles using anti-Alpha-Actinin-3 RabMAb
(cat. #2316-1).
Specificity
A synthetic peptide corresponding to residues on
the N-terminus of human alpha-actinin 3 was used as
an immunogen.