SAB4503474
Anti-Actinin α antibody produced in rabbit
affinity isolated antibody
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All Photos(3)
Synonym(s):
α-actinin skeletal muscle isoform 2, α-actinin-2, α-actinin-3, ACTN2, F-actin cross-linking protein
UNSPSC Code:
12352203
NACRES:
NA.41
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biological source
rabbit
Quality Level
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
form
buffered aqueous solution
mol wt
antigen 103 kDa
species reactivity
human, rat, mouse
concentration
~1 mg/mL
technique(s)
ELISA: 1:20000
immunofluorescence: 1:100-1:500
immunohistochemistry: 1:50-1:100
western blot: 1:500-1:1000
NCBI accession no.
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... ACTN2(88)
General description
Anti-Actinin α Antibody detects endogenous levels of total Actinin α protein.
α-actinin-2 (ACTN2) is a myofibrillar protein that is expressed in cardiac muscle. It is located at the the Z disk. The ACTN2 gene is mapped to human chromosome 1q43.
α-actinin-2 (ACTN2) is a myofibrillar protein that is expressed in cardiac muscle. It is located at the the Z disk. The ACTN2 gene is mapped to human chromosome 1q43.
Immunogen
The antiserum was produced against synthesized peptide derived from human Actinin alpha-2/3.
Immunogen Range: 31-80
Immunogen Range: 31-80
Biochem/physiol Actions
Mutation in the α-actinin-2 (ACTN2) gene leads to hypertrophic cardiomyopathy. It is essential in cross-linking actin and titin filaments in the Z-disc. ACTN2 is associated with a number of cytoskeletal and signaling molecules and is involved in the structural and modulatory roles in the organization of the cytoskeleton and muscle contraction.
Features and Benefits
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Physical form
Rabbit IgG in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
WGK
nwg
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Regulatory Information
常规特殊物品
Certificates of Analysis (COA)
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Clinical utility gene card for: dilated cardiomyopathy (CMD).
Posafalvi A, et al.
European Journal of Human Genetics, 21(10) (2013)
Chang Yoon Doh et al.
Biochimica et biophysica acta. Molecular basis of disease, 1865(3), 661-677 (2019-01-07)
Mutations in cardiac myosin binding protein C (cMyBPC) are a major cause of hypertrophic cardiomyopathy (HCM). In particular, a single amino acid substitution of tyrosine to serine at residue 237 in humans (residue 235 in mice) has been linked to
Hypertrophic cardiomyopathy mutations in the calponin-homology domain of ACTN2 affect actin binding and cardiomyocyte Z-disc incorporation.
Haywood N, et al.
The Biochemical Journal, 473(16), 2485?2493-2485?2493 (2016)
ACTN3 genotype is associated with increases in muscle strength in response to resistance training in women.
Clarkson P M, et al.
Journal of Applied Physiology, 99(1), 154-163 (2005)
AAV9 gene transfer of cMyBPC N-terminal domains ameliorates cardiomyopathy in cMyBPC-deficient mice.
Jiayang Li et al.
JCI insight, 5(17) (2020-08-05)
Decreased cardiac myosin-binding protein C (cMyBPC) expression due to inheritable mutations is thought to contribute to the hypertrophic cardiomyopathy (HCM) phenotype, suggesting that increasing cMyBPC content is of therapeutic benefit. In vitro assays show that cMyBPC N-terminal domains (NTDs) contain
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