Actin, alpha1, Polyclonal Antibody

Anti -Actin, alpha1, CT (ACTA1, ACTA, Actin, alpha skeletal muscle)

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 14; NC_000014.8 (69340840..69446083, complement). Location: 14q24; 14q22-q24

Polyclonal

IgG

Rabbit

Recognizes human Actin, alpha 1.

Affinity Purified
Purified by Protein A affinity chromatography.

Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.

1.25 mg/ml (lot specific)

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Small volumes of anti-ACTN1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

The product encoded by this gene belongs to the actin family of proteins, which are highly conserved proteins that play a role in cell motility, structure and integrity. Alpha, beta and gamma actin isoforms have been identified, with alpha actins being a major constituent of the contractile apparatus, while beta and gamma actins are involved in the regulation of cell motility. This actin is an alpha actin that is found in skeletal muscle. Mutations in this gene cause nemaline myopathy type 3, congenital myopathy with excess of thin myofilaments, congenital myopathy with cores, and congenital myopathy with fiber-type disproportion, diseases that lead to muscle fiber defects.

Antibodies; Abs to Actin Related Proteins

Western Blot (WB)

Suitable for use in Western Blot

Dilution:
Western Blot: 1:000
Optimal dilutions to be determined by the reseacher.

103,058 Da[Similar Products]

actinin, alpha 1

alpha-actinin-1; actinin 1 smooth muscle; non-muscle alpha-actinin-1; F-actin cross-linking protein; alpha-actinin cytoskeletal isoform

Alpha-actinin-1

ACTN1_HUMAN

Alpha actinins belong to the spectrin gene superfamily which represents a diverse group of cytoskeletal proteins, including the alpha and beta spectrins and dystrophins. Alpha actinin is an actin-binding protein with multiple roles in different cell types. In nonmuscle cells, the cytoskeletal isoform is found along microfilament bundles and adherens-type junctions, where it is involved in binding actin to the membrane. In contrast, skeletal, cardiac, and smooth muscle isoforms are localized to the Z-disc and analogous dense bodies, where they help anchor the myofibrillar actin filaments. This gene encodes a nonmuscle, cytoskeletal, alpha actinin isoform and maps to the same site as the structurally similar erythroid beta spectrin gene. Three transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

Function: F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein.

Subunit structure: Homodimer; antiparallel. Interacts with DDN, MYOZ2, PDLIM2, TTID and LPP. Interacts with PSD. Interacts with MICALL2

By similarity. Ref.15 Ref.16 Ref.18 Ref.19

Subcellular location: Cytoplasm › cytoskeleton. Cytoplasm › myofibril › sarcomere › Z line. Cell membrane

By similarity. Cell junction

By similarity. Cell projection › ruffle

By similarity. Note: Colocalizes with MYOZ2 and PPP3CA at the Z-line of heart and skeletal muscle. Colocalizes with PSD in membrane ruffles and central reticular structures

By similarity. Ref.14

Involvement in disease: Bleeding disorder, platelet-type 15 (BDPLT15) [MIM:615193]: An autosomal dominant form of macrothrombocytopenia. Affected individuals usually have no or only mild bleeding tendency, such as epistaxis. Laboratory studies show decreased numbers of large platelets and anisocytosis, but the platelets show no in vitro functional abnormalities.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.24

Sequence similarities: Belongs to the alpha-actinin family.Contains 1 actin-binding domain.Contains 2 CH (calponin-homology) domains.Contains 2 EF-hand domains.Contains 4 spectrin repeats.

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