antibody

Rabbit Actin-alpha-1 Antibody

MBS001201

0.05 ml

270 USD

polyclonal

rabbit

nonconjugated

human, mouse, rat

western blot, ELISA, immunohistochemistry, enzyme immunoassay

Antibody

Rabbit Actin-alpha-1 Antibody

Actin-alpha-1

Actin-a-1

actin, alpha skeletal muscle; Actin, alpha skeletal muscle; actin, alpha skeletal muscle; nemaline myopathy type 3; actin, alpha 1, skeletal muscle; Alpha-actin-1

ACTA1; ACTA1; ACTA; ASMA; CFTD; MPFD; NEM1; NEM2; NEM3; CFTD1; CFTDM; ACTA

ACTS_HUMAN

Rabbit

Human, mouse, rat

377

Phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol

1 mg/ml

ELISA (EIA), Immunohistochemistry (IHC), Western Blot (WB)

4501881

NP_001091.1

NM_001100.3

P68133

42,051 Da

Caspase Cascade In Apoptosis Pathway 137974!!Hypothetical Network For Drug Addiction Pathway 198776!!Myometrial Relaxation And Contraction Pathways 198759!!RhoA Signaling Pathway 169356!!Signaling Events Mediated By Focal Adhesion Kinase Pathway 169348!!Striated Muscle Contraction Pathway 198903

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. [provided by RefSeq, Jul 2008]

Function: Actins are highly conserved proteins that are involved in various types of cell motility and are ubiquitously expressed in all eukaryotic cells. Subunit structure: Polymerization of globular actin (G-actin) leads to a structural filament (F-actin) in the form of a two-stranded helix. Each actin can bind to 4 others. Identified in a complex composed of ACTA1, COBL, GSN AND TMSB4X . By similarity. Interacts with TTID. Interacts (via its C-terminus) with USP25; the interaction occurs for all USP25 isoforms but is strongest for isoform USP25m in muscle differentiating cells. Ref.8 Ref.9. Subcellular location: Cytoplasm cytoskeleton. Post-translational modification: Oxidation of Met-46 by MICALs (MICAL1, MICAL2 or MICAL3) to form methionine sulfoxide promotes actin filament depolymerization. Methionine sulfoxide is produced stereospecifically, but it is not known whether the (S)-S-oxide or the (R)-S-oxide is produced . By similarity. Involvement in disease: Nemaline myopathy 3 (NEM3) [MIM:161800]: A form of nemaline myopathy. Nemaline myopathies are muscular disorders characterized by muscle weakness of varying severity and onset, and abnormal thread-or rod-like structures in muscle fibers on histologic examination. Some patients present areas devoid of oxidative activity containg (cores) within myofibers. Core lesions are unstructured and poorly circumscribed.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.3 Ref.11 Ref.13 Ref.14 Ref.16 Ref.17 Ref.18 Ref.19 Ref.20 Ref.22Myopathy, actin, congenital, with excess of thin myofilaments (MPCETM) [MIM:161800]: A congenital muscular disorder characterized at histological level by areas of sarcoplasm devoid of normal myofibrils and mitochondria, and replaced with dense masses of thin filaments. Central cores, rods, ragged red fibers, and necrosis are absent.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.3Congenital myopathy with fiber-type disproportion (CFTD) [MIM:255310]: Genetically heterogeneous disorder in which there is relative hypotrophy of type 1 muscle fibers compared to type 2 fibers on skeletal muscle biopsy. However, these findings are not specific and can be found in many different myopathic and neuropathic conditions.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.15 Ref.21. Miscellaneous: In vertebrates 3 main groups of actin isoforms, alpha, beta and gamma have been identified. The alpha actins are found in muscle tissues and are a major constituent of the contractile apparatus. The beta and gamma actins coexist in most cell types as components of the cytoskeleton and as mediators of internal cell motility. Sequence similarities: Belongs to the actin family.

0.05 ml

270 USD