肌动蛋白α1抗体
规格：1尘驳/1尘濒
英文名： ACTA1
别名： ACTS_HUMAN; Actin, alpha skeletal muscle; Alpha-actin-1; ACTA1; ACTA; ASMA; CFTD; CFTD1; CFTDM; MPFD; NEM1; NEM2; NEM3; Actin alpha skeletal muscle; actin, alpha 1, skeletal muscle 1; actin, alpha 1,
分子量： 42kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human ACTA1
交叉反应：Human, Dog, Cow, Rabbit,
细胞定位：细胞浆
肌动蛋白α1抗体产物介绍：background: 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: 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. Interacts with TTID. Interacts (via its C-terminus) with USP25; the interaction occurs for all USP25 isoforms but is strongest for isoform USP25min muscle differentiating cells. Subcellular Location: Cytoplasm, cytoskeleton. Post-translational modifications: Oxidation of Met-46 and Met-49 by MICALs (MICAL1, MICAL2 or 肌动蛋白α1抗体MICAL3) to form methionine sulfoxide promotes actin filament depolymerization. MICAL1 and MICAL2 produce the (R)-S-oxide form. The (R)-S-oxide form is reverted by MSRB1 and MSRB2, which promote actin repolymerization. Monomethylation at Lys-86 (K84me1) regulates actin-myosin interaction and actomyosin-dependent processes. Demethylation by ALKBH4 is required for maintaining actomyosin dynamics supporting normal cleavage furrow ingression during cytokinesis and cell migration. 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-like or rod-shaped structures in muscle fibers on histologic examination. Note=The disease is caused by mutations affecting the gene represented in this entry. Myopathy, 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. Myopathy, 肌动蛋白α1抗体congenital, with fiber-type disproportion (CFTD) [MIM:255310]: A 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. Similarity: Belongs to the actin family. Gene ID: 58 Database links: Entrez Gene: 421534 Chicken Entrez Gene: 281592 Cow Entrez Gene: 58 Human Entrez Gene: 11459 Mouse Entrez Gene: 100154254 Pig Entrez Gene: 29437 Rat Omim: 102610 Human SwissProt: P68139 Chicken SwissProt: P68138 Cow SwissProt: P68133 Human SwissProt: P68134 Mouse SwissProt: P68137 Pig SwissProt: P68135 Rabbit SwissProt: P68136 Rat Unigene: 1288 Human Unigene: 214950 Mouse Unigene: 82732 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
肌动蛋白α1抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid