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Mouse Anti-Actin, alpha skeletal muscle  antibody (bsm-33160M)  
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產(chǎn)品編號 bsm-33160M
英文名稱 Mouse Anti-Actin, alpha skeletal muscle  antibody
中文名稱 肌動蛋白α1單克隆抗體
別    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, skeletal muscle; actina; actine; aktin; alpha Actin 1; alpha skeletal muscle Actin; alpha skeletal muscle; alpha-actin; Beta cytoskeletal actin; nemaline myopathy type 3.  
研究領域 細胞生物  發(fā)育生物學  細胞骨架  
抗體來源 Mouse
克隆類型 Monoclonal
克 隆 號 6C12
交叉反應 Human,Mouse,Rat
產(chǎn)品應用 WB=1:500-1000,IHC-P=1:100-500,IHC-F=1:500-1000,ICC/IF=1:100-500,IF=1:500-1000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 42kDa
細胞定位 細胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ACTA1 
亞    型 IgG
純化方法 affinity purified by Protein G
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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 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, 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.

SWISS:
P68133

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



產(chǎn)品圖片
25 ug total protein per lane of various lysates (see on figure) probed with Actin, alpha skeletal muscle monoclonal antibody, unconjugated (bsm-33160M) at 1:50000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.
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