骨骼肌蛋白FHL1抗体

英文名称    FHL1    

中文名称    骨骼肌蛋白FHL1抗体    

别    名    bA535K18.1; FHL 1; FHL 1B; FHL-1; FHL1; FHL1 protein; FHL1_HUMAN; FHL1A; FHL1B; FLH1A; Four and a half LIM domains 1; Four and a half LIM domains protein 1; Four and a half Lin11 Isl 1 and Mec 3 domains 1; KYO T; LIM protein SLIMMER; MGC111107; RAM14-1; RBP associated molecule 14-1; Skeletal muscle LIM protein 1; Skeletal muscle LIM-protein 1; SLIM 1; SLIM; SLIM-1; SLIM1; SLIMMER; XMPMA.    

说 明 书    0.1ml  0.2ml      

研究领域    心血管  转录调节因子  锌指蛋白  表观遗传学      

抗体来源    Rabbit    

克隆类型    Polyclonal    

交叉反应    Human, Mouse, Rat, Dog, Pig, Cow, Sheep,     

产品应用    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 骨骼肌蛋白FHL1抗体（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.    

分 子 量    35kDa    

细胞定位    细胞核 细胞浆     

性    状    Lyophilized or Liquid    

浓    度    1mg/1ml    

免 疫 原    KLH conjugated synthetic peptide derived from human FHL1    

亚    型    IgG    

纯化方法    affinity purified by Protein A    

储 存 液    Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4    

保存条件    Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.    

骨骼肌蛋白FHL1抗体产品介绍    background:

This gene encodes a member of the four-and-a-half-LIM-only protein family. Family members contain two highly conserved, tandemly arranged, zinc finger domains with four highly conserved cysteines binding a zinc atom in each zinc finger. Expression of these family members occurs in a cell- and tissue-specific mode and these proteins are involved in many cellular processes. Mutations in this gene have been found in patients with Emery-Dreifuss muscular dystrophy. Multiple alternately spliced transcript variants which encode different protein isoforms have been described.[provided by RefSeq, Nov 2009]

Function:
May have an involvement in muscle development or hypertrophy.

Subcellular Location:
Isoform 1: Cytoplasm. 
Isoform 3: Cytoplasm. Nucleus. 
Isoform 2: Nucleus. Cytoplasm, cytosol. Note=Predominantly nuclear in myoblasts but is cytosolic in differentiated myotubes.

Tissue Specificity:
Isoform 1 is highly expressed in skeletal muscle and to a lesser extent in heart, placenta, ovary, prostate, testis, small intestine, colon and spleen. Expression is barely detectable in brain, lung, liver, kidney, pancreas, thymus and peripheral blood leukocytes. Isoform 2 is expressed in brain, skeletal muscle and to a lesser extent in heart, colon, prostate and small intestine. Isoform 3 is expressed in testis, heart and skeletal muscle.

DISEASE:
Defects in FHL1 are the cause of X-linked dominant scapuloperoneal myopathy (SPM) [MIM:300695]. Scapuloperoneal syndrome (SPS) was initially described more than 120 years ago by Jules Broussard as 'une forme hereditaire d'atrophie musculaire progressive' beginning in the lower legs and affecting the shoulder region earlier and more severely than distal arm. The etiology of this condition remains unclear.
Defects in FHL1 are the cause of X-linked myopathy with postural muscle atrophy (XMPMA) [MIM:300696]. Myopathies are inherited muscle disorders characterized by weakness and atrophy of voluntary skeletal muscle, and several types of myopathy also show involvement of cardiac muscle. XMPMA is a distinct form of adult-onset X-linked recessive myopathy with several features in common with other myopathies, but the presentation of a pseudoathletic phenotype, scapuloperoneal weakness, and bent spine is unique and might render the clinical phenotype distinguishable from other myopathies. 
Defects in FHL1 are the cause of X-linked severe early-onset reducing body myopathy (RBM) [MIM:300717]. RBM is a rare muscle disorder causing progressive muscular weakness and characteristic intracytoplasmic inclusions in myofibers. Clinical presentations of RBM have ranged from early onset fatal to childhood onset to adult onset cases. 
Defects in FHL1 are the cause of X-linked childhood-onset reducing body myopathy (CO-RBM) [MIM:300718]. This disorder is allelic to severe early-onset reducing body myopathy (RBM) [MIM:300717].

Similarity:
Contains 3 LIM zinc-binding domains.

Gene ID:
2273    

骨骼肌蛋白FHL1抗体

hz-0135P HSP-90 Beta (heat sock protein-90 Beta)  热休克蛋白-90β （抗原）

hz-0284P IASPP(inhibitory mhzber of the ASPP family)  肿瘤细胞凋亡ASPP家族的另一个成员(抗原)

hz-0608P CD54/ICAM-1  细胞间粘附分子-1

hz-0812P IL-1 Beta(Interleukin-1 Beta)  白介素1β（白细胞介素-1β）(多肽抗原)

hz-0445P IL1RAPL1 (Interleukin 1 receptor accessory protein-like 1)  白介素受体相关蛋白样1前体蛋白（抗原）

hz-0379P IL-6(Interleukin-6)  白介素6（白细胞介素-6）(多肽抗原)

hz-0698P IL-10(Interleukin-10)  白介素10（白细胞介素-10）(抗原)

hz-0780P IL-8  白介素8（白细胞介素-8）(抗原)

hz-0681P Insulin Receptor(ISR)  胰岛素受体（抗原）

hz-0641P Integrin Alpha 4/ITGA4(Integrin alpha 4)  整合素-α（抗原）

hz-0705P LDL-R(Low-density lipoprotein receptor precursor)  低密度脂蛋白受体(抗原)

hz-0722P Ki-67 peptides  Ki-67 Antigen(多肽抗原)

hz-0749P Kiss-1(Metastasis-suppressor KiSS-1 precursor)  肿瘤转移抑制基因（抗原)

hz-0864P 重组K3蛋白  重组K3蛋白

hz-2143P Snake poison Protein  蛇毒蛋白

hz-2144P Snake poison Protein  蛇毒蛋白

hz-0910P Batroxobin Protein  蛇毒巴曲酶全蛋白

hz-0409P Leptin  瘦素（抗原)

hz-0410P Leptin receptor  瘦素受体（抗原）

hz-0759P Lpin1 protein  Lpin1 protein(抗原)

hz-0484P Lumbrokinase  蚯蚓纤溶酶(蚓激酶抗原)

hz-0738P Livin (Inhibitors of apoptosis proterins Livin)  一种新的凋亡抑制蛋白(抗原)

hz-0637P p38 MAPK/MKK3/MAPKK14  丝裂原活化蛋白激酶p38(抗原)

hz-0401P Metal ion transporter  拟南介金属离子转运蛋白(抗原)

hz-0642P MADCAM-1  黏膜选址素（抗原）

hz-0257P MAG-a/b (Myelin associated glycoprotein; L / S -MAG;)  髓鞘相关糖蛋白a/b(抗原)

hz-0258P MAG (Myelin associated glycoprotein; L-MAG;MAG-a )  髓鞘相关糖蛋白(抗原)

hz-0463P MMP-1(Mous hzatrix metalloproteinases-1)  基质金属蛋白酶-1（小鼠抗原）

hz-0761P midnolin isoform Protein 1  中脑核仁蛋白1（抗原）

hz-0762P midnolin isoform Protein 2  中脑核仁蛋白2（抗原）

hz-0832P MICA(MHC class I polypeptide-related sequence A)  一种细胞应激分子抗原

hz-0656P MRP3(Multidrug Resistanec-Associated Protein 3)  多药耐药相关蛋白3(抗原)

hz-0657P MRP1(Multidrug Resistanec-Associated Protein 1)  多药耐药相关蛋白1(抗原)