CMD1G,扩张型心肌病蛋白1G抗体

产品编号HZ-9861R
英文名称CMD1G
中文名称扩张型心肌病蛋白1G抗体
别 名MPRM; Cardiomyopathy dilated 1G (autosomal dominant); CMD1G; CMH 9; CMH9; CMPD 4; CMPD4; Connectin; DKFZp451N061; EOMFC; HMERF; LGMD2J; MU RMS 40.14; Rhabdomyosarcoma antigen; Rhabdomyosarcoma antigen MU RMS 40.14; Rhabdomyosarcoma antigen MU-RMS-40.14; Titin; TITIN_HUMAN; TMD; TTN.
说 明 书0.1ml 0.2ml
研究领域心血管 信号转导 表观遗传学
抗体来源Rabbit
克隆类型Polyclonal
交叉反应 Human, Mouse, Rat, Pig, Cow,
CMD1G,扩张型心肌病蛋白1G抗体产品应用ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （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3780kDa
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 Titin/CMD1G
亚 型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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.
CMD1G,扩张型心肌病蛋白1G抗体PubMedPubMed
产品介绍background:
Titin, also known as connectin, is a large protein involved in the temporal and spatial control of the assembly of the highly ordered sarcomeres (contractile units) of striated muscle. In addition to sarcomere assembly Titin also functions to maintain the structural integrity of the contracting myofibrils within the muscle and to organize the machinery for condensation of chromosomes in dividing cells. Titin is a giant protein composed of 27,000 amino acids and contains an autoregulated serine kinase catalytic domain as well as a calcium/calmodulin binding region that are involved in its activation. Activated Titin phosphorylates the muscle protein telethonin, a sarcomeric protein abundant in heart and skeletal muscle, implicating Titin activity in the reorganization of the cytoskeleton during myofibrillogenesis.

Function:
Key component in the assembly and functioning of vertebrate striated muscles. By providing connections at the level of individual microfilaments, it contributes to the fine balance of forces between the two halves of the sarcomere. The size and extensibility of the cross-links are the main determinants of sarcomere extensibility properties of muscle. In non-muscle cells, seems to play a role in chromosome condensation and chromosome segregation during mitosis. Might link the lamina network to chromatin or nuclear actin, or both during interphase.

Subunit:
Interacts with MYOM1, MYOM2, tropomyosin and myosin. Interacts with actin, primarily via the PEVK domains and with MYPN (By similarity). Interacts with FHL2, NEB, CRYAB, LMNA/lamin-A and LMNB/lamin-B. Interacts with TCAP/telethonin and/or ANK1 isoform Mu17/ank1.5, via the first two N-terminal immunoglobulin domains. Interacts with TRIM63 and TRIM55, through several domains including immunoglobulin domains 141 and 142. Interacts with ANKRD1, ANKRD2 and ANKRD23, via the region between immunoglobulin domains 77 and 78 and interacts with CAPN3, via immunoglobulin domain 79. Interacts with NBR1 through the protein kinase domain. Interacts with CALM/calmodulin. Isoform 8 interacts with OBSCN isoform 3.

Subcellular Location:
Cytoplasm. Nucleus.

CMD1G,扩张型心肌病蛋白1G抗体Tissue Specificity:
Isoform 3, isoform 7 and isoform 8 are expressed in cardiac muscle. Isoform 4 is expressed in vertebrate skeletal muscle. Isoform 6 is expressed in cardiac tissues.

Post-translational modifications:
Autophosphorylated. Phosphorylated upon DNA damage, probably by ATM or ATR.

Similarity:
Belongs to the protein kinase superfamily. CAMK Ser/Thr protein kinase family.
Contains 132 fibronectin type-III domains.
Contains 152 Ig-like (immunoglobulin-like) domains.
Contains 19 Kelch repeats.
Contains 1 protein kinase domain.
Contains 17 RCC1 repeats.
Contains 14 TPR repeats.
Contains 15 WD repeats.

Database links:
Entrez Gene: 7273 Human
Entrez Gene: 396682 Pig
Entrez Gene: 84015 Rat
Omim: 188840 Human
SwissProt: Q8WZ42 Human
Unigene: 134602 Human

CMD1G,扩张型心肌病蛋白1G抗体Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

产品图片

Tissue/cell: rat heart tissue; 4% Paraformaldehyde-fixed and paraffin-embedded;  Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min;  Incubation: Anti-Titin/CMD1G Polyclonal Antibody, Unconjugated(HZ-9861R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining