货号：MAB1553

品牌：Merck Millipore

规格：1mL

目录价：￥5207.00

市场价格：￥4425.95

会员价格：￥4165.60

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Titin Antibody, clone 9B9 | MAB1553 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: Human skeletal muscle titin. View All» Immunogen: Electrophoretically purified human skeletal muscle titin. View All» Clone: 9B9 View All» Isotype: IgG1 View All» Species Reactivity: Human View All» Application Notes: Immunochemistry: undiluted to 1:10 Western blotting: 1:10-1:20 Optimal dilutions must be determined by the end user. View All» Presentation: Liquid. Contains 0.1% sodium azide. View All» Storage Conditions: Maintain frozen at -20°C in undiluted aliquots for up to 12 months. Avoid repeated freeze/thaw cycles. View All» UniProt Number: Q8WZ42 View All» Entrez Gene Number:

• NM_003319.3

• NM_133378.3

• NM_133379.2

• NM_133432.2

• NM_133437.2
  View All» Gene Symbol:
  • TTN

  • HMERF

  • connectin

  • LGMD2J

  • MPRM

  • CMPD4

  • Connectin

  • FLJ39564

  • CMD1G

  • TMD

  • FLJ43066

  • CMH9

  • FLJ26409

  • Titin

  • FLJ26020

  • titin

  • DKFZp451N061

  • FLJ32040

  • FLJ34413

  • EC 2.7.11.1
    View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications:
    • Western Blotting

    • Immunohistochemistry
      View All» Entrez Gene Summary: This gene encodes a large abundant protein of striated muscle. The product of this gene is divided into two regions, a N-terminal I-band and a C-terminal A-band. The I-band, which is the elastic part of the molecule, contains two regions of tandem immunoglobulin domains on either side of a PEVK region that is rich in proline, glutamate, valine and lysine. The A-band, which is thought to act as a protein-ruler, contains a mixture of immunoglobulin and fibronectin repeats, and possesses kinase activity. A N-terminal Z-disc region and a C-terminal M-line region bind to the Z-line and M-line of the sarcomere respectively so that a single titin molecule spans half the length of a sarcomere. Titin also contains binding sites for muscle associated proteins so it serves as an adhesion template for the assembly of contractile machinery in muscle cells. It has also been identified as a structural protein for chromosomes. Considerable variability exists in the I-band, the M-line and the Z-disc regions of titin. Variability in the I-band region contributes to the differences in elasticity of different titin isoforms and, therefore, to the differences in elasticity of different muscle types. Of the many titin variants identified, five for which complete transcript information is available are described. Mutations in this gene are associated with familial hypertrophic cardiomyopathy 9 and autoantibodies to titin are produced in patients with the autoimmune disease scleroderma. View All» UniProt Summary: FUNCTION: SwissProt: Q8WZ42 # 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.COFACTOR: Magnesium.SIZE: 34350 amino acids; 3816188 Da 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 (Probable). Nucleus.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.DOMAIN: SwissProt: Q8WZ42 ZIS1 and ZIS5 regions contain multiple SPXR consensus sites for ERK- and CDK-like protein kinases as well as multiple SP motifs. ZIS1 could adopt a closed conformation which would block the TCAP-binding site. & The PEVK region may serve as an entropic spring of a chain of structural folds and may also be an interaction site to other myofilament proteins to form interfilament connectivity in the sarcomere.PTM: Autophosphorylated (By similarity). Phosphorylated upon DNA damage, probably by ATM or ATR.DISEASE: SwissProt: Q8WZ42 # Defects in TTN are the cause of hereditary myopathy with early respiratory failure (HMERF) [MIM:603689]; also known as Edstrom myopathy. HMERF is an autosomal dominant, adult-onset myopathy with early respiratory muscle involvement. & Defects in TTN are the cause of familial hypertrophic cardiomyopathy type 9 (CMH9) [MIM:188840]; also designated HCM or FHC. Familial hypertrophic cardiomyopathy [MIM:192600] is a heart disorder characterized by ventricular hypertrophy, which is usually asymmetric and often involves the interventricular septum. The prevalence of the disease in the general population is 0.2%. FHC is clinically heterogeneous, with inter- and intrafamilial variations ranging from benign to malignant forms with high risk of cardiac failure and sudden cardiac death. & Defects in TTN are the cause of dilated cardiomyopathy 1G (CMD1G) [MIM:604145]. Dilated cardiomyopathy [MIM:115200] is a disorder characterized by cardiac dilation and reduced systolic function. CMD1G inheritance is autosomal dominant. & Defects in TTN are the cause of tardive tibial muscular dystrophy (TMD) [MIM:600334]; also known as Udd myopathy. TMD is an autosomal dominant, late-onset distal myopathy. Muscle weakness and atrophy are usually confined to the anterior compartment of the lower leg, in particular the tibialis anterior muscle. Clinical symptoms usually occur at age 35-45 years or much later. & Defects in TTN are the cause of limb-girdle muscular dystrophy type 2J (LGMD2J) [MIM:608807]. Type 2 limb-girdle muscular dystrophies (LGMD2) are a heterogeneous group of diseases with high clinical variability and autosomal recessive inheritance. They are characterized by progressive weakness of the pelvic and shoulder girdle muscles and elevated serum creatine kinase. The severity of the disease depends on age at onset which may vary from early to late childhood or even adulthood. Homozygous mutations in the titin gene cause LGMD2J, while heterozygous mutations cause tardive tibial muscular dystrophy.SIMILARITY: 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.MISCELLANEOUS: In some isoforms, after the PEVK repeat region there is a long PEVK duplicated region. On account of this region, it has been very difficult to sequence the whole protein. The length of this region (ranging from 183 to 2174 residues), may be a key elastic element of titin. View All» Brand Family: Chemicon View All» Product Name: Anti-Titin Antibody, clone 9B9 | MAB1553 View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 1 mL View All» Format: Culture Supernatant View All» Host: Mouse View All»
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