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Key Specifications Table
| Species Reactivity | Key Applications | Host | Format | Antibody Type |
|---|---|---|---|---|
| H | WB, IHC | M | Culture Supernatant | Monoclonal Antibody |
| Description | |
|---|---|
| Catalogue Number | MAB1553 |
| Brand Family | Chemicon® |
| Trade Name |
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| Description | Anti-Titin Antibody, clone 9B9 |
| Product Information | |
|---|---|
| Format | Culture Supernatant |
| Presentation | Liquid. Contains 0.1% sodium azide. |
| Quality Level | MQ100 |
| Applications | |
|---|---|
| Application | This Anti-Titin Antibody, clone 9B9 is validated for use in WB, IH for the detection of Titin. |
| Key Applications |
|
| Application Notes | Immunochemistry: undiluted to 1:10 Western blotting: 1:10-1:20 Optimal dilutions must be determined by the end user. |
| Biological Information | |
|---|---|
| Immunogen | Electrophoretically purified human skeletal muscle titin. |
| Clone | 9B9 |
| Host | Mouse |
| Specificity | Human skeletal muscle titin. |
| Isotype | IgG1 |
| Species Reactivity |
|
| Antibody Type | Monoclonal Antibody |
| Entrez Gene Number |
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| 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. |
| Gene Symbol |
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| UniProt Number |
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| 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. |
| Product Usage Statements | |
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| Usage Statement |
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| Storage and Shipping Information | |
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| Storage Conditions | Maintain frozen at -20°C in undiluted aliquots for up to 12 months. Avoid repeated freeze/thaw cycles. |
| Packaging Information | |
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| Material Size | 1 mL |