货号：MABS53

品牌：Merck Millipore

规格：EA

目录价：￥3108.00

市场价格：￥2641.80

会员价格：￥2486.40

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-DMPK Antibody, clone 9H9 | MABS53 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Molecular Weight: ~3-70 kDa observed. This protein has 14 isoforms which range from 3-70 kDa. View All» Immunogen: Human DMPK protein fragment containing catalytic and coil domains View All» Clone: 9H9 View All» Isotype: IgG1κ View All» Background Information: DMPK is also known as myotonic dystrophy protein kinase and a CTG extension in the 3’-ultratranslated region is show to result in myotonic dystrophy type 1 (DM1). DMPK seems to play a role in intracellular trafficking of insulin and IGF-1 receptors. DMPK is an 80 kDa protein that is mostly found in smooth, skeletal and cardiac muscle. View All» Species Reactivity: Human View All» Species Reactivity Note: Demonstrated to react with human. View All» Control: Human fetal skeletal muscle tissue lysate View All» Quality Assurance: Evaluated by Western Blot in human fetal skeletal muscle tissue lysate.Western Blot Analysis: 0.5 µg/mL of this antibody detected DMPK on 10 µg of human fetal skeletal muscle tissue lysate. View All» Purification Method: Protein G View All» Presentation: Purified mouse monoclonal IgG1κ in buffer containing 0.1 M Tris-Glycine (pH 7.4), 150 mM NaCl with 0.05% sodium azide. View All» Storage Conditions: Stable for 1 year at 2-8°C from date of receipt. View All» UniProt Number: Q09013 View All» Entrez Gene Number: NP_001075029 View All» Gene Symbol:

• DMK

• MDPK

• MT-PK

• DM

• DM1PK

• DM1

• DMPK
  View All» Alternate Names:
  • dystrophia myotonica-protein kinase

  • DM-kinase

  • DM protein kinase

  • myotonic dystrophy associated protein kinase

  • thymopoietin homolog

  • myotonin protein kinase A

  • myotonin-protein kinase

  • Myotonic dystrophy protein kinase
    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 View All» Entrez Gene Summary: The protein encoded by this gene is a serine-threonine kinase that is closely related to other kinases that interact with members of the Rho family of small GTPases. Substrates for this enzyme include myogenin, the beta-subunit of the L-type calcium channels, and phospholemman. The 3' untranslated region of this gene contains 5-37 copies of a CTG trinucleotide repeat. Expansion of this unstable motif to 50-5,000 copies causes myotonic dystrophy type I, which increases in severity with increasing repeat element copy number. Repeat expansion is associated with condensation of local chromatin structure that disrupts the expression of genes in this region. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined. [provided by RefSeq]. View All» UniProt Summary: FUNCTION: Critical to the modulation of cardiac contractility and to the maintenance of proper cardiac conduction activity. Phosphorylates phospholamban. Ref.11 Ref.13CATALYTIC ACTIVITY: ATP + a protein = ADP + a phosphoprotein.COFACTOR: Magnesium.ENZYME REGULATION: Activated in response to G protein second messengers. Maintained in an inactive conformation by the negative autoregulatory C-terminal coiled-coil region. Coiled-coil mediated oligomerization correlated with enhanced catalytic activity as is proteolytical cleavage near the C-terminus. SUBUNIT STRUCTURE: Homodimer. TISSUE SPECIFICTY: Most isoforms are expressed in many tissues including heart, skeletal muscle, liver and brain, except for isoform 2 which is only found in the heart and skeletal muscle, and isoform 14 which is only found in the brain, with high levels in the striatum, cerebellar cortex and pons.INVOLVEMENT IN DISEASE: Defects in DMPK are the cause of dystrophia myotonica type 1 (DM1) [MIM:160900]; also known as Steinert disease. A muscular disorder characterized by myotonia, muscle wasting in the distal extremities, cataract, hypogonadism, defective endocrine functions, male baldness and cardiac arrhythmias. Note=The causative mutation is a CTG expansion in the 3'-UTR of the DMPK gene. A length exceeding 50 CTG repeats is pathogenic, while normal individuals have 5 to 37 repeats. Intermediate alleles with 35-49 triplets are not disease-causing but show instability in intergenerational transmissions. Disease severity varies with the number of repeats: mildly affected persons have 50 to 150 repeats, patients with classic DM have 100 to 1,000 repeats, and those with congenital onset can have more than 2,000 repeats. SEQUENCE SIMILARITIES: Belongs to the protein kinase superfamily. AGC Ser/Thr protein kinase family. DMPK subfamily.Contains 1 AGC-kinase C-terminal domain.Contains 1 protein kinase domain.SEQUENCE CAUTION: Isoform 13: The sequence AAA64884.1 differs from that shown. Reason: Frameshift at position 15. View All» Product Name: Anti-DMPK, clone 9H9 View All» Concentration: 0.5 mg/mL View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 μg View All» Format: Purified View All» Host: Mouse View All»
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