DMPK, Blocking Peptide

DM1PK; MDPK; Myotonin-protein kinase; MT-PK; DM-kinase; DMK; DM1 protein kinase; DMPK; Myotonic dystrophy protein kinase

For Research Use Only. Not for use in diagnostic procedures.

Synthetic

>85%

Lyophilized powder

Shipped at 4 degree C. Store at -20 degree C for one year.

Small volumes of DMPK blocking peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

The peptide is used to block Anti-DMPK Antibody reactivity.

Blocking (BL)

Blocking Peptide to the diluted primary antibody in a molar ratio of 10:1 (peptide to antibody) and incubate the mixture at 4 degree C for overnight or at room temperature for 2 hours.

60,790 Da

dystrophia myotonica-protein kinase

myotonin-protein kinase; DM protein kinase; DM1 protein kinase; thymopoietin homolog; myotonin protein kinase A; myotonic dystrophy associated protein kinase

Myotonin-protein kinase

DM1PK; MDPK; MT-PK; DMK  [Similar Products]

DMPK_HUMAN

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, Jul 2008]

DMPK1: Non-receptor serine/threonine protein kinase which is necessary for the maintenance of skeletal muscle structure and function. May play a role in myocyte differentiation and survival by regulating the integrity of the nuclear envelope and the expression of muscle-specific genes. May also phosphorylate PPP1R12A and inhibit the myosin phosphatase activity to regulate myosin phosphorylation. Also critical to the modulation of cardiac contractility and to the maintenance of proper cardiac conduction activity probably through the regulation of cellular calcium homeostasis. Phosphorylates PLN, a regulator of calcium pumps and may regulate sarcoplasmic reticulum calcium uptake in myocytes. May also phosphorylate FXYD1/PLM which is able to induce chloride currents. May also play a role in synaptic plasticity. Defects in DMPK are the cause of dystrophia myotonica type 1 (DM1); 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. 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. Belongs to the protein kinase superfamily. AGC Ser/Thr protein kinase family. DMPK subfamily. 12 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Protein kinase, Ser/Thr (non-receptor); Kinase, protein; Mitochondrial; Protein kinase, AGC; EC 2.7.11.1; AGC group; DMPK family; GEK subfamily

Chromosomal Location of Human Ortholog: 19q13.3

Cellular Component: nuclear outer membrane; endoplasmic reticulum membrane; sarcoplasmic reticulum membrane; nuclear membrane; plasma membrane; cytosol; integral to mitochondrial outer membrane

Molecular Function: protein serine/threonine kinase activity; myosin phosphatase regulator activity; protein binding; metal ion binding; ATP binding

Biological Process: cellular calcium ion homeostasis; regulation of catalytic activity; protein amino acid phosphorylation; regulation of heart contraction; nuclear membrane organization and biogenesis

Disease: Myotonic Dystrophy 1

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.