DMPK, Polyclonal Antibody

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

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

Polyclonal

Rabbit Ig

Rabbit

This DMPK antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 11-39 amino acids from the N-terminal region of human DMPK.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-DMPK antibody 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 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.

Neuroscience; Signal Transduction

Western Blot (WB), ELISA (EIA)

WB~~1:1000

DMPK Antibody (N-term) western blot analysis in Jurkat cell line lysates (35ug/lane).This demonstrates the DMPK antibody detected the DMPK protein (arrow).

69385

dystrophia myotonica-protein kinase

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

Chromosomal Location of Human Ortholog: 19q13.3

Cellular Component: nuclear outer membrane; endoplasmic reticulum membrane; nuclear membrane; sarcoplasmic reticulum 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

Theerasasawat, S., et al. J Clin Neurosci 17(12):1520-1522(2010)
Santoro, M., et al. Exp. Mol. Pathol. 89(2):158-168(2010)
Bailey, S.D., et al. Diabetes Care 33(10):2250-2253(2010)
Vignaud, A., et al. Neuromuscul. Disord. 20(5):319-325(2010)
Kwon, M.J., et al. Ann. Clin. Lab. Sci. 40(2):156-162(2010)

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