Rabbit anti-Human DMPK Polyclonal Antibody | anti-DMPK antibody

DMPK (Dystrophy Myotonic Protein Kinase Protein)

Cat. Num. AAA610305

• Gene Names: DMPK; DM; DM1; DMK; MDPK; DM1PK; MT-PK
• Reactivity: Human
• Applications: ELISA, Western Blot
• Purity: Purified; Purified by epitope affinity chromatography.
• Synonyms: DMPK; Polyclonal Antibody; DMPK (Dystrophy Myotonic Protein Kinase Protein); Anti -DMPK (Dystrophy Myotonic Protein Kinase Protein); anti-DMPK antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Specificity: Specific for the ~80kD DMPK Protein. Reactivity is confirmed with mouse heart and skeletal muscle homogenates.
• Purity/Purification: Purified; Purified by epitope affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.4, 0.05% sodium azide.

• Applicable Applications for anti-DMPK antibody: ELISA (EL/EIA), Western Blot (WB)
• Application Notes: Suitable for use in ELISA and Western Blot.; Dilution: ELISA: 0.1-1ug/ml; Western Blot: 1-3ug/ml
• Immunogen: Synthetic peptide from the C-terminal region of the mouse Myotonic Dystrophy Protein kinase (DMPK) protein
• Preparation and Storage: May be stored at 4 degree C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and add glycerol (40-50%). Freeze at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Related Product Information for anti-DMPK antibody

Myotonic dystrophy (DM) is a highly variable multisystemic disease that leads to muscle wasting, myotonia, cataracts, heart block and neurobehavioral abnormalities. Myotonic dystrophy is caused by a polymorphic expansion of trinucleotide (CTG) repeats in the 3' untranslated region (UTR) of a c-AMP-dependent protein kinase (myotonic dystrophy protein kinase, DMPK) gene. Strong nucleosome positioning signals created by this expanded repeat cause a reduction in gene expression within the region. The molecular weight of DMPK is 80kD and it is expressed in the cytosolic fraction of skeletal muscles. DMPK is localized at the gap junction level in the heart, specifically in the intercalated disc. Heart transcripts of the DMPK (DMRB15) gene are subject to alternative splicing in both human and mouse

Product Categories/Family for anti-DMPK antibody

Antibodies; Abs to Protein Kinases

NCBI and Uniprot Product Information

• NCBI GI #: 71051932
• NCBI GeneID: 1760
• UniProt Accession #: Q09013; Q16205; Q6P5Z6; Q59FU6
• Molecular Weight: 70,371 Da
• NCBI Official Full Name: DMPK protein
• NCBI Official Synonym Full Names: dystrophia myotonica-protein kinase
• NCBI Official Symbol: DMPK
• NCBI Official Synonym Symbols: DM; DM1; DMK; MDPK; DM1PK; MT-PK
• NCBI Protein Information: myotonin-protein kinase; DM-kinase; DM protein kinase; thymopoietin homolog; myotonin protein kinase A; myotonic dystrophy protein kinase; myotonic dystrophy associated protein kinase
• UniProt Protein Name: Myotonin-protein kinase
• Protein Family: Myotonin-protein kinase
• UniProt Gene Name: DMPK
• UniProt Synonym Gene Names: MDPK
• UniProt Entry Name: DMPK_HUMAN

NCBI Description

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]

Uniprot Description

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: EC 2.7.11.1; Protein kinase, Ser/Thr (non-receptor); Protein kinase, AGC; Kinase, protein; Mitochondrial; Membrane protein, integral; 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: myosin phosphatase regulator activity; protein serine/threonine kinase activity; protein binding; metal ion binding; ATP binding

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

Disease: Myotonic Dystrophy 1