Rabbit DMPK Polyclonal Antibody | anti-DMPK antibody

DMPK (Myotonin-protein Kinase, Myotonic Dystrophy Protein Kinase, MDPK, DM-Kinase, DMK, MT-PK, DM-kinase) APC

Cat. Num. AAA6376360

• Gene Names: DMPK; DM; DM1; DMK; MDPK; DM1PK; MT-PK
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: DMPK; Polyclonal Antibody; DMPK (Myotonin-protein Kinase; Myotonic Dystrophy Protein Kinase; MDPK; DM-Kinase; DMK; MT-PK; DM-kinase) APC; anti-DMPK antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human DMPK. Species Crossreactivity: mouse and rat.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Allophycocyanin (APC).
• Sequence Length: 629

• Applicable Applications for anti-DMPK antibody: Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Full length human DMPK, aa1-629 (NP_004400.4).
• Immunogen Sequence: MSAEVRLRRLQQLVLDPGFLGLEPLLDLLLGVHQELGASELAQDKYVADFLQWAEPIVVRLKEVRLQRDDFEILKVIGRGAFSEVAVVKMKQTGQVYAMKIMNKWDMLKRGEVSCFREERDVLVNGDRRWITQLHFAFQDENYLYLVMEYYVGGDLLTLLSKFGERIPAEMARFYLAEIVMAIDSVHRLGYVHRDIKPDNILLDRCGHIRLADFGSCLKLRADGTVRSLVAVGTPDYLSPEILQAVGGGPGTGSYGPECDWWALGVFAYEMFYGQTPFYADSTAETYGKIVHYKEHLSLPLVDEGVPEEARDFIQRLLCPPETRLGRGGAGDFRTHPFFFGLDWDGLRDSVPPFTPDFEGATDTCNFDLVEDGLTAMVSGGGETLSDIREGAPLGVHLPFVGYSYSCMALRDSEVPGPTPMELEAEQLLEPHVQAPSLEPSVSPQDETAEVAVPAAVPAAEAEAEVTLRELQEALEEEVLTRQSLSREMEAIRTDNQNFASQLREAEARNRDLEAHVRQLQERMELLQAEGATAVTGVPSPRATDPPSHLDGPPAVAVGQCPLVGPGPMHRRHLLLPARVPRPGLSEALSLLLFAVVLSRAAALGCIGLVAHAGQLTAVWRRPGAARAP
• Conjugate: APC
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: APC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(DMPK rabbit polyclonal antibody. Western Blot analysis of DMPK expression in HeLa.)

Western Blot (WB)

(DMPK rabbit polyclonal antibody. Western Blot analysis of DMPK expression in PC-12.)

Western Blot (WB)

(DMPK rabbit polyclonal antibody. Western Blot analysis of DMPK expression in NIH/3T3.)

Western Blot (WB)

(Western Blot analysis of DMPK expression in transfected 293T cell line by DMPK polyclonal antibody. Lane 1: DMPK transfected lysate (69.4kD). Lane 2: Non-transfected lysate.)

Related Product Information for anti-DMPK antibody

DMPK, a member of the Ser/Thr protein kinase family, may play a role in intracellular communication. Most DMPK 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. The poly-Gln region upstream/downstream of the gene is highly polymorphic (5 to 27 repeats) in the normal population and is expanded up to 50-3000 or more repeats in DM patients. The repeat length usually increases in successive generations, but not always. Defects in DMPK are the cause of myotonic dystrophy (DM), also known as Steinert disease. DM is an autosomal dominant neurodegenerative disorder characterized by myotonia, muscle wasting in the distal extremities, cataract, hypogonadism, defective endocrine functions, male baldness, and cardiac arrhythmias. DM patients show decreased levels of kinase expression inversely related to repeat length. The minimum estimated incidence is 1 in 8000 live births.

Product Categories/Family for anti-DMPK antibody

Antibodies; Protein Kinases

NCBI and Uniprot Product Information

• NCBI GI #: 47059024
• NCBI GeneID: 1760
• NCBI Accession #: NP_004400.4
• NCBI GenBank Nucleotide #: NM_004409.2
• UniProt Accession #: Q09013
• NCBI Official Full Name: myotonin-protein kinase isoform 2
• NCBI Official Synonym Full Names: DM1 protein kinase
• NCBI Official Symbol: DMPK
• NCBI Official Synonym Symbols: DM; DM1; DMK; MDPK; DM1PK; MT-PK
• NCBI Protein Information: myotonin-protein kinase
• UniProt Protein Name: Myotonin-protein kinase
• Protein Family: Myotonin-protein kinase
• UniProt Gene Name: DMPK
• UniProt Synonym Gene Names: DM1PK; MDPK; MT-PK; DMK
• 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 5-38 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 2016]

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: Membrane protein, integral; EC 2.7.11.1; Protein kinase, Ser/Thr (non-receptor); Mitochondrial; Protein kinase, AGC; Kinase, protein; 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; nuclear membrane organization and biogenesis; regulation of heart contraction

Disease: Myotonic Dystrophy 1