Product Name	DMPK
Chinese Name	肌强直性营养不良蛋白激酶抗体
Alias	Dystrophia myotonica protein kinase; DM 1; DM; DM kinase; DM protein kinase; DM-kinase; DM1; DM1 protein kinase; DM1PK; DMK; DMPK; DMPK_HUMAN; Dystrophia myotonica 1; Dystrophia myotonica protein kinase; MDPK; MT PK; MT-PK; Myotonic dystrophy associated protein kinase; Myotonic dystrophy protein kinase; Myotonin protein kinase A; Myotonin protein kinase; Myotonin-protein kinase; Thymopoietin homolog.
Research Area	Cardiovascular  Neurobiology  Signal transduction  Kinases and Phosphatases
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Mouse,  (predicted: Human, Rat, Dog, Pig, Cow, Sheep, )
Applications	ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	69kDa
Cellular localization	The nucleus cytoplasmic The cell membrane
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human DMPK: 51-120/629
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	Myotonic dystrophy protein kinase is a multi-domain protein kinase found in muscle that is activated in response to G protein second messengers and proteolysis (1). DMPK is implicated in myotonic muscular dystrophy (DM), an autosomal dominant-inherited disorder that predominately affects skeletal and cardiac muscle and causes defects in cardiac conduction (2,3). DM arises through expansion of CTG repeats in the 3’-UTR of the DMPK gene (4). Mutant DMPK transcripts with an extended region of CUG repeats are retained in the nucleus (5). These transcripts also influence the expression of the DM locus-associated homeodomain protein (DMAHP)/SIX5, to mediate in part the DM phenotype (6). Other substrates for DMPK include myogenin, L-type calcium channels, and Phospholemman (PLM) (1). Function: Non-receptor serine/threonine protein kinase which isnecessary for the maintenance of skeletal muscle structure andfunction. May play a role in myocyte differentiation and survivalby regulating the integrity of the nuclear envelope and theexpression of muscle-specific genes. May also phosphorylatePPP1R12A and inhibit the myosin phosphatase activity to regulatemyosin phosphorylation. Also critical to the modulation of cardiaccontractility and to the maintenance of proper cardiac conductionactivity probably through the regulation of cellular calciumhomeostasis. Phosphorylates PLN, a regulator of calcium pumps andmay regulate sarcoplasmic reticulum calcium uptake in myocytes. Mayalso phosphorylate FXYD1/PLM which is able to induce chloridecurrents. May also play a role in synaptic plasticity. Subunit: Homodimer; homodimerization stimulates the kinaseactivity. Interacts with HSPB2; may enhance DMPK kinase activity.Interacts with PLN; phosphorylates PLN. May interact with RAC1; mayregulate DMPK kinase activity. Interacts with LMNA; may regulatenuclear envelope stability. Subcellular Location: Endoplasmic reticulum membrane; Single-passtype IV membrane protein; Cytoplasmic side (By similarity). Nucleusouter membrane; Single-pass type IV membrane protein; Cytoplasmicside (Probable). Mitochondrion outer membrane; Single-pass type IVmembrane protein (Probable). Sarcoplasmic reticulum membrane (Bysimilarity). Cell membrane (By similarity). Cytoplasm, cytosol (Bysimilarity). Note=Localizes to sarcoplasmic reticulum membranes ofcardiomyocytes (By similarity). Isoform 1: Mitochondrion membrane. Isoform 3: Mitochondrion membrane. Tissue Specificity: 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. Post-translational modifications: Phosphorylated. Autophosphorylates. Phosphorylation by RAF1may result in activation of DMPK. Proteolytic processing of the C-terminus may remove thetransmembrane domain and release the kinase from membranesstimulating its activity. 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. Similarity: 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. SWISS: Q09013 Gene ID: 1760 Database links: Entrez Gene: 1760 Human Entrez Gene: 13400 Mouse Entrez Gene: 308405 Rat Omim: 605377 Human SwissProt: Q09013 Human SwissProt: P54265 Mouse Unigene: 631596 Human Unigene: 6529 Mouse Unigene: 130535 Rat
Product Picture	Paraformaldehyde-fixed, paraffin embedded (Mouse cerebellum); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (DMPK) Polyclonal Antibody, Unconjugated (SL11742R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.

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