Dystrophin (DMD) Recombinant Protein | DMD recombinant protein

Recombinant Dystrophin (DMD)

Cat. Num. AAA2010814

• Gene Names: Dmd; dys; mdx; pke; Dp71; Dp427; DXSmh7; DXSmh9
• Applications: SDS-Page, Western Blot, ELISA, Immunoprecipitation
• Purity: > 95%
• Synonyms: Dystrophin (DMD); Recombinant Dystrophin (DMD); DMD recombinant protein

• Host: E Coli
• Purity/Purification: > 95%
• Form/Format: Supplied as lyophilized form in PBS,pH7.4, containing 5% sucrose, 0.01% sarcosyl.
• Sequence: The target protein is fused with N-terminal His-Tag, its sequence is listed below.; MGHHHHHHSGSEF- SP NKVPYYINHE TQTTCWDHPK MTELYQSLAD LNNVRFSAYR TAMKLRRLQK ALCLDLLSLS AACDALDQHN LKQNDQPMDI LQIINCLTTI YDRLEQEHNN LVNVPLCVDM CLNWLLNVYD TGRTGRIRVL SFKTGIISLC KAHLEDKYRY LFKQVASSTG FCDQRRLGLL LHDSIQIPRQ LGEVASFGGS NIEPSVRSCF QFANNKPEIE AALFLDWMRL EPQSMVWLPV LHRVAAAETA KHQAKCNICK ECPI
• Sequence Length: 3678

• Applicable Applications for DMD recombinant protein: SDS-PAGE, Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)
• Organism: Mus musculus (Mouse)
• Expression System: Prokaryotic expression
• Residues: Ser3059~Ile3314 (Accession # P11531) with N-terminal His-Tag
• Endotoxin Level: <1.0EU per 1ug (determined by the LAL method)
• Reconstitution: Reconstitute in sterile PBS, pH7.2-pH7.4.
• Preparation and Storage: Avoid repeated freeze/thaw cycles. Store at 2-8 degree C for one month. Aliquot and store at -80 degree C for 12 months.; Stability Test: The thermal stability is described by the loss rate of the targetprotein. The loss rate was determined by accelerated thermal degradation test,that is, incubate the protein at 37 degree C for 48h, and no obvious degradation andprecipitation were observed. (Referring from China Biological Products Standard,which was calculated by the Arrhenius equation.) The loss of this protein is lessthan 5% within the expiration date under appropriate storage condition.

SDS-Page

NCBI and Uniprot Product Information

• NCBI GI #: 6681203
• NCBI GeneID: 13405
• NCBI Accession #: NP_031894.1
• NCBI GenBank Nucleotide #: NM_007868.5
• UniProt Accession #: P11531; O35653; Q60703; A2A9Z0
• Molecular Weight: 30.8kDa
• NCBI Official Full Name: dystrophin
• NCBI Official Synonym Full Names: dystrophin, muscular dystrophy
• NCBI Official Symbol: Dmd
• NCBI Official Synonym Symbols: dys; mdx; pke; Dp71; Dp427; DXSmh7; DXSmh9
• NCBI Protein Information: dystrophin; X-linked muscular dystrophy
• UniProt Protein Name: Dystrophin
• Protein Family: Dystrophin
• UniProt Gene Name: Dmd
• UniProt Entry Name: DMD_MOUSE

Uniprot Description

dystrophin: Anchors the extracellular matrix to the cytoskeleton via F-actin. Ligand for dystroglycan. Component of the dystrophin- associated glycoprotein complex which accumulates at the neuromuscular junction (NMJ) and at a variety of synapses in the peripheral and central nervous systems and has a structural function in stabilizing the sarcolemma. Also implicated in signaling events and synaptic transmission. Defects in DMD are the cause of Duchenne muscular dystrophy (DMD). DMD is the most common form of muscular dystrophy; a sex-linked recessive disorder. It typically presents in boys aged 3 to 7 year as proximal muscle weakness causing waddling gait, toe-walking, lordosis, frequent falls, and difficulty in standing up and climbing up stairs. The pelvic girdle is affected first, then the shoulder girdle. Progression is steady and most patients are confined to a wheelchair by age of 10 or 12. Flexion contractures and scoliosis ultimately occur. About 50% of patients have a lower IQ than their genetic expectations would suggest. There is no treatment. Defects in DMD are the cause of Becker muscular dystrophy (BMD). BMD resembles DMD in hereditary and clinical features but is later in onset and more benign. Defects in DMD are a cause of cardiomyopathy dilated X- linked type 3B (CMD3B); also known as X-linked dilated cardiomyopathy (XLCM). Dilated cardiomyopathy is a disorder characterized by ventricular dilation and impaired systolic function, resulting in congestive heart failure and arrhythmia. Patients are at risk of premature death. 6 isoforms of the human protein are produced by alternative splicing.

Protein type: Cytoskeletal; Motility/polarity/chemotaxis

Cellular Component: filopodium membrane; cell surface; costamere; protein complex; cell; Z disc; cell-matrix junction; lipid raft; dystrophin-associated glycoprotein complex; postsynaptic membrane; cytoskeleton; membrane; cytoplasm; plasma membrane; synapse; intracellular; cell junction; nucleus; sarcolemma; filopodium

Molecular Function: protein binding; myosin binding; structural constituent of cytoskeleton; zinc ion binding; structural constituent of muscle; metal ion binding; actin binding; nitric-oxide synthase binding; vinculin binding

Biological Process: regulation of skeletal muscle contraction via regulation of the release of sequestered calcium ion; muscle development; skeletal muscle development; establishment of blood-nerve barrier; regulation of heart rate; neurotransmitter receptor metabolic process; olfactory nerve structural organization; negative regulation of peptidyl-serine phosphorylation; myotube cell development; muscle attachment; nucleus localization; peptide biosynthetic process; muscle maintenance; regulation of membrane potential; cellular protein complex assembly; regulation of transcription, DNA-dependent; regulation of gene expression; regulation of skeletal muscle contraction; positive regulation of neuron differentiation; muscle fiber development; positive regulation of cell-matrix adhesion; cardiac muscle contraction