NeoBiotechnologies

Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) Monoclonal Antibody (DMD/3676)

Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) Antibody in Immunohistochemistry (Paraffin) (IHC (P))

图: 1 / 4

Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) Antibody (1756-MSM6-P1) in IHC (P)

Formalin-fixed, paraffin-embedded human skeletal muscle stained with Dystrophin Monospecific Mouse Monoclonal Antibody (DMD/3676).

Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) Antibody in Peptide array (ARRAY)

Dystrophin (DMD) (Marker of Duchenne and Becker Muscular Dystrophy) Antibody in Immunoelectrophoresis (IE)

Product Image Gallery

产品信息

1756-MSM6-P1

应用

建议稀释比

已发表文章

免疫组化（石蜡） (IHC (P))

1-2 µg/mL

酶联免疫吸附实验 (ELISA)

Assay-dependent

肽阵列 (Array)

Assay-dependent

免疫电泳 (IE)

Assay-dependent

产品规格

种属反应

Human

宿主/亚型

Mouse / IgG2b, kappa

分类

Monoclonal

类型

Antibody

克隆号

DMD/3676

抗原

A recombinant fragment (around aa 114-263) of human DMD protein.

查看免疫原

偶联物

Unconjugated Unconjugated Unconjugated

形式

Liquid

浓度

200 µg/mL

纯化类型

Protein A/G

保存液

PBS with 0.05% BSA

内含物

0.05% sodium azide

保存条件

4° C

运输条件

Ambient (domestic); Wet ice (international)

产品详细信息

Antibody is stable for 24 months.

Positive Control: Human skeletal muscle and heart muscle tissues (IHC). Cellular Localization: Cell surface. Cytoplasm.

Specificity Comments: Dystrophin-glycoprotein complex (DGC) connects the F-Actin cytoskeleton on the inner surface of muscle fibers to the surrounding extracellular matrix, through the cell membrane interface. A deficiency in this protein contributes to Duchenne (DMD) and Becker (BMD) muscular dystrophies. The human dystrophin gene measures 2.4 megabases, has more than 80 exons, produces a 14 kb mRNA and contains at least 8 independent tissue-specific promoters and 2 poly A sites. The dystrophin mRNA can undergo differential splicing and produce a range of transcripts that encode a large set of proteins. Dystrophin represents approximately 0.002% of total striated muscle protein and localizes to triadic junctions in skeletal muscle, where it is thought to influence calcium ion homeostasis and force transmission.

靶标信息

Dystrophin is the 427kDa protein product of the DMB/BMD gene located on the X chromosome at position Xp21. Western blotting and immunohistochemistry are the two established methods for the detection of abnormalities of dystrophin expression in muscle biopsies. Dystrophin abnormalities are thought to occur in 100% of patients with DMD/BMD, although genetic abnormalities may only be detected in up to 65% of cases.

仅用于科研。不用于诊断过程。未经明确授权不得转售。