Anti-Dystrophin Antibody

ヒトジストロフィン分子のC末端ドメイン(アミノ酸残基3558-3684)は、正常筋組織に存在しています。また、ベッカ-型筋ジストロフィ-のほぼすべてに存在していますが、デュシェンヌ型筋ジストロフィ-の症例やジストロフィ-マウス(mdx)には存在しません。

Dystrophin is a muscle membrane protein (427 kDa) which is absent, reduced or altered as a result of mutation in Duchenne and Becker muscular dystrophies (DMD/BMD) or its homologue in the mouse. Severe DMD is associated with a marked dystrophin deficiency whereas patients with the milder form of DMD show less pronounced abnormalities of protein expression. Because abnormalities in the protein expression occur specifically in patients with these types of muscular dystrophy, dystrophin analysis may be used to distinguish these conditions from other neuromuscular diseases. Predictions from the sequence suggest a structural protein on the inner face of the membrane, consisting of a 25-repeat, rod-like triple-helical domain separating an N-terminal actin-binding domain from two C-terminal domains, one of which is rich in cysteine. The large size of dystrophin and its low abundance (<0.01% of the total muscle protein) are a hindrance to the isolation of intact, native protein for structure/function studies.

The antibody recognizes an epitope located on the 128 amino acids at the end of the C-terminal domain of the human dystrophin molecule (amino acid residues 3558-3684). Immunohistochemical staining of muscle tissue results in a clear labeling confined to the periphery (plasma membrane) of normal striated muscle fibers. By immunoblotting, the antibody stains dystrophin (427 kDa) in muscle and brain extracts. It also stains the 70-75 kDa protein known as Apo-Dystrophin-1 or Dp71.4 This is detected in the brain as well as in lymphoblastoid cells, cultures of brain astroglial and neuronal cells, liver and Hep G2 cells (human hepatoma). The epitope recognized by the antibody is sensitive to formalin fixation and paraffin embedding. The antibody exhibits a wide interspecies cross-reactivity. It is useful in ELISA and capture ELISA. The antibody is specific to dystrophin and does not react with α-actinin and utrophin, an autosomal homologue of dystrophin, also called dystrophin-related protein (DRP).

fusion protein containing the C-terminal amino acids of human dystrophine.

Applications in which this antibody has been used successfully, and the associated peer-reviewed papers, are given below.
Immunofluorescence (1 paper)
Western Blotting (1 paper)

Mouse monoclonal clone MANDRA1 anti-Dystrophin antibody may be used for the localization of dystrophin using various immunochemical assays such as ELISA, capture ELISA, immunoblot, and immunohistochemistry. Monoclonal antibodies against defined regions of dystrophin provide a means for studying its structure and function, interactions with other proteins and the nature of the partial gene products produced in some patients carrying deletions in the dystrophin gene. The antibodies are useful in the prenatal or post-abortion diagnosis of muscular dystrophy carriers by immunohistological analyses.

本抗体は、ヒトジストロフィン分子のC末端ドメイン末端部の128アミノ酸(アミノ酸残基3558-3684)に位置するエピト-プを認識します。筋組織の免疫組織化学染色では、正常横紋筋線維の表面(形質膜)に限局して明瞭な標識が認められています。イムノブロッティングにおいて、抗体により筋および脳抽出物のジストロフィン(427 kDa)が染色されています。また、アポジストロフィン-1あるいはDp71.4として知られている70-75 kDaのタンパク質も染色されます。これは、脳をはじめリンパ芽球様細胞、脳のアストログリアおよび神経細胞培養物、肝臓およびHep G2細胞(ヒト肝細胞腫)で検出されています。抗体により認識されるエピト-プは、ホルマリン固定、パラフィン包埋には不安定です。抗体は、広範な種間交差反応性を示します。ELISAおよび捕獲ELISAに使用できます。抗体はジストロフィンに特異的であり、α-アクチニンおよびユ-トロフィン(ジストロフィン関連タンパク質(DRP)とも呼ばれる常染色体性のジストロフィン相同体)とは反応しません。

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