Human skeletal muscle: immunohistochemical staining for Dystrophin. Note membrane staining of normal muscle fibers (A) and reduced and variable staining of revertant muscle fibers (B). Dystrophin: clone 13H6

Dystrophin Antibodies

dystrophin-antibodies

Antigen Background

Duchenne Muscular dystrophy (DMD) is the most common of the muscular dystrophies resulting in progressive muscular wasting and death. Dystrophin is the 427kD protein product of the DMD gene located on the X chromosome at position Xp21. Abnormalities in protein expression occur in patients with DMD/BMD and dystrophin analysis may be used to distinguish these conditions from other neuromuscular diseases. Severe Duchenne muscular dystrophy is associated with a marked dystrophin deficiency, whereas patients with the milder form of Becker muscular dystrophy show less pronounced abnormalities of protein expression. The immunolabeling patterns for DYS1, DYS2 and DYS3 are similar; however, the use of all three antibodies is recommended to avoid the possibility of occasional false negative results.

Dystrophin Antibodies are recommended for the detection of specific antigens of interest in normal and neoplastic tissues, as an adjunct to conventional histopathology using non-immunologic histochemical stains.

  • DYSA
    1ml NCL-DYSA
    13H6
    Lyophilised Concentrate
    P (HIER)
  • DYSB
    1ml NCL-DYSB
    34C5
    Lyophilised Concentrate
    P (HIER)
  • This item replaces DYS1DYS1-S
    DYS1-CE
    2.5ml NCL-DYS1
    DY4/6D3
    Lyophilised Concentrate
    F
  • This item replaces DYS2DYS2-CE-S
    DYS2-CE
    2.5ml NCL-DYS2
    DY8/6C5
    Lyophilised Concentrate
    F
  • This item replaces DYS3-SDYS3-CE-S
    DYS3-CE
    2.5ml NCL-DYS3
    DY10/12B2
    Lyophilised Concentrate
    F

Product Specifications

Product Specifications

DYSA
Muscle Pathology
13H6
Lyophilised Concentrate
No
P (HIER)
Mono
Mouse
Research Use Only
1ml
DYSB
Muscle Pathology
34C5
Lyophilised Concentrate
No
P (HIER)
Mono
Mouse
Research Use Only
1ml
DYS1-CE
Muscle Pathology
DY4/6D3
Lyophilised Concentrate
No
F
Mono
Mouse
In Vitro Diagnostic Use
1ml
DYS2-CE
Muscle Pathology
DY8/6C5
Lyophilised Concentrate
No
F
Mono
Mouse
In Vitro Diagnostic Use
1ml
DYS3-CE
Muscle Pathology
DY10/12B2
Lyophilised Concentrate
No
F
Mono
Mouse
In Vitro Diagnostic Use
1ml

Documents

Documents

DYSA
DYSB
DYS1-CE
DYS2-CE
DYS3-CE

Resources

Resources

Antigen Background

Duchenne Muscular dystrophy (DMD) is the most common of the muscular dystrophies resulting in progressive muscular wasting and death. Dystrophin is the 427kD protein product of the DMD gene located on the X chromosome at position Xp21. Abnormalities in protein expression occur in patients with DMD/BMD and dystrophin analysis may be used to distinguish these conditions from other neuromuscular diseases. Severe Duchenne muscular dystrophy is associated with a marked dystrophin deficiency, whereas patients with the milder form of Becker muscular dystrophy show less pronounced abnormalities of protein expression. The immunolabeling patterns for DYS1, DYS2 and DYS3 are similar; however, the use of all three antibodies is recommended to avoid the possibility of occasional false negative results.

Dystrophin Antibodies are recommended for the detection of specific antigens of interest in normal and neoplastic tissues, as an adjunct to conventional histopathology using non-immunologic histochemical stains.

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