Labelling Review row-Online

Primary Antibodies
Dysferlin
Clone Ham1/7B6
1 mL, 0.1 mL lyophilized NCL-Hamlet FPW
Clone Ham3/17B2
1 mL, 0.1 mL lyophilized NCL-Hamlet-2 F P (HIER) W
Dysferlin is the protein product of the 2p13 gene that is defective in
individuals with Limb-Girdle Muscular Dystrophy type 2B (LGMD2B) and
Miyoshi Myopathy (MM). Dysferlin is normally localized to the muscle
plasma membrane. In individuals with LGMD2B and MM, immunoreactivity
to dysferlin is reported to be severely reduced or lost, depending on the type
of mutation. Individuals with other neuromuscular conditions demonstrate
normal labeling patterns.
Product Specific Information
NCL-Hamlet may require heat induced epitope retrieval in some cases.
labeling with an antibody to beta-spectrin, to monitor membrane integrity, is
an essential immunohistochemical control.
Dystrophin Antibodies
Clone Dy4/6D3
2.5 mL, 1 mL lyophilized Dystrophin (Rod Domain)
NCL-DYS1 FWE
Clone Dy8/6C5
2.5 mL, 1 mL lyophilized Dystrophin (C-terminus)
NCL-DYS2 FWE
Clone Dy10/12B2
2.5 mL, 1 mL lyophilized Dystrophin (N-terminus)
NCL-DYS3 FWE
Clone 13H6
1 mL lyophilized Dystrophin (C-terminus)
NCL-DYSA P (HIER)
Clone 34C5
1 mL lyophilized Dystrophin (N-terminus)
NCL-DYSB P (HIER)
Duchenne Muscular dystrophy (DMD) is the most severe of the muscular
dystrophies resulting in progressive muscular wasting and death.
Dystrophin is the 427 kD protein product of the DMD/BMD gene located on
the X chromosome at position Xp2. Western blotting and immunohistochemistry
are the two established methods for the detection of
abnormalities of dystrophin expression in muscle samples.
Product Specific Information
NCL-DYS1, NCL-DYS2 and NCL-DYS3 map within amino acids 1181-1388,
3669-3685 and 321-494, respectively, on the dystrophin molecule. The
immunolabeling patterns for NCL-DYS1, NCL-DYS2 and NCL-DYS3 are similar.
NCL-DYSA is raised to a region of the dystrophin molecule, upstream from the
C-terminal region and NCL-DYSB is raised to a region of the N-terminus of the
dystrophin molecule. NCL-DYSA and NCL-DYSB will be of particular interest
in the investigation of archived formalin-fixed, paraffin-embedded material.
Labeling with an antibody to beta-spectrin, to monitor membrane integrity, is
an essential immunohistochemical control.
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Human skeletal muscle: immunohistochemical staining for dystrophin using NCL-DYSA. Note
membrane staining of normal muscle fibers (A) and reduced and variable staining of muscle
fibers in an individual with Duchenne and Becker muscular dystrophy (B). Paraffin section.
E-Cadherin
Clone 36B5
1 mL, 0.1 mL lyophilized NCL-E-Cad P (HIER)
7 mL ready-to-use RTU-E-Cad P (HIER)
7 mL Bond ready-to-use PA0387 P (HIER)
E-cadherin is a Ca 2+ -dependent, transmembrane cell adhesion molecule. It
plays an important role in the growth, development and the intercellular
adhesion of epithelial cells. Most tumors have an abnormal architecture and
any subsequent loss of adhesiveness is thought to be an important step in
the development of local invasion. E-cadherin may have a role in neoplastic
progression, particularly as a suppressor of invasion. In prostate cancers,
for example, the expression of E-cadherin is reported to be reduced or
absent in comparison with its expression in normal prostate which is
uniformly strong. Reduced expression or absence of E-cadherin in addition
to alpha, beta and gamma-catenin in primary breast carcinomas has also
been reported and these four proteins are associated with the development
of metastases.The main immunohistochemical utility of E-cadherin is to
highlight differential expression of this protein in lobular and ductal
carcinomas.
Refer to page 28 for the Bond ready-to-use format.
Human pulmonary adenocarcinoma: immunohistochemical staining for E-cadherin using
NCL-E-Cad. Note intense cytoplasmic and membrane staining of tumor cells. Paraffin section.
Products in this catalog are subject to regulatory approval.
This catalog is not for use in the USA.