Labelling Review row-Online
Labelling Review row-Online
Labelling Review row-Online
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Elastin<br />
Clone BA-4<br />
0.5 mL lyophilized NCL-ELASTIN P (Enzyme)<br />
Elastin is a polymeric protein found in connective tissue which imparts the<br />
property of elasticity to vertebrate elastic tissue. It is synthesized and<br />
secreted as a soluble, single-chain protein (tropoelastin) which undergoes a<br />
number of post-ribosomal modifications prior to its organization into an<br />
elastic fiber in the extracellular space. Once secreted, tropoelastin<br />
molecules are joined covalently via chemical modification and cross-linking<br />
of specific lysyl residues to form the mature insoluble elastin.<br />
Ultrastructurally, it is predominantly an amorphous material which may<br />
change its morphology with ageing and different disease states. The<br />
abnormal accumulation of elastic tissue in blood vessels is found in<br />
atherosclerosis and hypertension. Genetic defects in the elastin molecule<br />
are reported to lead to inherited diseases such as Marfan's syndrome,<br />
pseudoxanthoma elasticum and the Bushke-Ollendorf syndrome.<br />
Human aorta: immunohistochemical staining for elastin using NCL-ELASTIN. Note extracellular<br />
staining within the arterial wall. Paraffin section.<br />
Emerin<br />
Clone 4G5<br />
1 mL, 0.1 mL lyophilized NCL-EMERIN F P (HIER) W<br />
Emery-Dreifuss muscular dystrophy (EDMD) is a late onset X-linked<br />
recessive disorder characterized by slowly progressing contractures,<br />
wasting of skeletal muscle and cardiomyopathy usually presented as heart<br />
block. Contractures are seen in the elbows, Achilles tendons and<br />
postcervical muscles with humero-peroneal distribution early in the course<br />
of the disease. The STA gene, at Xq28 locus, encodes a serine-rich 34 kD<br />
protein, emerin, which is ubiquitous in tissues and is found in highest<br />
concentration in skeletal and cardiac muscle. Emerin is localized in the<br />
nuclear membrane of normal muscle cells and its deficiency plays a crucial<br />
part in the pathology of EDMD.<br />
Product Specific Information<br />
NCL-EMERIN is of use in the detection of the normal STA gene product.<br />
Human skeletal muscle: immunohistochemical staining for emerin using NCL-EMERIN.<br />
Note perinuclear staining of all cell nuclei. Paraffin section.<br />
Endoglin (CD105)<br />
Clone 4G11<br />
1 mL, 0.1 mL lyophilized NCL-CD105 P (HIER)<br />
CD105, also known as Endoglin, is an endothelial homodimeric membrane<br />
glycoprotein containing the peptide sequence RGD which is a recognition<br />
motif for adhesion receptors of the integrin family. It has been proposed that<br />
endoglin is a TGF-beta receptor. CD105 antigen is reported to be expressed<br />
on endothelial cells of capillaries, arterioles and venules in a variety of<br />
tissues and at low levels on acute lymphoblastic and myelocytic leukemia<br />
cells. ENDOGLIN expression may be of interest in the study of monocyte<br />
differentiation into macrophages, studies of cellular adhesion of circulating<br />
blood cells and in the lysis of CD105 positive cells in the presence of<br />
complement.<br />
Normal human ovary: immunohistochemical staining for endoglin using NCL-CD105. Note<br />
membrane staining of cells in both the theca interna and theca externa. Paraffin section.<br />
Endothelial Cell Marker (CD34)<br />
Clone QBEnd/10<br />
1 mL, 0.1 mL lyophilized NCL-END F P (Enzyme) C<br />
1 mL liquid NCL-L-END F P (Enzyme) C<br />
7 mL ready-to-use RTU-END F P (Enzyme)<br />
7 mL Bond ready-to-use PA0212 P (HIER)<br />
Refer to page 77 for further information about CD34.<br />
Endothelin-1 Receptor (ETA)<br />
Clone RJT24<br />
1 mL, 0.1 mL liquid NCL-L-ETA P (HIER)<br />
Endothelins (ET) are potent vasoconstrictive peptides originally isolated<br />
from vascular endothelial cells. Their biological effects are mediated<br />
through two different receptors, endothelin-1-selective endothelin receptor<br />
(ETA) and the non-selective receptor sub-type (ETB). Analysis of mRNA by<br />
Northern blotting reported high levels in aorta, lung, atrium, colon and<br />
placenta with moderate levels in the cerebral cortex, cerebellum, ventricle,<br />
kidney, adrenal glands and duodenum, whereas liver was negative. Studies<br />
using immunohistochemistry have reported the presence of ETA in medial<br />
smooth muscle of arteries, full-term placenta, normal and diseased gall<br />
bladder and ovarian luteinized granulosa cells. ETA has also been reported<br />
to have a role in gastric ulcer healing and in the development of neural-crest<br />
derived cells.<br />
F Frozen I Immunofluorescence E Electron microscopy<br />
P Paraffin C Flow cytometry O Other applications<br />
W Western blotting<br />
/ 103<br />
Primary Antibodies